18 Neonatal surgery

Anorectal malformations

Anorectal malformations

In an anorectal malformation, there is usually no opening in boys, and the rectum ends either blindly (notably in aneuploi dies) or with a fistula to the bulbar urethra ( Figure 18.13a prostate or bladder neck. Occasionally , there is a rectoperineal fistula in a boy . In contrast, there is usually a rectovestibular ( Figure 18.13b ) or rectoperineal fistula in gir ls; meconium is passed and therefore, many are missed on cursory newborn examinations. In girls, the rectum may join a common channel with the vagina and urethra; this is referred to as a cloaca ( Figure 18.13c ). In boys, a divided proximal sigmoid colostomy allows feed ing. A contrast study is performed through the defunctioned end ( Figure 18.14 ). Repair of prostatic and bladder neck fistu lae may be approached with a combined laparoscopic and per ineal approach, wher eas prostatic and bulbar urethral fistulae can both be approached in a posterior sagittal anorectoplasty (PSARP). The stoma is closed at a third stage. Most perineal and some vestibular fistulae can be transposed into the muscle complex without a stoma.

(a) Figure 18.13 (a) Rectobulbar urethral /f_i stula in a boy. (b) /uni00A0 Rectovestibular /f_i stula in a girl. (c) Cloaca in a girl. B R

Figure 18.14 A rectourethral /f_i stula, visible on a contrast study per

formed via a sigmoid colostomy. The bladder is /f_i lled with contrast via the /f_i stula and the radio-opaque dot has been placed on the infant’s perineum over the normal site of the anus. B, bladder; R, rectum.

Anorectal malformations

In an anorectal malformation, there is usually no opening in boys, and the rectum ends either blindly (notably in aneuploi dies) or with a fistula to the bulbar urethra ( Figure 18.13a prostate or bladder neck. Occasionally , there is a rectoperineal fistula in a boy . In contrast, there is usually a rectovestibular ( Figure 18.13b ) or rectoperineal fistula in gir ls; meconium is passed and therefore, many are missed on cursory newborn examinations. In girls, the rectum may join a common channel with the vagina and urethra; this is referred to as a cloaca ( Figure 18.13c ). In boys, a divided proximal sigmoid colostomy allows feed ing. A contrast study is performed through the defunctioned end ( Figure 18.14 ). Repair of prostatic and bladder neck fistu lae may be approached with a combined laparoscopic and per ineal approach, wher eas prostatic and bulbar urethral fistulae can both be approached in a posterior sagittal anorectoplasty (PSARP). The stoma is closed at a third stage. Most perineal and some vestibular fistulae can be transposed into the muscle complex without a stoma.

(a) Figure 18.13 (a) Rectobulbar urethral /f_i stula in a boy. (b) /uni00A0 Rectovestibular /f_i stula in a girl. (c) Cloaca in a girl. B R

Figure 18.14 A rectourethral /f_i stula, visible on a contrast study per

formed via a sigmoid colostomy. The bladder is /f_i lled with contrast via the /f_i stula and the radio-opaque dot has been placed on the infant’s perineum over the normal site of the anus. B, bladder; R, rectum.

Anorectal malformations

In an anorectal malformation, there is usually no opening in boys, and the rectum ends either blindly (notably in aneuploi dies) or with a fistula to the bulbar urethra ( Figure 18.13a prostate or bladder neck. Occasionally , there is a rectoperineal fistula in a boy . In contrast, there is usually a rectovestibular ( Figure 18.13b ) or rectoperineal fistula in gir ls; meconium is passed and therefore, many are missed on cursory newborn examinations. In girls, the rectum may join a common channel with the vagina and urethra; this is referred to as a cloaca ( Figure 18.13c ). In boys, a divided proximal sigmoid colostomy allows feed ing. A contrast study is performed through the defunctioned end ( Figure 18.14 ). Repair of prostatic and bladder neck fistu lae may be approached with a combined laparoscopic and per ineal approach, wher eas prostatic and bulbar urethral fistulae can both be approached in a posterior sagittal anorectoplasty (PSARP). The stoma is closed at a third stage. Most perineal and some vestibular fistulae can be transposed into the muscle complex without a stoma.

(a) Figure 18.13 (a) Rectobulbar urethral /f_i stula in a boy. (b) /uni00A0 Rectovestibular /f_i stula in a girl. (c) Cloaca in a girl. B R

Figure 18.14 A rectourethral /f_i stula, visible on a contrast study per

formed via a sigmoid colostomy. The bladder is /f_i lled with contrast via the /f_i stula and the radio-opaque dot has been placed on the infant’s perineum over the normal site of the anus. B, bladder; R, rectum.

Biliary atresia choledochal malformation

Biliary atresia/choledochal malformation

Congenital or acquired (e.g. cytomegalovirus) extrahepatic biliary atresia is a progressive obliterative cholangiopathy with absent or narrow bile ducts. Type I involves the common bile duct, type II the common hepatic duct, and 80% have the most common type III, involving the proximal bile ducts. Biliary atresia presents with conjugated hyperbilirubinaemia, pale stools and dark urine in the first few weeks of life. If presenting Morio Kasai , 1922–2008, pioneering Japanese surgeon, trained by C Everett Koop. César Roux , 1857–1934, Swiss surgeon, assistant to Theodor Kocher. - ) - - late, there may be malabsorption, growth failure and coagulop - athy . Some associations appear in Table 18.1 . The diagnosis is confirmed with a radionucleotide hepatobiliary iminodia - cetic acid (HIDA) scan. Early diagnosis and avoiding sepsis ma y prevent irreversible liver fibrosis and death. The Kasai he patico-portoenterostomy using a jejunal Roux-en-Y loop anastomosed to the portal plate gives drainage for some years,

Figure 18.10 A ventilated neonate with an acute abdomen awaiting laparotomy. Figure 18.11 Abdominal radiograph showing pneumatosis.

but many need liver transplantation. Congenital choledochal malformations manifest as cystic dilatations of the biliary tree and are also managed with resection and portoenterostomy .

Figure 18.12 Operative appearance of neonatal necrotising entero colitis.

Biliary atresia/choledochal malformation

Congenital or acquired (e.g. cytomegalovirus) extrahepatic biliary atresia is a progressive obliterative cholangiopathy with absent or narrow bile ducts. Type I involves the common bile duct, type II the common hepatic duct, and 80% have the most common type III, involving the proximal bile ducts. Biliary atresia presents with conjugated hyperbilirubinaemia, pale stools and dark urine in the first few weeks of life. If presenting Morio Kasai , 1922–2008, pioneering Japanese surgeon, trained by C Everett Koop. César Roux , 1857–1934, Swiss surgeon, assistant to Theodor Kocher. - ) - - late, there may be malabsorption, growth failure and coagulop - athy . Some associations appear in Table 18.1 . The diagnosis is confirmed with a radionucleotide hepatobiliary iminodia - cetic acid (HIDA) scan. Early diagnosis and avoiding sepsis ma y prevent irreversible liver fibrosis and death. The Kasai he patico-portoenterostomy using a jejunal Roux-en-Y loop anastomosed to the portal plate gives drainage for some years,

Figure 18.10 A ventilated neonate with an acute abdomen awaiting laparotomy. Figure 18.11 Abdominal radiograph showing pneumatosis.

but many need liver transplantation. Congenital choledochal malformations manifest as cystic dilatations of the biliary tree and are also managed with resection and portoenterostomy .

Figure 18.12 Operative appearance of neonatal necrotising entero colitis.

Biliary atresia/choledochal malformation

Congenital or acquired (e.g. cytomegalovirus) extrahepatic biliary atresia is a progressive obliterative cholangiopathy with absent or narrow bile ducts. Type I involves the common bile duct, type II the common hepatic duct, and 80% have the most common type III, involving the proximal bile ducts. Biliary atresia presents with conjugated hyperbilirubinaemia, pale stools and dark urine in the first few weeks of life. If presenting Morio Kasai , 1922–2008, pioneering Japanese surgeon, trained by C Everett Koop. César Roux , 1857–1934, Swiss surgeon, assistant to Theodor Kocher. - ) - - late, there may be malabsorption, growth failure and coagulop - athy . Some associations appear in Table 18.1 . The diagnosis is confirmed with a radionucleotide hepatobiliary iminodia - cetic acid (HIDA) scan. Early diagnosis and avoiding sepsis ma y prevent irreversible liver fibrosis and death. The Kasai he patico-portoenterostomy using a jejunal Roux-en-Y loop anastomosed to the portal plate gives drainage for some years,

Figure 18.10 A ventilated neonate with an acute abdomen awaiting laparotomy. Figure 18.11 Abdominal radiograph showing pneumatosis.

but many need liver transplantation. Congenital choledochal malformations manifest as cystic dilatations of the biliary tree and are also managed with resection and portoenterostomy .

Figure 18.12 Operative appearance of neonatal necrotising entero colitis.

Congenital mesoblastic nephroma

Congenital mesoblastic nephroma

This renal tumour may present as a large palpable mass in a newborn with some having hypertension and hypercal - caemia (paraneoplastic syndromes). Cross-sectional imaging distinguishes a tumour from a multicystic dysplastic kidney . A nephroureterectomy is usually curative, but some may recur locally or metastasise. Congenital mesoblastic nephroma

This renal tumour may present as a large palpable mass in a newborn with some having hypertension and hypercal - caemia (paraneoplastic syndromes). Cross-sectional imaging distinguishes a tumour from a multicystic dysplastic kidney . A nephroureterectomy is usually curative, but some may recur locally or metastasise. Congenital mesoblastic nephroma

This renal tumour may present as a large palpable mass in a newborn with some having hypertension and hypercal - caemia (paraneoplastic syndromes). Cross-sectional imaging distinguishes a tumour from a multicystic dysplastic kidney . A nephroureterectomy is usually curative, but some may recur locally or metastasise.

