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Nigeria's free digital learning hub. Videos, lecture notes, practice questions and leaderboards aligned with CCMAS, NBTE, NCCE, WAEC, NECO, IJMB and JAMB curricula.
This is the complete lecture note for SUG403 Paediatric Surgery. You can read it online below, open it in the full viewer, or download the PDF for offline study. It pairs with the course's video lectures, so you can read and watch together.
la-NET | ONLINE LEARNING VIDEO PLATFORM FOR POST-SECONDARY & HIGHER INSTITUTION STUDENTS
la-NET | ONLINE LEARNING VIDEO PLATFORM FOR POST-SECONDARY & HIGHER INSTITUTION STUDENTS Course code: SUG 403 Course title: Paediatric Surgery Course credit load: 2 Units Series 1: Congenital Gastrointestinal Anomalies • Part 1.1: Abdominal Wall Defects o Sub Part 1.1.1: Exomphalos o Sub Part 1.1.2: Gastroschisis o Sub Part 1.1.3: Management and outcomes • Part 1.2: Intestinal Atresia o Sub Part 1.2.1: Oesophageal atresia and tracheo-oesophageal fistula o Sub Part 1.2.2: Duodenal and jejunoileal atresia o Sub Part 1.2.3: Management of intestinal atresia • Part 1.3: Malrotation and Midgut Volvulus o Sub Part 1.3.1: Embryology and pathophysiology o Sub Part 1.3.2: Clinical features and diagnosis o Sub Part 1.3.3: Management • Part 1.4: Hirschsprung's Disease and Anorectal Malformations o Sub Part 1.4.1: Hirschsprung's disease o Sub Part 1.4.2: Anorectal malformations o Sub Part 1.4.3: Management and complications Introduction
la-NET | ONLINE LEARNING VIDEO PLATFORM FOR POST-SECONDARY & HIGHER INSTITUTION STUDENTS Congenital gastrointestinal anomalies are structural defects present at birth that affect the alimentary tract and abdominal wall. They are among the most challenging conditions in paediatric surgical practice, requiring prompt recognition, careful resuscitation, and timely surgical correction. Many present in the neonatal period and are life-threatening if not identified and managed urgently. This Series covers the major congenital GI anomalies relevant to Nigerian paediatric surgical practice. We begin with abdominal wall defects (exomphalos and gastroschisis), then examine intestinal atresia, including oesophageal atresia and duodenal atresia. We then address malrotation and midgut volvulus, and close with Hirschsprung's disease and anorectal malformations. Series Learning Outcomes Upon completing this Series, you should be able to: • SLO 1.1: identify the clinical features of exomphalos and gastroschisis and describe their management, • SLO 1.2: describe the clinical presentation, diagnosis, and surgical management of oesophageal atresia, duodenal atresia, and jejunoileal atresia, • SLO 1.3: describe the embryological basis, clinical features, and management of malrotation and midgut volvulus, and • SLO 1.4: describe the clinical features, diagnosis, and management of Hirschsprung's disease and anorectal malformations, and state the complications that may arise. 1.1 Abdominal Wall Defects 1.1.1 Exomphalos Exomphalos (omphalocele) is a midline abdominal wall defect at the umbilical ring through which abdominal contents herniate into the base of the umbilical cord, covered by a sac of amnion and peritoneum. The sac may contain small bowel, large bowel, stomach, and liver. It results from failure of the midgut to return to the abdominal cavity during the 10th week of gestation. Exomphalos is classified as minor (sac contains only bowel; defect less than 5 cm) or major (sac contains liver; defect above 5 cm). Associated anomalies are present in up to 70 percent of cases and include cardiac defects (most common), chromosomal abnormalities (trisomy 13, 18, and 21), Beckwith-Wiedemann syndrome (macroglossia, macrosomia, and hypoglycaemia), and neural tube defects. Antenatal
la-NET | ONLINE LEARNING VIDEO PLATFORM FOR POST-SECONDARY & HIGHER INSTITUTION STUDENTS diagnosis by ultrasound is common. At birth, the sac must be kept moist and the baby resuscitated before surgery. Fill in the blank: Exomphalos results from failure of the midgut to return to the abdominal cavity at the ________ week of gestation. Associated anomalies occur in up to ________ percent of cases, the most common being ________ defects. 1.1.2 Gastroschisis Gastroschisis is a full-thickness defect of the abdominal wall, typically to the right of the umbilicus, through which abdominal contents herniate without any covering sac. Unlike exomphalos, the bowel is directly exposed to amniotic fluid, causing a chemical peritonitis that results in a thickened, matted, oedematous, and foreshortened bowel (the peel). The defect is usually small (2 to 4 cm). Gastroschisis is not typically associated with chromosomal anomalies, though prematurity and low birth weight are common. Gastroschisis is a neonatal surgical emergency. The exposed bowel must be covered immediately with a warm saline-soaked gauze wrap and a plastic bag or cling film to prevent heat loss, fluid loss, and further contamination. A nasogastric tube is passed to decompress the stomach. IV fluid resuscitation is essential as fluid losses from the exposed bowel are massive. Fill in the blank: Gastroschisis is located to the ________ of the umbilicus and has ________ covering sac. The thickened oedematous bowel in gastroschisis is caused by exposure to ________ fluid and is called the peel. 1.1.3 Management and outcomes Surgical repair of exomphalos: small defects are closed primarily. Large defects with liver in the sac may require staged repair: the sac is preserved and used as coverage, or a silo (a synthetic mesh bag sutured to the fascial edges) is created to gradually reduce the contents over 7 to 14 days before fascial closure. Rupture of the exomphalos sac is an emergency requiring immediate surgery. Surgical repair of gastroschisis: primary closure (reduction of all bowel into the abdomen and primary fascial closure in a single operation) is performed if the abdominal domain is adequate and the bowel is not too oedematous. If primary closure is not possible (excessive intra-abdominal pressure would compromise respiration and bowel perfusion), a silo is fashioned and the bowel reduced gradually over several days before closure. Complications include short bowel syndrome (if bowel is resected), prolonged ileus, and sepsis. Survival in centres with adequate neonatal intensive care exceeds 90 percent. Fill in the blank: A ________ is a synthetic mesh bag used to gradually reduce abdominal contents over days before fascial closure in both exomphalos and gastroschisis. The most serious long-term complication of gastroschisis is ________ bowel syndrome if bowel is resected.