Duodenal atresia

Duodenal atresia

The obstruction in duodenal atresia usually lies just distal to the ampulla of Vater. The proximal duodenum and pylorus dilate with swallowed amniotic fluid, rendering the pylorus temporarily incompetent. Occasionally , there is a web that may stretch like a windsock. If suspected antenatally , duodenal atresia is confirmed postnatally on an abdominal radiograph, showing a ‘double bubble’ ( Figure 18.3 ); if missed, it presents with bilious vomiting. The hepatopancreatic duct may have openings on either side of the atresia, and a little gas can pass distally . Repair is by open Kimura duodenoduodenostomy ( Figure 18.4 ) with or without a trans-anastomotic tube. Duodenal atresia

The obstruction in duodenal atresia usually lies just distal to the ampulla of Vater. The proximal duodenum and pylorus dilate with swallowed amniotic fluid, rendering the pylorus temporarily incompetent. Occasionally , there is a web that may stretch like a windsock. If suspected antenatally , duodenal atresia is confirmed postnatally on an abdominal radiograph, showing a ‘double bubble’ ( Figure 18.3 ); if missed, it presents with bilious vomiting. The hepatopancreatic duct may have openings on either side of the atresia, and a little gas can pass distally . Repair is by open Kimura duodenoduodenostomy ( Figure 18.4 ) with or without a trans-anastomotic tube. Duodenal atresia

The obstruction in duodenal atresia usually lies just distal to the ampulla of Vater. The proximal duodenum and pylorus dilate with swallowed amniotic fluid, rendering the pylorus temporarily incompetent. Occasionally , there is a web that may stretch like a windsock. If suspected antenatally , duodenal atresia is confirmed postnatally on an abdominal radiograph, showing a ‘double bubble’ ( Figure 18.3 ); if missed, it presents with bilious vomiting. The hepatopancreatic duct may have openings on either side of the atresia, and a little gas can pass distally . Repair is by open Kimura duodenoduodenostomy ( Figure 18.4 ) with or without a trans-anastomotic tube.

Exomphalos

Exomphalos

Exomphalos describes a central abdominal wall defect in which prolapsed viscera are covered in a thin, three-layered membrane (peritoneum, Wharton’s jelly and amnion) in continuity with the umbilical cord. Exomphalos minor (<5 /uni00A0 cm, liver not involved) is commonly associated with other anomalies ( Table 18.1 ) and the defect is easily closed. In exomphalos major ( Figure 18.19 ) the sac can be dressed with a topical antibacterial agent (e.g. manuka honey , silver sulfadiazine), allowing epithelialisation Soave described in 1964. and later closure of the ventral hernia. If an early closure of an exomphalos major is attempted, close observation for an abdominal compartment syndrome is mandated.

Figure 18.18 Gastroschisis in a preformed silo. Figure 18.19 Exomphalos major.

Exomphalos

Exomphalos describes a central abdominal wall defect in which prolapsed viscera are covered in a thin, three-layered membrane (peritoneum, Wharton’s jelly and amnion) in continuity with the umbilical cord. Exomphalos minor (<5 /uni00A0 cm, liver not involved) is commonly associated with other anomalies ( Table 18.1 ) and the defect is easily closed. In exomphalos major ( Figure 18.19 ) the sac can be dressed with a topical antibacterial agent (e.g. manuka honey , silver sulfadiazine), allowing epithelialisation Soave described in 1964. and later closure of the ventral hernia. If an early closure of an exomphalos major is attempted, close observation for an abdominal compartment syndrome is mandated.

Figure 18.18 Gastroschisis in a preformed silo. Figure 18.19 Exomphalos major.

Exomphalos

Exomphalos describes a central abdominal wall defect in which prolapsed viscera are covered in a thin, three-layered membrane (peritoneum, Wharton’s jelly and amnion) in continuity with the umbilical cord. Exomphalos minor (<5 /uni00A0 cm, liver not involved) is commonly associated with other anomalies ( Table 18.1 ) and the defect is easily closed. In exomphalos major ( Figure 18.19 ) the sac can be dressed with a topical antibacterial agent (e.g. manuka honey , silver sulfadiazine), allowing epithelialisation Soave described in 1964. and later closure of the ventral hernia. If an early closure of an exomphalos major is attempted, close observation for an abdominal compartment syndrome is mandated.

Figure 18.18 Gastroschisis in a preformed silo. Figure 18.19 Exomphalos major.

Gastroschisis

Gastroschisis

In gastroschisis, an abdominal wall defect lies to the right- hand side of the umbilical cord and transmits the small and large intestine, stomach, bladder and sometimes the ovaries or undescended abdominal testes ( Figure 18.16 ). Risk factors include teenage pregnancy , recreational drugs, smoking and genitourinary infection in pregnancy . It is easily diagnosed antenatally , allowing delivery near a surgical unit. Vaginal delivery is appropriate. After birth, twisting or kinking of the mesenteric blood supply must be avoided. The abdomen and viscera are wrapped using a transparent plastic food wrap (e.g. cling film, Saran wrap), a large-bore nasogastric tube is placed, and fluid resuscitation is initiated. The bowel may have a thick wall and be matted together. Sometimes, the defect closes antenatally , causing an atresia and an exter length of damaged intestine ( Figure 18.17 ). A narrow defect may need to be widened and a sutured silo (silo: structure for storage) created using Silastic sheeting or an empty intravenous fluid bag. Primary closure under general anaesthesia usually requires NICU admission for postoperative ventilation. An alternative is to place a preformed silo at the bedside ( Figure 18.18 ), followed by gradual reduction, which sometimes avoids general anaesthesia altogether. PN is needed for around 4 /uni00A0 weeks or longer while intestinal motility improves. Orvar Swenson , 1909–2012, Swedish-born American paediatric surgeon who discovered the cause of Hirschsprung’s disease. Bernard Duhamel , 1917–1996, Professor of Surgery , Hôpital Saint-Denis, Paris, France. Asa G Yancey , 1916–2013, African-American surgeon who described in 1952 what Thomas Wharton , 1614–1673, English anatomist: ‘The description of the glands of the entire body’, 1656. nal

Figure 18.16 Gastroschisis. Figure 18.17 Closing gastroschisis: the defect has narrowed and occluded the vessels to a few loops of intestine. Note the atresia found at laparotomy.

Gastroschisis

In gastroschisis, an abdominal wall defect lies to the right- hand side of the umbilical cord and transmits the small and large intestine, stomach, bladder and sometimes the ovaries or undescended abdominal testes ( Figure 18.16 ). Risk factors include teenage pregnancy , recreational drugs, smoking and genitourinary infection in pregnancy . It is easily diagnosed antenatally , allowing delivery near a surgical unit. Vaginal delivery is appropriate. After birth, twisting or kinking of the mesenteric blood supply must be avoided. The abdomen and viscera are wrapped using a transparent plastic food wrap (e.g. cling film, Saran wrap), a large-bore nasogastric tube is placed, and fluid resuscitation is initiated. The bowel may have a thick wall and be matted together. Sometimes, the defect closes antenatally , causing an atresia and an exter length of damaged intestine ( Figure 18.17 ). A narrow defect may need to be widened and a sutured silo (silo: structure for storage) created using Silastic sheeting or an empty intravenous fluid bag. Primary closure under general anaesthesia usually requires NICU admission for postoperative ventilation. An alternative is to place a preformed silo at the bedside ( Figure 18.18 ), followed by gradual reduction, which sometimes avoids general anaesthesia altogether. PN is needed for around 4 /uni00A0 weeks or longer while intestinal motility improves. Orvar Swenson , 1909–2012, Swedish-born American paediatric surgeon who discovered the cause of Hirschsprung’s disease. Bernard Duhamel , 1917–1996, Professor of Surgery , Hôpital Saint-Denis, Paris, France. Asa G Yancey , 1916–2013, African-American surgeon who described in 1952 what Thomas Wharton , 1614–1673, English anatomist: ‘The description of the glands of the entire body’, 1656. nal

Figure 18.16 Gastroschisis. Figure 18.17 Closing gastroschisis: the defect has narrowed and occluded the vessels to a few loops of intestine. Note the atresia found at laparotomy.

Gastroschisis

In gastroschisis, an abdominal wall defect lies to the right- hand side of the umbilical cord and transmits the small and large intestine, stomach, bladder and sometimes the ovaries or undescended abdominal testes ( Figure 18.16 ). Risk factors include teenage pregnancy , recreational drugs, smoking and genitourinary infection in pregnancy . It is easily diagnosed antenatally , allowing delivery near a surgical unit. Vaginal delivery is appropriate. After birth, twisting or kinking of the mesenteric blood supply must be avoided. The abdomen and viscera are wrapped using a transparent plastic food wrap (e.g. cling film, Saran wrap), a large-bore nasogastric tube is placed, and fluid resuscitation is initiated. The bowel may have a thick wall and be matted together. Sometimes, the defect closes antenatally , causing an atresia and an exter length of damaged intestine ( Figure 18.17 ). A narrow defect may need to be widened and a sutured silo (silo: structure for storage) created using Silastic sheeting or an empty intravenous fluid bag. Primary closure under general anaesthesia usually requires NICU admission for postoperative ventilation. An alternative is to place a preformed silo at the bedside ( Figure 18.18 ), followed by gradual reduction, which sometimes avoids general anaesthesia altogether. PN is needed for around 4 /uni00A0 weeks or longer while intestinal motility improves. Orvar Swenson , 1909–2012, Swedish-born American paediatric surgeon who discovered the cause of Hirschsprung’s disease. Bernard Duhamel , 1917–1996, Professor of Surgery , Hôpital Saint-Denis, Paris, France. Asa G Yancey , 1916–2013, African-American surgeon who described in 1952 what Thomas Wharton , 1614–1673, English anatomist: ‘The description of the glands of the entire body’, 1656. nal

Figure 18.16 Gastroschisis. Figure 18.17 Closing gastroschisis: the defect has narrowed and occluded the vessels to a few loops of intestine. Note the atresia found at laparotomy.

Hirschsprung’s disease

Hirschsprung’s disease

Genetic defects (e.g. RET , EDNRB, EDN3 ) can a ff ect the - migration of neural crest-derived intestinal neurones (neuro - ), cristopathy), leading to aganglionosis and thickened nerve trunks in the distal bowel. There may be a family history . Agan - glionic bowel fails to relax, causing a functional obstruction. Aganglionosis extends from the an us to the sigmoid colon in 75%, the proximal colon in 15%, and the terminal ileum in 10% of cases. A transition zone lies between dilated, proximal, normal bo wel and narrow , distal aganglionic bowel. Neonatal Hirschsprung’s disease presents with delayed passage of meco - - nium, abdominal distension and bilious vomiting requiring resuscitation, gastric decompression, antibiotics and a bowel - washout. The diagnosis is made on a cot-side suction rectal - biopsy . A contrast enema may show the narrow aganglionic segment, a cone and dilated pr oximal bowel ( Figure 18.15 ). Daily rectal washouts may allow a period of growth at home before surgery . If decompression fails, a stoma is fashioned using frozen section histopathology to identify ganglionic bowel. Definitive surgery removes the aganglionic segment

(b) (c)

and brings ganglionic bowel to the anus; Swenson, Duhamel, Yancey–Soave and transanal ‘pull-throughs’ are options. Most children achieve reasonable bowel control, but some have residual constipation, incontinence or episodes of enterocolitis.

Figure 18.15 Barium enema in an infant, showing a ‘transition zone’ in the proximal sigmoid colon between the dilated proximal normally innervated bowel and the contracted aganglionic rectum.

Hirschsprung’s disease

Genetic defects (e.g. RET , EDNRB, EDN3 ) can a ff ect the - migration of neural crest-derived intestinal neurones (neuro - ), cristopathy), leading to aganglionosis and thickened nerve trunks in the distal bowel. There may be a family history . Agan - glionic bowel fails to relax, causing a functional obstruction. Aganglionosis extends from the an us to the sigmoid colon in 75%, the proximal colon in 15%, and the terminal ileum in 10% of cases. A transition zone lies between dilated, proximal, normal bo wel and narrow , distal aganglionic bowel. Neonatal Hirschsprung’s disease presents with delayed passage of meco - - nium, abdominal distension and bilious vomiting requiring resuscitation, gastric decompression, antibiotics and a bowel - washout. The diagnosis is made on a cot-side suction rectal - biopsy . A contrast enema may show the narrow aganglionic segment, a cone and dilated pr oximal bowel ( Figure 18.15 ). Daily rectal washouts may allow a period of growth at home before surgery . If decompression fails, a stoma is fashioned using frozen section histopathology to identify ganglionic bowel. Definitive surgery removes the aganglionic segment

(b) (c)

and brings ganglionic bowel to the anus; Swenson, Duhamel, Yancey–Soave and transanal ‘pull-throughs’ are options. Most children achieve reasonable bowel control, but some have residual constipation, incontinence or episodes of enterocolitis.

Figure 18.15 Barium enema in an infant, showing a ‘transition zone’ in the proximal sigmoid colon between the dilated proximal normally innervated bowel and the contracted aganglionic rectum.

Hirschsprung’s disease

Genetic defects (e.g. RET , EDNRB, EDN3 ) can a ff ect the - migration of neural crest-derived intestinal neurones (neuro - ), cristopathy), leading to aganglionosis and thickened nerve trunks in the distal bowel. There may be a family history . Agan - glionic bowel fails to relax, causing a functional obstruction. Aganglionosis extends from the an us to the sigmoid colon in 75%, the proximal colon in 15%, and the terminal ileum in 10% of cases. A transition zone lies between dilated, proximal, normal bo wel and narrow , distal aganglionic bowel. Neonatal Hirschsprung’s disease presents with delayed passage of meco - - nium, abdominal distension and bilious vomiting requiring resuscitation, gastric decompression, antibiotics and a bowel - washout. The diagnosis is made on a cot-side suction rectal - biopsy . A contrast enema may show the narrow aganglionic segment, a cone and dilated pr oximal bowel ( Figure 18.15 ). Daily rectal washouts may allow a period of growth at home before surgery . If decompression fails, a stoma is fashioned using frozen section histopathology to identify ganglionic bowel. Definitive surgery removes the aganglionic segment

(b) (c)

and brings ganglionic bowel to the anus; Swenson, Duhamel, Yancey–Soave and transanal ‘pull-throughs’ are options. Most children achieve reasonable bowel control, but some have residual constipation, incontinence or episodes of enterocolitis.

Figure 18.15 Barium enema in an infant, showing a ‘transition zone’ in the proximal sigmoid colon between the dilated proximal normally innervated bowel and the contracted aganglionic rectum.

Introduction

INTRODUCTION

Neonatal surgeons are paediatric surgeons who manage life-threatening non-cardiac congenital anomalies and the acquired condition necrotising enterocolitis (NEC), seen in premature babies. Structural anomalies are associated with gene defects, aneuploidies (abnormal number of chromo somes), infections (e.g. toxoplasmosis, cytomegalovirus, rubella) and teratogens (e.g. drugs, smoking, alcohol). Late mechanical aetiologies are illustrated by ileal volvulus in cystic fibrosis, some lung hypoplasia in congenital diaphragmatic hernias and small intestine loss in closing g astroschisis. Insults acting during gastrulation /uni00A0 – /uni00A0 when cells are told what to do and where to go /uni00A0 – /uni00A0 may cause multiple anomalies, e.g. V ACTERL syndrome ( v ertebral, a norectal, c ardiac, t racheo e sophageal, r enal and l imb anomalies) and CHARGE syndrome ( c oloboma, defects, choanal a tresia, growth r etardation, g enital anomalies and e ar anomalies). See Chapter 44 for neural tube defects and Chapter 59 for heart defects. See Chapter 48 for the over lap with general surgery of childhood. Table 18.1 illustrates the need for careful examination, imaging and genetic inves tigations to screen for associations when an anomaly is found. When well-recognised anomalies ar e identified antenatally , neonatal surgeons, working with fetal medicine specialists and neonatologists, counsel parents about prognosis and postnatal surgical management. Friedrich Trendelenburg , 1844–1924, Professor of Surgery successively at Rostock (1875–1882), Bonn (1882–1895), Leipzig (1895–1911), Germany . The Tren delenburg position was first described in 1885.

Outline the presentation and management of necrotising • enterocolitis Describe two newborn tumours • Explain why adult surgeons need an overview of neonatal • surgery

Learning objectives

Learning objectives

To be able to: List /f_i ve aetiological classes underlying structural • congenital anomalies Give /f_i ve examples of how neonatal physiology and • anatomy in /f_l uence surgical care Describe at least /f_i ve congenital anomalies managed by • neonatal surgeons Learning objectives

To be able to: List /f_i ve aetiological classes underlying structural • congenital anomalies Give /f_i ve examples of how neonatal physiology and • anatomy in /f_l uence surgical care Describe at least /f_i ve congenital anomalies managed by • neonatal surgeons Learning objectives

To be able to: List /f_i ve aetiological classes underlying structural • congenital anomalies Give /f_i ve examples of how neonatal physiology and • anatomy in /f_l uence surgical care Describe at least /f_i ve congenital anomalies managed by • neonatal surgeons

Malrotation and volvulus

Malrotation and volvulus

Complex rotations in utero give the small bowel mesentery its broad, stable base, running from the duodenal–jejunal (DJ) flexure in the left upper quadrant to the caecum in the right lower quadrant. Incomplete rotations leave the mesentery with a narrow , unstable base at risk of twisting. Sometimes, fibrous Ladd’s bands run between a central upper abdominal caecum and the right lateral abdominal wall, obstructing the duodenum ( Figure 18.6 ). A chronic volvulus that spares the vessels may present with protein-losing enteropathy or chylous ascites, while acute luminal obstruction presents with green - bilious vomiting and is the harbinger of vascular compromise lus suspected in an acidotic baby vomiting bile with a gasless abdominal radiograph and a scaphoid abdomen demands an immediate laparotomy . Well babies presenting with bilious vomiting have a contrast study to locate the DJ flexure. If the DJ flexure lies to the left of the vertebral column, at the level of the pylorus, the mesentery is likely to be stable. At operation, the bowel is placed in the non-rotated position and the mesentery is broadened /uni00A0 – /uni00A0 Ladd’s procedure ( Figure 18.8 ).

Figure 18.5 Small bowel atresia. Figure 18.6 The narrow origin of the small bowel mesentery predis poses to midgut volvulus. Figure 18.7 An acute small bowel volvulus with vascular compromise: the baby’s head is to the right. Note that the terminal ileum, caecum and appendix in the upper central abdomen are well perfused. Figure 18.8 Ladd’s procedure: the duodenum is placed on the right, the colon on the left and the mesentery is broadened. The duodenum appears to lie near the colon since the wound is small. The appendix may be removed.

Malrotation and volvulus

Complex rotations in utero give the small bowel mesentery its broad, stable base, running from the duodenal–jejunal (DJ) flexure in the left upper quadrant to the caecum in the right lower quadrant. Incomplete rotations leave the mesentery with a narrow , unstable base at risk of twisting. Sometimes, fibrous Ladd’s bands run between a central upper abdominal caecum and the right lateral abdominal wall, obstructing the duodenum ( Figure 18.6 ). A chronic volvulus that spares the vessels may present with protein-losing enteropathy or chylous ascites, while acute luminal obstruction presents with green - bilious vomiting and is the harbinger of vascular compromise lus suspected in an acidotic baby vomiting bile with a gasless abdominal radiograph and a scaphoid abdomen demands an immediate laparotomy . Well babies presenting with bilious vomiting have a contrast study to locate the DJ flexure. If the DJ flexure lies to the left of the vertebral column, at the level of the pylorus, the mesentery is likely to be stable. At operation, the bowel is placed in the non-rotated position and the mesentery is broadened /uni00A0 – /uni00A0 Ladd’s procedure ( Figure 18.8 ).

Figure 18.5 Small bowel atresia. Figure 18.6 The narrow origin of the small bowel mesentery predis poses to midgut volvulus. Figure 18.7 An acute small bowel volvulus with vascular compromise: the baby’s head is to the right. Note that the terminal ileum, caecum and appendix in the upper central abdomen are well perfused. Figure 18.8 Ladd’s procedure: the duodenum is placed on the right, the colon on the left and the mesentery is broadened. The duodenum appears to lie near the colon since the wound is small. The appendix may be removed.

Malrotation and volvulus

Complex rotations in utero give the small bowel mesentery its broad, stable base, running from the duodenal–jejunal (DJ) flexure in the left upper quadrant to the caecum in the right lower quadrant. Incomplete rotations leave the mesentery with a narrow , unstable base at risk of twisting. Sometimes, fibrous Ladd’s bands run between a central upper abdominal caecum and the right lateral abdominal wall, obstructing the duodenum ( Figure 18.6 ). A chronic volvulus that spares the vessels may present with protein-losing enteropathy or chylous ascites, while acute luminal obstruction presents with green - bilious vomiting and is the harbinger of vascular compromise lus suspected in an acidotic baby vomiting bile with a gasless abdominal radiograph and a scaphoid abdomen demands an immediate laparotomy . Well babies presenting with bilious vomiting have a contrast study to locate the DJ flexure. If the DJ flexure lies to the left of the vertebral column, at the level of the pylorus, the mesentery is likely to be stable. At operation, the bowel is placed in the non-rotated position and the mesentery is broadened /uni00A0 – /uni00A0 Ladd’s procedure ( Figure 18.8 ).