la-NET | ONLINE LEARNING VIDEO PLATFORM FOR POST-SECONDARY & HIGHER INSTITUTION STUDENTS Figure 1.1: Comparison of exomphalos and gastroschisis and their management Pilot questions 1.1 1. Distinguish exomphalos from gastroschisis in terms of location, sac, and associated anomalies. (Linked to SLO 1.1) 2. Describe the immediate management of a neonate born with gastroschisis. (Linked to SLO 1.1) 3. Describe the Beckwith-Wiedemann syndrome and state its association with exomphalos. (Linked to SLO 1.1) 4. Describe the use of a silo in the management of abdominal wall defects. (Linked to SLO 1.1) 5. State the complications of gastroschisis repair. (Linked to SLO 1.1) 1.2 Intestinal Atresia 1.2.1 Oesophageal atresia and tracheo-oesophageal fistula Oesophageal atresia (OA) is a congenital interruption of the continuity of the oesophagus. It is classified by the Gross classification into five types. The most common type (Type C: approximately 85 percent) is OA with a distal tracheo-oesophageal fistula (TOF): the upper oesophagus ends in a blind pouch, and the lower oesophagus connects abnormally to the trachea. Type A (pure OA without a fistula: approximately 8 percent) is the next most common.
la-NET | ONLINE LEARNING VIDEO PLATFORM FOR POST-SECONDARY & HIGHER INSTITUTION STUDENTS Associated anomalies are grouped in the VACTERL association (Vertebral, Anorectal, Cardiac, Tracheo-Oesophageal, Renal, and Limb anomalies). Clinical features of OA: the neonate presents with excessive drooling and salivation (accumulation of secretions in the blind upper pouch), choking and cyanosis during feeds (aspiration), and respiratory distress. The diagnosis is suspected when a nasogastric tube cannot be passed beyond 10 to 12 cm from the mouth and is confirmed by CXR showing the tube coiled in the upper oesophageal pouch. The presence of gas in the stomach on CXR indicates a distal TOF (gas entering the stomach via the fistula). Fill in the blank: The most common type of oesophageal atresia is Type C, which consists of OA with a ________ tracheo-oesophageal fistula. A neonate with OA is suspected when a nasogastric tube cannot be passed beyond ________ to 12 cm and the tube is seen ________ in the upper pouch on CXR. 1.2.2 Duodenal and jejunoileal atresia Duodenal atresia is a congenital obstruction of the duodenum, most commonly at or just distal to the ampulla of Vater (post-ampullary: 85 percent). It results from failure of recanalisation of the duodenal lumen during the 8th to 10th week of gestation. There is a strong association with Down syndrome (trisomy 21: present in approximately 30 percent of cases) and with other anomalies (cardiac defects, malrotation, and annular pancreas). The classic radiographic finding is the double bubble sign (air in the stomach and the proximal duodenum with no distal gas) on plain AXR. Jejunoileal atresia results from an intrauterine vascular accident (mesenteric ischaemia causing bowel infarction and resorption) rather than failure of recanalisation. It is classified into four types (I: mucosal web; II: fibrous cord between two blind ends; IIIa: gap with V-shaped mesenteric defect; IIIb: apple-peel or Christmas tree deformity: the most severe, with risk of short bowel syndrome; IV: multiple atresias). Unlike duodenal atresia, jejunoileal atresia is not strongly associated with chromosomal anomalies. Fill in the blank: Duodenal atresia is associated with Down syndrome in approximately ________ percent of cases. The classic X-ray finding is the ________ bubble sign. Jejunoileal atresia results from an intrauterine ________ accident rather than failure of recanalisation. 1.2.3 Management of intestinal atresia Management of OA: preoperative resuscitation (IV fluids, keep the baby warm, elevate the head to reduce aspiration), continuous suctioning of the upper pouch with a
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