Figure 18.5 Small bowel atresia. Figure 18.6 The narrow origin of the small bowel mesentery predis poses to midgut volvulus. Figure 18.7 An acute small bowel volvulus with vascular compromise: the baby’s head is to the right. Note that the terminal ileum, caecum and appendix in the upper central abdomen are well perfused. Figure 18.8 Ladd’s procedure: the duodenum is placed on the right, the colon on the left and the mesentery is broadened. The duodenum appears to lie near the colon since the wound is small. The appendix may be removed.

Meconium ileus

Meconium ileus

Inspissated meconium may cause a distal ileal obstruction. A segmental ileal volvulus can follow and create an atresia. If the ileum perforates, it may seal or persist and cause a large meconium pseudocyst. An abdominal radiograph shows obstruction with a ground-glass appearance. Peritoneal calci - fication indicates an antenatal perforation. Simple cases are managed with a water-soluble hyperosmolar contrast enema (diatrizoate) using fluoroscopy in a well-hydrated neonate ( Figure 18.9 ). Complicated cases require a laparotomy and enterotomy for a luminal washout; a temporary stoma may be required. Postoperatively , N-acetylcysteine can be given by nasogastric tube and as enemas to loosen residual meconium. Genetic investigations look for defects in the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Meconium ileus

Inspissated meconium may cause a distal ileal obstruction. A segmental ileal volvulus can follow and create an atresia. If the ileum perforates, it may seal or persist and cause a large meconium pseudocyst. An abdominal radiograph shows obstruction with a ground-glass appearance. Peritoneal calci - fication indicates an antenatal perforation. Simple cases are managed with a water-soluble hyperosmolar contrast enema (diatrizoate) using fluoroscopy in a well-hydrated neonate ( Figure 18.9 ). Complicated cases require a laparotomy and enterotomy for a luminal washout; a temporary stoma may be required. Postoperatively , N-acetylcysteine can be given by nasogastric tube and as enemas to loosen residual meconium. Genetic investigations look for defects in the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Meconium ileus

Inspissated meconium may cause a distal ileal obstruction. A segmental ileal volvulus can follow and create an atresia. If the ileum perforates, it may seal or persist and cause a large meconium pseudocyst. An abdominal radiograph shows obstruction with a ground-glass appearance. Peritoneal calci - fication indicates an antenatal perforation. Simple cases are managed with a water-soluble hyperosmolar contrast enema (diatrizoate) using fluoroscopy in a well-hydrated neonate ( Figure 18.9 ). Complicated cases require a laparotomy and enterotomy for a luminal washout; a temporary stoma may be required. Postoperatively , N-acetylcysteine can be given by nasogastric tube and as enemas to loosen residual meconium. Genetic investigations look for defects in the cystic fibrosis transmembrane conductance regulator (CFTR) protein.

NEONATAL GASTROINTESTINAL SURGERY Oesophageal atre

NEONATAL GASTROINTESTINAL SURGERY Oesophageal atresia/ tracheoesophageal fi stula (OA/TOF)

Type D Type E Figure 18.1 Anatom

ical variations in tracheoesophageal /f_i s t u l a w i t h o r w i t h o u t oesophageal atresia. In Type C, the upper pouch ends in the neck or upper chest but occasionally it reaches the /f_i stula where muscle /f_i bres are shared.

/uni25CF /uni25CF /uni25CF /uni2192 /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF Robert L Replogle , 1931–2016, Chicago, the last trainee of Robert E Gross. A /uni00A0 nasogastric tube coiled in the upper oesophageal pouch on a chest radiograph suggests the diagnosis. A nasal or oral sump Replogle tube is placed to drain saliva and prevent aspiration. Positive airway pressure is avoided as air passing through the fistula causes gastric distension, compromised ventilation, and risks perforation ( Figure 18.2 ). If pressure support is needed, perhaps because of RDS, prompt fistula ligation is needed. Types A and B typically have a long gap and may require oesophageal replacement; options include colonic or jeju - nal interposition or gastric transposition some months after a cervical oesophagostomy and a gastrostomy . In many cases the ends can be brought together by progressive traction and yed anastomosis. dela In types C and D, the fistula is divided through a right y or thoracoscopically . If the neonate is stable thoracotom and the gap favourable, an anastomosis is fashioned over a ly feeding. If a primary trans-anastomotic tube, facilitating ear anastomosis is not possible, then options include a delayed anastomosis after a few weeks of growth, or the use of traction sutures and an earlier anastomosis, or a much later interposition. Traction sutures can be internal or external. Nutrition is supported through a gastrostomy . Complications after a repair include anastomotic leaks, oesophageal strictures and refistulation. Minor leaks often set - tle without intervention, strictures need dilating with a bougie or a balloon, and refistulation needs repair. Type E is an isolated ‘H’-type tracheoesophageal fistula he fistula is usually found in the neck on a without atresia. T rent chest infec - contrast swallow . Type E presents with recur tions or coughing after feeds and is usually repaired in the neck.

infants. Airway Intubation can be challenging as the occiput /f_l exes the neck, the tongue is large and the epiglottis is long, angulated and positioned high and close to the soft palate. A straight blade laryngoscope, an uncuffed tube and a neutral position for the neck Abdomen The liver is large and fragile and the bladder rises out of the pelvis. The abdomen must be entered carefully. The umbilical vein is patent for many days after birth and is ligated before being divided. Respiratory (respiratory distress syndrome [RDS], chronic lung disease) Preterm delivery, gestational diabetes and birth asphyxia all lower pulmonary surfactant levels, resulting in decreased lung volume and compliance and promoting airway collapse on expiration and atelectasis. Fewer type 1 muscle /f_i bres in the diaphragm and intercostals increases early fatigue. Chronic in /f_l ammatory lung disease with scarring is seen in pr eterm babies from prolonged ventilation, overin /f_l ation, high pressures and oxygen toxicity. Surfactant, oxygen, continuous positive airway pressure (CPAP) or mechanical ventilation Cardiovascular A fall in pulmonary vascular resistance (PVR) at birth helps establish the postnatal circulation. In the early postnatal period, hypoxia, stress, high P CO or 2 acidosis may raise PVR; if the ductus arteriosus and foramen ovale are open, blood shunts R L causing hypoxaemia An underdeveloped barore /f_l ex means unchecked blood loss leads rapidly to hypotension Fluids and electrolytes Excess total body water and extracellular /f_l uid are excreted after birth in a physiological diuresis. Insensible losses increase with low birth weight and low gestational age. The immature kidney loses sodium, bicarbonate, glucose, amino acids and phosphates. Low glycogen stores at birth promote hypoglycaemia, particularly in the preterm. Use local neonatal intensive care unit (NICU) protocols. Maintenance /f_l uids need 10% glucose and appropriate electrolytes Watch for hyperglycaemia with hypernatraemia, which increases the risk of intraventricular haemorrhage in the preterm Replace nasogastric losses or stoma losses (>15 /uni00A0 mL/kg/day) millilitre for millilitre with 0.9% NaCl, 0.15% KCl Nutrition Reserves are de /f_i cient in the premature and postnatal starvation affects neurological development. Start central parenteral nutrition as a matter of urgency Thermoregulation A high surface area to bodyweight ratio increases heat loss; particularly during exposure for anaesthesia (exacerbated by vasodilation) and surgery, there is an inability to shiver. Low temperatures promote coagulopathy, which is compounded by the acidosis from poor peripheral perfusion and myocardial depression. Warm incubators, limit exposure for procedures, warm theatre, warm /f_l uids Figure 18.2 Tracheoesophageal /f_i stula/oesophageal atresia with gastric perforation in a 28-week gestation, 1000-g baby. Note the endotracheal tube (ET), Replogle tube in the upper pouch, the umbil

ical venous catheter (UVC), free abdominal air around the liver and either side of the falciform ligament above the UVC and patchy lung /f_i elds of respiratory distress syndrome.

Figure 18.3 Double bubble in duodenal atresia (gastric and /f_i rst part of the duodenum). Note the umbilical cord and clamp in the lower part of the image.

NEONATAL GASTROINTESTINAL SURGERY Oesophageal atresia/ tracheoesophageal fi stula (OA/TOF)

Type D Type E Figure 18.1 Anatom

ical variations in tracheoesophageal /f_i s t u l a w i t h o r w i t h o u t oesophageal atresia. In Type C, the upper pouch ends in the neck or upper chest but occasionally it reaches the /f_i stula where muscle /f_i bres are shared.

/uni25CF /uni25CF /uni25CF /uni2192 /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF Robert L Replogle , 1931–2016, Chicago, the last trainee of Robert E Gross. A /uni00A0 nasogastric tube coiled in the upper oesophageal pouch on a chest radiograph suggests the diagnosis. A nasal or oral sump Replogle tube is placed to drain saliva and prevent aspiration. Positive airway pressure is avoided as air passing through the fistula causes gastric distension, compromised ventilation, and risks perforation ( Figure 18.2 ). If pressure support is needed, perhaps because of RDS, prompt fistula ligation is needed. Types A and B typically have a long gap and may require oesophageal replacement; options include colonic or jeju - nal interposition or gastric transposition some months after a cervical oesophagostomy and a gastrostomy . In many cases the ends can be brought together by progressive traction and yed anastomosis. dela In types C and D, the fistula is divided through a right y or thoracoscopically . If the neonate is stable thoracotom and the gap favourable, an anastomosis is fashioned over a ly feeding. If a primary trans-anastomotic tube, facilitating ear anastomosis is not possible, then options include a delayed anastomosis after a few weeks of growth, or the use of traction sutures and an earlier anastomosis, or a much later interposition. Traction sutures can be internal or external. Nutrition is supported through a gastrostomy . Complications after a repair include anastomotic leaks, oesophageal strictures and refistulation. Minor leaks often set - tle without intervention, strictures need dilating with a bougie or a balloon, and refistulation needs repair. Type E is an isolated ‘H’-type tracheoesophageal fistula he fistula is usually found in the neck on a without atresia. T rent chest infec - contrast swallow . Type E presents with recur tions or coughing after feeds and is usually repaired in the neck.

infants. Airway Intubation can be challenging as the occiput /f_l exes the neck, the tongue is large and the epiglottis is long, angulated and positioned high and close to the soft palate. A straight blade laryngoscope, an uncuffed tube and a neutral position for the neck Abdomen The liver is large and fragile and the bladder rises out of the pelvis. The abdomen must be entered carefully. The umbilical vein is patent for many days after birth and is ligated before being divided. Respiratory (respiratory distress syndrome [RDS], chronic lung disease) Preterm delivery, gestational diabetes and birth asphyxia all lower pulmonary surfactant levels, resulting in decreased lung volume and compliance and promoting airway collapse on expiration and atelectasis. Fewer type 1 muscle /f_i bres in the diaphragm and intercostals increases early fatigue. Chronic in /f_l ammatory lung disease with scarring is seen in pr eterm babies from prolonged ventilation, overin /f_l ation, high pressures and oxygen toxicity. Surfactant, oxygen, continuous positive airway pressure (CPAP) or mechanical ventilation Cardiovascular A fall in pulmonary vascular resistance (PVR) at birth helps establish the postnatal circulation. In the early postnatal period, hypoxia, stress, high P CO or 2 acidosis may raise PVR; if the ductus arteriosus and foramen ovale are open, blood shunts R L causing hypoxaemia An underdeveloped barore /f_l ex means unchecked blood loss leads rapidly to hypotension Fluids and electrolytes Excess total body water and extracellular /f_l uid are excreted after birth in a physiological diuresis. Insensible losses increase with low birth weight and low gestational age. The immature kidney loses sodium, bicarbonate, glucose, amino acids and phosphates. Low glycogen stores at birth promote hypoglycaemia, particularly in the preterm. Use local neonatal intensive care unit (NICU) protocols. Maintenance /f_l uids need 10% glucose and appropriate electrolytes Watch for hyperglycaemia with hypernatraemia, which increases the risk of intraventricular haemorrhage in the preterm Replace nasogastric losses or stoma losses (>15 /uni00A0 mL/kg/day) millilitre for millilitre with 0.9% NaCl, 0.15% KCl Nutrition Reserves are de /f_i cient in the premature and postnatal starvation affects neurological development. Start central parenteral nutrition as a matter of urgency Thermoregulation A high surface area to bodyweight ratio increases heat loss; particularly during exposure for anaesthesia (exacerbated by vasodilation) and surgery, there is an inability to shiver. Low temperatures promote coagulopathy, which is compounded by the acidosis from poor peripheral perfusion and myocardial depression. Warm incubators, limit exposure for procedures, warm theatre, warm /f_l uids Figure 18.2 Tracheoesophageal /f_i stula/oesophageal atresia with gastric perforation in a 28-week gestation, 1000-g baby. Note the endotracheal tube (ET), Replogle tube in the upper pouch, the umbil

ical venous catheter (UVC), free abdominal air around the liver and either side of the falciform ligament above the UVC and patchy lung /f_i elds of respiratory distress syndrome.

Figure 18.3 Double bubble in duodenal atresia (gastric and /f_i rst part of the duodenum). Note the umbilical cord and clamp in the lower part of the image.

NEONATAL GASTROINTESTINAL SURGERY Oesophageal atresia tracheoesophageal fi stula (OA TOF)

NEONATAL GASTROINTESTINAL SURGERY Oesophageal atresia/ tracheoesophageal fi stula (OA/TOF)

Type D Type E Figure 18.1 Anatom

ical variations in tracheoesophageal /f_i s t u l a w i t h o r w i t h o u t oesophageal atresia. In Type C, the upper pouch ends in the neck or upper chest but occasionally it reaches the /f_i stula where muscle /f_i bres are shared.

/uni25CF /uni25CF /uni25CF /uni2192 /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF Robert L Replogle , 1931–2016, Chicago, the last trainee of Robert E Gross. A /uni00A0 nasogastric tube coiled in the upper oesophageal pouch on a chest radiograph suggests the diagnosis. A nasal or oral sump Replogle tube is placed to drain saliva and prevent aspiration. Positive airway pressure is avoided as air passing through the fistula causes gastric distension, compromised ventilation, and risks perforation ( Figure 18.2 ). If pressure support is needed, perhaps because of RDS, prompt fistula ligation is needed. Types A and B typically have a long gap and may require oesophageal replacement; options include colonic or jeju - nal interposition or gastric transposition some months after a cervical oesophagostomy and a gastrostomy . In many cases the ends can be brought together by progressive traction and yed anastomosis. dela In types C and D, the fistula is divided through a right y or thoracoscopically . If the neonate is stable thoracotom and the gap favourable, an anastomosis is fashioned over a ly feeding. If a primary trans-anastomotic tube, facilitating ear anastomosis is not possible, then options include a delayed anastomosis after a few weeks of growth, or the use of traction sutures and an earlier anastomosis, or a much later interposition. Traction sutures can be internal or external. Nutrition is supported through a gastrostomy . Complications after a repair include anastomotic leaks, oesophageal strictures and refistulation. Minor leaks often set - tle without intervention, strictures need dilating with a bougie or a balloon, and refistulation needs repair. Type E is an isolated ‘H’-type tracheoesophageal fistula he fistula is usually found in the neck on a without atresia. T rent chest infec - contrast swallow . Type E presents with recur tions or coughing after feeds and is usually repaired in the neck.

infants. Airway Intubation can be challenging as the occiput /f_l exes the neck, the tongue is large and the epiglottis is long, angulated and positioned high and close to the soft palate. A straight blade laryngoscope, an uncuffed tube and a neutral position for the neck Abdomen The liver is large and fragile and the bladder rises out of the pelvis. The abdomen must be entered carefully. The umbilical vein is patent for many days after birth and is ligated before being divided. Respiratory (respiratory distress syndrome [RDS], chronic lung disease) Preterm delivery, gestational diabetes and birth asphyxia all lower pulmonary surfactant levels, resulting in decreased lung volume and compliance and promoting airway collapse on expiration and atelectasis. Fewer type 1 muscle /f_i bres in the diaphragm and intercostals increases early fatigue. Chronic in /f_l ammatory lung disease with scarring is seen in pr eterm babies from prolonged ventilation, overin /f_l ation, high pressures and oxygen toxicity. Surfactant, oxygen, continuous positive airway pressure (CPAP) or mechanical ventilation Cardiovascular A fall in pulmonary vascular resistance (PVR) at birth helps establish the postnatal circulation. In the early postnatal period, hypoxia, stress, high P CO or 2 acidosis may raise PVR; if the ductus arteriosus and foramen ovale are open, blood shunts R L causing hypoxaemia An underdeveloped barore /f_l ex means unchecked blood loss leads rapidly to hypotension Fluids and electrolytes Excess total body water and extracellular /f_l uid are excreted after birth in a physiological diuresis. Insensible losses increase with low birth weight and low gestational age. The immature kidney loses sodium, bicarbonate, glucose, amino acids and phosphates. Low glycogen stores at birth promote hypoglycaemia, particularly in the preterm. Use local neonatal intensive care unit (NICU) protocols. Maintenance /f_l uids need 10% glucose and appropriate electrolytes Watch for hyperglycaemia with hypernatraemia, which increases the risk of intraventricular haemorrhage in the preterm Replace nasogastric losses or stoma losses (>15 /uni00A0 mL/kg/day) millilitre for millilitre with 0.9% NaCl, 0.15% KCl Nutrition Reserves are de /f_i cient in the premature and postnatal starvation affects neurological development. Start central parenteral nutrition as a matter of urgency Thermoregulation A high surface area to bodyweight ratio increases heat loss; particularly during exposure for anaesthesia (exacerbated by vasodilation) and surgery, there is an inability to shiver. Low temperatures promote coagulopathy, which is compounded by the acidosis from poor peripheral perfusion and myocardial depression. Warm incubators, limit exposure for procedures, warm theatre, warm /f_l uids Figure 18.2 Tracheoesophageal /f_i stula/oesophageal atresia with gastric perforation in a 28-week gestation, 1000-g baby. Note the endotracheal tube (ET), Replogle tube in the upper pouch, the umbil

ical venous catheter (UVC), free abdominal air around the liver and either side of the falciform ligament above the UVC and patchy lung /f_i elds of respiratory distress syndrome.

Figure 18.3 Double bubble in duodenal atresia (gastric and /f_i rst part of the duodenum). Note the umbilical cord and clamp in the lower part of the image.

NEWBORN PHYSIOLOGY AND THE PRINCIPLES OF NEONATAL

NEWBORN PHYSIOLOGY AND THE PRINCIPLES OF NEONATAL SURGERY

Neonatal physiology can pose challenges when there is a surgi - cal condition ( Table 18.2 ) . Perioperatively , a newborn needing - ventilation is best managed in a surgical unit co-located with a medical neonatal intensive care unit (NICU). Some babies are transferred many miles between units. If a neonatal surgical transfer team is not available, accompanying clinicians should be reminded that neonates with bowel obstruction need a nasogastric tube to decompress the stomach; this should be left on free drainage and regularly aspirated. Neonates, especially the premature, lose heat and fluid rap - idly , have poor nutritional reserves, are susceptible to infection h eart and have an immature blood-brain barrier. Surgery should be e ffi ciently performed in a warm environment with gentle tissue handling and broad-spectrum antibiotic cov er. The liver can - be fragile, and blood should be available for laparotomies. Sick babies with NEC or an acute volvulus may be best operated - upon at the cot side in a NICU. In an unstable infant with an acute abdomen, damage control principles should be applied with a second-look laparotomy and definitive repair following stabilisation. Electrocautery is delivered on the lowest working setting. Avoid the Trendelenburg position in the preterm when placing a diathermy pad as elevation risks intraventricular haemorrhages. A laparotomy may be performed through a right-sided transverse muscle-cutting incision just above the umbilicus, taking care to avoid the large, fragile liver, or through a mid - line longitudinal incision, avoiding the bladder, which rises into the abdomen. A muscle-sparing symmetrical supraumbilical - incision or an omega incision are options with the recti pulled laterally . A left-sided diaphragmatic hernia can be approached through a subcostal incision. The umbilical vein remains patent for many days and is divided between ligatures if the midline is crossed. Gastrointestinal surgery may involve bowel resection with a single layer interrupted or continuously sutured anas tomosis or formation of a temporary spouted enterostomy . In stable, well babies, laparoscopy is suitable for some procedures (e.g. duodenal atresia repair , malrotation without acute volvu lus, excision of large ovarian cysts). Central venous access is needed for parenteral nutrition (PN) if enteral feeds are likely to be delayed. Harald Hirschsprung , 1830–1916, fi rst Danish paediatrician, considered Hirschsprung’s disease to be developmental.

Associations illustrating the need for screening Primary abnormality Incidence/ 100 /uni00A0 000 live births Oesophageal atresia/ 24 VACTERL, CHARGE, aneuploidy/other gene defects, duodenal atresia, anorectal tracheoesophageal /f_i stula malformations, tracheomalacia, gastroesophageal re /f_l ux Duodenal atresia 13 Trisomy 21, other intestinal atresias, oesophageal atresia/tracheoesophageal /f_i stula, intestinal malrotation Intestinal atresias 10 Cystic /f_i brosis, other atresias Anorectal malformations 26 Oesophageal atresia/tracheoesophageal /f_i stula, VACTERL, Trisomy 21 Hirschsprung’s disease 14 Trisomy 21, other chromosomal defects, familial Structural anomalies atresias/anorectal malformations Syndromic hypoventilation syndrome (Ondine’s curse) Biliary atresia 3 Biliary atresia splenic malformation (BASM) syndrome: polysplenia, vascular and cardiac anomalies, defects of situs Cytomegalovirus Gastroschisis 20 Intestinal atresias, undescended testes Exomphalos (major and minor) 12 Aneuploidy, chromosomal anomalies, cardiac defects, Beckwith–Wiedemann syndrome Congenital diaphragmatic hernia 20 Aneuploidy, chromosomal anomalies, CHARGE, malrotation. Syndromic Congenital pulmonary airway 10 Vertebral/chest wall deformities, intestinal duplications, cardiac anomalies, congenital malformations (CPAM) diaphragmatic hernia CHARGE, c oloboma, h eart defects, choanal a tresia, growth r etardation, c ardiac, t racheoesophageal, r enal and l imb anomalies. Type A Type B Type C : Cardiac, craniofacial, cleft palate, polydactyly, intestinal : Multiple endocrine neoplasia, Waardenburg–Shah syndrome, congenital g enital anomalies and e ar anomalies; VACTERL, v ertebral, a norectal,

NEWBORN PHYSIOLOGY AND THE PRINCIPLES OF NEONATAL SURGERY

NEWBORN PHYSIOLOGY AND THE PRINCIPLES OF NEONATAL SURGERY

Neonatal physiology can pose challenges when there is a surgi - cal condition ( Table 18.2 ) . Perioperatively , a newborn needing - ventilation is best managed in a surgical unit co-located with a medical neonatal intensive care unit (NICU). Some babies are transferred many miles between units. If a neonatal surgical transfer team is not available, accompanying clinicians should be reminded that neonates with bowel obstruction need a nasogastric tube to decompress the stomach; this should be left on free drainage and regularly aspirated. Neonates, especially the premature, lose heat and fluid rap - idly , have poor nutritional reserves, are susceptible to infection h eart and have an immature blood-brain barrier. Surgery should be e ffi ciently performed in a warm environment with gentle tissue handling and broad-spectrum antibiotic cov er. The liver can - be fragile, and blood should be available for laparotomies. Sick babies with NEC or an acute volvulus may be best operated - upon at the cot side in a NICU. In an unstable infant with an acute abdomen, damage control principles should be applied with a second-look laparotomy and definitive repair following stabilisation. Electrocautery is delivered on the lowest working setting. Avoid the Trendelenburg position in the preterm when placing a diathermy pad as elevation risks intraventricular haemorrhages. A laparotomy may be performed through a right-sided transverse muscle-cutting incision just above the umbilicus, taking care to avoid the large, fragile liver, or through a mid - line longitudinal incision, avoiding the bladder, which rises into the abdomen. A muscle-sparing symmetrical supraumbilical - incision or an omega incision are options with the recti pulled laterally . A left-sided diaphragmatic hernia can be approached through a subcostal incision. The umbilical vein remains patent for many days and is divided between ligatures if the midline is crossed. Gastrointestinal surgery may involve bowel resection with a single layer interrupted or continuously sutured anas tomosis or formation of a temporary spouted enterostomy . In stable, well babies, laparoscopy is suitable for some procedures (e.g. duodenal atresia repair , malrotation without acute volvu lus, excision of large ovarian cysts). Central venous access is needed for parenteral nutrition (PN) if enteral feeds are likely to be delayed. Harald Hirschsprung , 1830–1916, fi rst Danish paediatrician, considered Hirschsprung’s disease to be developmental.

Associations illustrating the need for screening Primary abnormality Incidence/ 100 /uni00A0 000 live births Oesophageal atresia/ 24 VACTERL, CHARGE, aneuploidy/other gene defects, duodenal atresia, anorectal tracheoesophageal /f_i stula malformations, tracheomalacia, gastroesophageal re /f_l ux Duodenal atresia 13 Trisomy 21, other intestinal atresias, oesophageal atresia/tracheoesophageal /f_i stula, intestinal malrotation Intestinal atresias 10 Cystic /f_i brosis, other atresias Anorectal malformations 26 Oesophageal atresia/tracheoesophageal /f_i stula, VACTERL, Trisomy 21 Hirschsprung’s disease 14 Trisomy 21, other chromosomal defects, familial Structural anomalies atresias/anorectal malformations Syndromic hypoventilation syndrome (Ondine’s curse) Biliary atresia 3 Biliary atresia splenic malformation (BASM) syndrome: polysplenia, vascular and cardiac anomalies, defects of situs Cytomegalovirus Gastroschisis 20 Intestinal atresias, undescended testes Exomphalos (major and minor) 12 Aneuploidy, chromosomal anomalies, cardiac defects, Beckwith–Wiedemann syndrome Congenital diaphragmatic hernia 20 Aneuploidy, chromosomal anomalies, CHARGE, malrotation. Syndromic Congenital pulmonary airway 10 Vertebral/chest wall deformities, intestinal duplications, cardiac anomalies, congenital malformations (CPAM) diaphragmatic hernia CHARGE, c oloboma, h eart defects, choanal a tresia, growth r etardation, c ardiac, t racheoesophageal, r enal and l imb anomalies. Type A Type B Type C : Cardiac, craniofacial, cleft palate, polydactyly, intestinal : Multiple endocrine neoplasia, Waardenburg–Shah syndrome, congenital g enital anomalies and e ar anomalies; VACTERL, v ertebral, a norectal,

NEWBORN PHYSIOLOGY AND THE PRINCIPLES OF NEONATAL

NEWBORN PHYSIOLOGY AND THE PRINCIPLES OF NEONATAL SURGERY

Neonatal physiology can pose challenges when there is a surgi - cal condition ( Table 18.2 ) . Perioperatively , a newborn needing - ventilation is best managed in a surgical unit co-located with a medical neonatal intensive care unit (NICU). Some babies are transferred many miles between units. If a neonatal surgical transfer team is not available, accompanying clinicians should be reminded that neonates with bowel obstruction need a nasogastric tube to decompress the stomach; this should be left on free drainage and regularly aspirated. Neonates, especially the premature, lose heat and fluid rap - idly , have poor nutritional reserves, are susceptible to infection h eart and have an immature blood-brain barrier. Surgery should be e ffi ciently performed in a warm environment with gentle tissue handling and broad-spectrum antibiotic cov er. The liver can - be fragile, and blood should be available for laparotomies. Sick babies with NEC or an acute volvulus may be best operated - upon at the cot side in a NICU. In an unstable infant with an acute abdomen, damage control principles should be applied with a second-look laparotomy and definitive repair following stabilisation. Electrocautery is delivered on the lowest working setting. Avoid the Trendelenburg position in the preterm when placing a diathermy pad as elevation risks intraventricular haemorrhages. A laparotomy may be performed through a right-sided transverse muscle-cutting incision just above the umbilicus, taking care to avoid the large, fragile liver, or through a mid - line longitudinal incision, avoiding the bladder, which rises into the abdomen. A muscle-sparing symmetrical supraumbilical - incision or an omega incision are options with the recti pulled laterally . A left-sided diaphragmatic hernia can be approached through a subcostal incision. The umbilical vein remains patent for many days and is divided between ligatures if the midline is crossed. Gastrointestinal surgery may involve bowel resection with a single layer interrupted or continuously sutured anas tomosis or formation of a temporary spouted enterostomy . In stable, well babies, laparoscopy is suitable for some procedures (e.g. duodenal atresia repair , malrotation without acute volvu lus, excision of large ovarian cysts). Central venous access is needed for parenteral nutrition (PN) if enteral feeds are likely to be delayed. Harald Hirschsprung , 1830–1916, fi rst Danish paediatrician, considered Hirschsprung’s disease to be developmental.

Associations illustrating the need for screening Primary abnormality Incidence/ 100 /uni00A0 000 live births Oesophageal atresia/ 24 VACTERL, CHARGE, aneuploidy/other gene defects, duodenal atresia, anorectal tracheoesophageal /f_i stula malformations, tracheomalacia, gastroesophageal re /f_l ux Duodenal atresia 13 Trisomy 21, other intestinal atresias, oesophageal atresia/tracheoesophageal /f_i stula, intestinal malrotation Intestinal atresias 10 Cystic /f_i brosis, other atresias Anorectal malformations 26 Oesophageal atresia/tracheoesophageal /f_i stula, VACTERL, Trisomy 21 Hirschsprung’s disease 14 Trisomy 21, other chromosomal defects, familial Structural anomalies atresias/anorectal malformations Syndromic hypoventilation syndrome (Ondine’s curse) Biliary atresia 3 Biliary atresia splenic malformation (BASM) syndrome: polysplenia, vascular and cardiac anomalies, defects of situs Cytomegalovirus Gastroschisis 20 Intestinal atresias, undescended testes Exomphalos (major and minor) 12 Aneuploidy, chromosomal anomalies, cardiac defects, Beckwith–Wiedemann syndrome Congenital diaphragmatic hernia 20 Aneuploidy, chromosomal anomalies, CHARGE, malrotation. Syndromic Congenital pulmonary airway 10 Vertebral/chest wall deformities, intestinal duplications, cardiac anomalies, congenital malformations (CPAM) diaphragmatic hernia CHARGE, c oloboma, h eart defects, choanal a tresia, growth r etardation, c ardiac, t racheoesophageal, r enal and l imb anomalies. Type A Type B Type C : Cardiac, craniofacial, cleft palate, polydactyly, intestinal : Multiple endocrine neoplasia, Waardenburg–Shah syndrome, congenital g enital anomalies and e ar anomalies; VACTERL, v ertebral, a norectal,

Necrotising enterocolitis

Necrotising enterocolitis

NEC is a patchy haemorrhagic enteritis seen in about 10% of preterm babies on NICUs; all are more than a few days old and have been fed. NEC is less common in breastfed than in formula-fed babies. NEC commonly involves the colon and terminal ileum, less commonly the jejunum and rarely the duodenum. Mild NEC presents with feed intolerance, bilious aspirates, distension and rectal bleeding /uni00A0 – /uni00A0 gut rest, antibiotics and PN may be su ffi cient treatment. If tenderness and signs of sepsis are present surgical intervention may be needed, especially if there is a deterioration needing mechanical venti lation and inotropes. Some can be observed closely for a short time. A laparotomy is needed if there is no improvement, a persistent mass, worsening obstruction, perforation, or further e a rapidly progressing deterioration. The sickest babies hav multiorgan failure with a discoloured abdomen ( Figure 18.10 and a mortality of around 30%. Radiological signs include pneumatosis intestinalis ( Figure 18.11 ), gas in the portal vein and if perforated, a pneumoperitoneum. A peritoneal drain can occasionally be su ffi cient, but definitive surgery is usually needed. At laparotomy ( Figure 18.12 ), bowel resection and anastomosis or a defunctioning stoma are options. In the sick est babies, dead intestine is removed, open bowel ends closed (clip and drop), and the abdomen left open (laparostomy) with a vacuum dressing applied on low suction. Definitive surgery follows stabilisation. Survivors with >40 /uni00A0 cm of small intes ver a few months, but others may need tine usually adapt o prolonged admissions or home PN.

Figure 18.9 Water-soluble contrast in meconium ileus showing a microcolon.

Necrotising enterocolitis

NEC is a patchy haemorrhagic enteritis seen in about 10% of preterm babies on NICUs; all are more than a few days old and have been fed. NEC is less common in breastfed than in formula-fed babies. NEC commonly involves the colon and terminal ileum, less commonly the jejunum and rarely the duodenum. Mild NEC presents with feed intolerance, bilious aspirates, distension and rectal bleeding /uni00A0 – /uni00A0 gut rest, antibiotics and PN may be su ffi cient treatment. If tenderness and signs of sepsis are present surgical intervention may be needed, especially if there is a deterioration needing mechanical venti lation and inotropes. Some can be observed closely for a short time. A laparotomy is needed if there is no improvement, a persistent mass, worsening obstruction, perforation, or further e a rapidly progressing deterioration. The sickest babies hav multiorgan failure with a discoloured abdomen ( Figure 18.10 and a mortality of around 30%. Radiological signs include pneumatosis intestinalis ( Figure 18.11 ), gas in the portal vein and if perforated, a pneumoperitoneum. A peritoneal drain can occasionally be su ffi cient, but definitive surgery is usually needed. At laparotomy ( Figure 18.12 ), bowel resection and anastomosis or a defunctioning stoma are options. In the sick est babies, dead intestine is removed, open bowel ends closed (clip and drop), and the abdomen left open (laparostomy) with a vacuum dressing applied on low suction. Definitive surgery follows stabilisation. Survivors with >40 /uni00A0 cm of small intes ver a few months, but others may need tine usually adapt o prolonged admissions or home PN.

Figure 18.9 Water-soluble contrast in meconium ileus showing a microcolon.

Necrotising enterocolitis

NEC is a patchy haemorrhagic enteritis seen in about 10% of preterm babies on NICUs; all are more than a few days old and have been fed. NEC is less common in breastfed than in formula-fed babies. NEC commonly involves the colon and terminal ileum, less commonly the jejunum and rarely the duodenum. Mild NEC presents with feed intolerance, bilious aspirates, distension and rectal bleeding /uni00A0 – /uni00A0 gut rest, antibiotics and PN may be su ffi cient treatment. If tenderness and signs of sepsis are present surgical intervention may be needed, especially if there is a deterioration needing mechanical venti lation and inotropes. Some can be observed closely for a short time. A laparotomy is needed if there is no improvement, a persistent mass, worsening obstruction, perforation, or further e a rapidly progressing deterioration. The sickest babies hav multiorgan failure with a discoloured abdomen ( Figure 18.10 and a mortality of around 30%. Radiological signs include pneumatosis intestinalis ( Figure 18.11 ), gas in the portal vein and if perforated, a pneumoperitoneum. A peritoneal drain can occasionally be su ffi cient, but definitive surgery is usually needed. At laparotomy ( Figure 18.12 ), bowel resection and anastomosis or a defunctioning stoma are options. In the sick est babies, dead intestine is removed, open bowel ends closed (clip and drop), and the abdomen left open (laparostomy) with a vacuum dressing applied on low suction. Definitive surgery follows stabilisation. Survivors with >40 /uni00A0 cm of small intes ver a few months, but others may need tine usually adapt o prolonged admissions or home PN.

Figure 18.9 Water-soluble contrast in meconium ileus showing a microcolon.

Pulmonary airway malformations

Pulmonary airway malformations

There are three groups: congenital cystic adenomatoid malfor - mations (CCAMs), bronchopulmonary sequestrations (BPSs) - and congenital lobar emphysema (CLE). The sequestrations may be intralobar or extralobar and are accompanied by an aberrant arterial supply from a major vessel, usually the aorta. Hybrid lesions have both a CCAM and a BPS. Some malf orma - tions cause respiratory or cardiovascular compromise at birth and require excision, but most are asymptomatic and imaging may be delayed for several months. Cross-sectional computed tomy and vasculature. tomography imaging defines the ana Pulmonary airway malformations

There are three groups: congenital cystic adenomatoid malfor - mations (CCAMs), bronchopulmonary sequestrations (BPSs) - and congenital lobar emphysema (CLE). The sequestrations may be intralobar or extralobar and are accompanied by an aberrant arterial supply from a major vessel, usually the aorta. Hybrid lesions have both a CCAM and a BPS. Some malf orma - tions cause respiratory or cardiovascular compromise at birth and require excision, but most are asymptomatic and imaging may be delayed for several months. Cross-sectional computed tomy and vasculature. tomography imaging defines the ana Pulmonary airway malformations

There are three groups: congenital cystic adenomatoid malfor - mations (CCAMs), bronchopulmonary sequestrations (BPSs) - and congenital lobar emphysema (CLE). The sequestrations may be intralobar or extralobar and are accompanied by an aberrant arterial supply from a major vessel, usually the aorta. Hybrid lesions have both a CCAM and a BPS. Some malf orma - tions cause respiratory or cardiovascular compromise at birth and require excision, but most are asymptomatic and imaging may be delayed for several months. Cross-sectional computed tomy and vasculature. tomography imaging defines the ana

Sacrococcygeal teratoma

Sacrococcygeal teratoma

These germ cell tumours arise from the coccyx and are usually diagnosed antenatally . They may have internal and external components or both ( Figure 18.20 ). Most are benign mature teratomas, but some contain immature embryonic elements. Complete excision is usually achieved in the prone position the pelvic floor. If the intra-abdominal component is large or vascular, the median sacral artery can be ligated through the abdomen. Bladder and bowel function are assessed following the pelvic floor repair. α -Fetoprotein levels are measured to detect recurrence.

Figure 18.20 Sacrococcygeal teratoma.

Sacrococcygeal teratoma

These germ cell tumours arise from the coccyx and are usually diagnosed antenatally . They may have internal and external components or both ( Figure 18.20 ). Most are benign mature teratomas, but some contain immature embryonic elements. Complete excision is usually achieved in the prone position the pelvic floor. If the intra-abdominal component is large or vascular, the median sacral artery can be ligated through the abdomen. Bladder and bowel function are assessed following the pelvic floor repair. α -Fetoprotein levels are measured to detect recurrence.

Figure 18.20 Sacrococcygeal teratoma.

Sacrococcygeal teratoma

These germ cell tumours arise from the coccyx and are usually diagnosed antenatally . They may have internal and external components or both ( Figure 18.20 ). Most are benign mature teratomas, but some contain immature embryonic elements. Complete excision is usually achieved in the prone position the pelvic floor. If the intra-abdominal component is large or vascular, the median sacral artery can be ligated through the abdomen. Bladder and bowel function are assessed following the pelvic floor repair. α -Fetoprotein levels are measured to detect recurrence.

Figure 18.20 Sacrococcygeal teratoma.

Small bowel atresias

Small bowel atresias

Small bowel atresias may be isolated ( Figure 18.5 ) or multiple. If seen without other anomalies, they are thought to have been caused by localised vascular events occurring after organogene sis. Rarely multiple intestinal atresias are seen with an immune Abraham Vater , 1684–1751, Professor of Anatomy and Botany , and later of Pathology and Therapeutics, Wittenberg, Germany . Ken Kimura , contemporary , paediatric surgeon, Kobe University , Kobe, Japan, and Iowa City , IA, USA. William E Ladd , 1880–1967, American surgeon, regarded as a founder of paediatric surgery . deficiency related to mutations of the tetratricopeptide gene (TTC7A). A segmental ileal volvulus can cause an atresia; thick meconium in cystic fibrosis is a risk factor. The upstream bowel dilates and becomes dysmotile, while the downstream bowel remains narrow; a primary anastomosis can accommodate up to a 5:1 discrepancy . Resection of the dilated portion is appro - priate if this does not sacrifice too much intestine. Otherwise, a temporary stoma and mucous fistula facilitate a staged repair.

B C D A A D C B (b) A D C B Figure 18.4 (a, b) Duodenal atresia and the incisions used to repair it: a diamond anastomosis is shown.

Small bowel atresias

Small bowel atresias may be isolated ( Figure 18.5 ) or multiple. If seen without other anomalies, they are thought to have been caused by localised vascular events occurring after organogene sis. Rarely multiple intestinal atresias are seen with an immune Abraham Vater , 1684–1751, Professor of Anatomy and Botany , and later of Pathology and Therapeutics, Wittenberg, Germany . Ken Kimura , contemporary , paediatric surgeon, Kobe University , Kobe, Japan, and Iowa City , IA, USA. William E Ladd , 1880–1967, American surgeon, regarded as a founder of paediatric surgery . deficiency related to mutations of the tetratricopeptide gene (TTC7A). A segmental ileal volvulus can cause an atresia; thick meconium in cystic fibrosis is a risk factor. The upstream bowel dilates and becomes dysmotile, while the downstream bowel remains narrow; a primary anastomosis can accommodate up to a 5:1 discrepancy . Resection of the dilated portion is appro - priate if this does not sacrifice too much intestine. Otherwise, a temporary stoma and mucous fistula facilitate a staged repair.

B C D A A D C B (b) A D C B Figure 18.4 (a, b) Duodenal atresia and the incisions used to repair it: a diamond anastomosis is shown.

Small bowel atresias

Small bowel atresias may be isolated ( Figure 18.5 ) or multiple. If seen without other anomalies, they are thought to have been caused by localised vascular events occurring after organogene sis. Rarely multiple intestinal atresias are seen with an immune Abraham Vater , 1684–1751, Professor of Anatomy and Botany , and later of Pathology and Therapeutics, Wittenberg, Germany . Ken Kimura , contemporary , paediatric surgeon, Kobe University , Kobe, Japan, and Iowa City , IA, USA. William E Ladd , 1880–1967, American surgeon, regarded as a founder of paediatric surgery . deficiency related to mutations of the tetratricopeptide gene (TTC7A). A segmental ileal volvulus can cause an atresia; thick meconium in cystic fibrosis is a risk factor. The upstream bowel dilates and becomes dysmotile, while the downstream bowel remains narrow; a primary anastomosis can accommodate up to a 5:1 discrepancy . Resection of the dilated portion is appro - priate if this does not sacrifice too much intestine. Otherwise, a temporary stoma and mucous fistula facilitate a staged repair.

B C D A A D C B (b) A D C B Figure 18.4 (a, b) Duodenal atresia and the incisions used to repair it: a diamond anastomosis is shown.

THORACIC SURGERY Congenital diaphragmatic hernia

THORACIC SURGERY Congenital diaphragmatic hernia

Several genes share roles in diaphragmatic, pulmonary , cardiac and foregut development; consequently , congenital diaphrag matic hernias are associated with lung hypoplasia, pulmonary hypertension, cardiac defects and gastroesophageal reflux, with half having additional anomalies. The pulmonary hypoplasia due to lung compression from herniated abdominal is partly contents (mechanical aetiology) and partly due to genetic causes FOG2, GATA4 ). Commonly , there is a left posterolateral or (e.g. Bochdalek defect, less commonly a ventral or Morgagni defect. Vincent Alexander Bochdalek , 1801–1883, Professor of Anatomy , Prague, Czech Republic. Giovanni Battista Morgagni , 1682–1771, Italian anatomist, the father of modern anatomical pathology . John Bruce Beckwith , 1933, American paediatric pathologist. Hans-Rudolf Wiedemann , 1915-2006, German paediatrician. Both Beckwith and Wiedemann reported cases of the syndrome independently in 1963 and 1964. Neonatal abdominal wall defects /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF The diagnosis is usually made antenatally . A prognosis based on an observed-to-expected lung–head ratio (ultrasound), total fetal lung volume (magnetic resonance imaging) and whether or not the fetal liver is in the chest informs counselling. After birth, intubation, muscle relaxation and gentle ventilation aim to maintain pH, and oxygen saturation so avoiding right-to-left shunting. Permissive hypercapnia and . Pulmonary hypertension high-frequency oscillation may help may lead to vascular shunting, hypoxia, hypercapnia and cardiac dysfunction. Cardiac dysfunction, assessed by echo - cardiography , may respond to nitric oxide, prostaglandin /uni00A0 E1, milrinone and inotropes, but severe dysfunction requires extracorporeal life support (ECLS). Repair is o ff ered if circulation stabilises. Unlike in traumatic the pulmonary diaphragmatic hernias, urgently reducing the bowel does not improve gas exchange in a congenital diaphragmatic hernia. The defect can be approached from the abdomen or the chest, either open or minimally invasively . The defect may be small, needing only a few sutures, or larger, needing a conical Silastic or GOR-TEX patch. A hernial sac may be present, which may be removed or plicated.

Gastroschisis Environmental risk factors (young maternal age, drug use) Bowel complications common (atresia, matting, volvulus) Associated anomalies rare Exomphalos Frequent association with aneuploidies and other genetic syndromes (e.g. Beckwith–Wiedemann) Other structural anomalies common (e.g. cardiac malformations) Large defects may contain liver (exomphalos major) and require delayed or staged closure

THORACIC SURGERY Congenital diaphragmatic hernia

Several genes share roles in diaphragmatic, pulmonary , cardiac and foregut development; consequently , congenital diaphrag matic hernias are associated with lung hypoplasia, pulmonary hypertension, cardiac defects and gastroesophageal reflux, with half having additional anomalies. The pulmonary hypoplasia due to lung compression from herniated abdominal is partly contents (mechanical aetiology) and partly due to genetic causes FOG2, GATA4 ). Commonly , there is a left posterolateral or (e.g. Bochdalek defect, less commonly a ventral or Morgagni defect. Vincent Alexander Bochdalek , 1801–1883, Professor of Anatomy , Prague, Czech Republic. Giovanni Battista Morgagni , 1682–1771, Italian anatomist, the father of modern anatomical pathology . John Bruce Beckwith , 1933, American paediatric pathologist. Hans-Rudolf Wiedemann , 1915-2006, German paediatrician. Both Beckwith and Wiedemann reported cases of the syndrome independently in 1963 and 1964. Neonatal abdominal wall defects /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF The diagnosis is usually made antenatally . A prognosis based on an observed-to-expected lung–head ratio (ultrasound), total fetal lung volume (magnetic resonance imaging) and whether or not the fetal liver is in the chest informs counselling. After birth, intubation, muscle relaxation and gentle ventilation aim to maintain pH, and oxygen saturation so avoiding right-to-left shunting. Permissive hypercapnia and . Pulmonary hypertension high-frequency oscillation may help may lead to vascular shunting, hypoxia, hypercapnia and cardiac dysfunction. Cardiac dysfunction, assessed by echo - cardiography , may respond to nitric oxide, prostaglandin /uni00A0 E1, milrinone and inotropes, but severe dysfunction requires extracorporeal life support (ECLS). Repair is o ff ered if circulation stabilises. Unlike in traumatic the pulmonary diaphragmatic hernias, urgently reducing the bowel does not improve gas exchange in a congenital diaphragmatic hernia. The defect can be approached from the abdomen or the chest, either open or minimally invasively . The defect may be small, needing only a few sutures, or larger, needing a conical Silastic or GOR-TEX patch. A hernial sac may be present, which may be removed or plicated.

Gastroschisis Environmental risk factors (young maternal age, drug use) Bowel complications common (atresia, matting, volvulus) Associated anomalies rare Exomphalos Frequent association with aneuploidies and other genetic syndromes (e.g. Beckwith–Wiedemann) Other structural anomalies common (e.g. cardiac malformations) Large defects may contain liver (exomphalos major) and require delayed or staged closure

THORACIC SURGERY Congenital diaphragmatic hernia

Several genes share roles in diaphragmatic, pulmonary , cardiac and foregut development; consequently , congenital diaphrag matic hernias are associated with lung hypoplasia, pulmonary hypertension, cardiac defects and gastroesophageal reflux, with half having additional anomalies. The pulmonary hypoplasia due to lung compression from herniated abdominal is partly contents (mechanical aetiology) and partly due to genetic causes FOG2, GATA4 ). Commonly , there is a left posterolateral or (e.g. Bochdalek defect, less commonly a ventral or Morgagni defect. Vincent Alexander Bochdalek , 1801–1883, Professor of Anatomy , Prague, Czech Republic. Giovanni Battista Morgagni , 1682–1771, Italian anatomist, the father of modern anatomical pathology . John Bruce Beckwith , 1933, American paediatric pathologist. Hans-Rudolf Wiedemann , 1915-2006, German paediatrician. Both Beckwith and Wiedemann reported cases of the syndrome independently in 1963 and 1964. Neonatal abdominal wall defects /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF The diagnosis is usually made antenatally . A prognosis based on an observed-to-expected lung–head ratio (ultrasound), total fetal lung volume (magnetic resonance imaging) and whether or not the fetal liver is in the chest informs counselling. After birth, intubation, muscle relaxation and gentle ventilation aim to maintain pH, and oxygen saturation so avoiding right-to-left shunting. Permissive hypercapnia and . Pulmonary hypertension high-frequency oscillation may help may lead to vascular shunting, hypoxia, hypercapnia and cardiac dysfunction. Cardiac dysfunction, assessed by echo - cardiography , may respond to nitric oxide, prostaglandin /uni00A0 E1, milrinone and inotropes, but severe dysfunction requires extracorporeal life support (ECLS). Repair is o ff ered if circulation stabilises. Unlike in traumatic the pulmonary diaphragmatic hernias, urgently reducing the bowel does not improve gas exchange in a congenital diaphragmatic hernia. The defect can be approached from the abdomen or the chest, either open or minimally invasively . The defect may be small, needing only a few sutures, or larger, needing a conical Silastic or GOR-TEX patch. A hernial sac may be present, which may be removed or plicated.

Gastroschisis Environmental risk factors (young maternal age, drug use) Bowel complications common (atresia, matting, volvulus) Associated anomalies rare Exomphalos Frequent association with aneuploidies and other genetic syndromes (e.g. Beckwith–Wiedemann) Other structural anomalies common (e.g. cardiac malformations) Large defects may contain liver (exomphalos major) and require delayed or staged closure

WHY ADUL T SURGEONS NEED AN OVERVIEW OF NEONATAL S

WHY ADUL T SURGEONS NEED AN OVERVIEW OF NEONATAL SURGERY

Rarely , malrotation, congenital diaphragmatic hernias and lung malformations described in this chapter may present for the first time in adulthood. More commonly , adults who had neonatal surgery may present with incidental or related pathology . Children who had anorectal malformations or Hirschsprung’s disease need colorectal follow-up, with girls also needing a gynaecologist and obstetrician who understands the pathology; those with oesophageal atresia/tracheoesophageal fistula need an upper gastrointestinal surgeon. WHY ADUL T SURGEONS NEED AN OVERVIEW OF NEONATAL SURGERY

Rarely , malrotation, congenital diaphragmatic hernias and lung malformations described in this chapter may present for the first time in adulthood. More commonly , adults who had neonatal surgery may present with incidental or related pathology . Children who had anorectal malformations or Hirschsprung’s disease need colorectal follow-up, with girls also needing a gynaecologist and obstetrician who understands the pathology; those with oesophageal atresia/tracheoesophageal fistula need an upper gastrointestinal surgeon.

WHY ADUL T SURGEONS NEED AN OVERVIEW OF NEONATAL SURGERY

WHY ADUL T SURGEONS NEED AN OVERVIEW OF NEONATAL SURGERY

Rarely , malrotation, congenital diaphragmatic hernias and lung malformations described in this chapter may present for the first time in adulthood. More commonly , adults who had neonatal surgery may present with incidental or related pathology . Children who had anorectal malformations or Hirschsprung’s disease need colorectal follow-up, with girls also needing a gynaecologist and obstetrician who understands the pathology; those with oesophageal atresia/tracheoesophageal fistula need an upper gastrointestinal surgeon.