Acta Prediatr Scand 80: 1233-1 234, 199 1
CASE REPORT Intrauterine lntussusception and Ileal Atresia Presenting as Acute Perforation S. B. KELLY and M. P. SINGH From the Paediatric Surgery Unit, Royal Postgraduate Medical School, Hammersmith Hospital, London, U.K.
ABSTRACT. Kelly, S. B. and Singh, M. P. (Paediatric Surgery Unit, Royal Postgraduate Medical School, Hammersmith Hospital, London, U.K.). Intrauterine intussusception and ileal atresia presenting as acute perforation. Acta Paediatr Scand 80: 1233,1991. A case is reported of a girl born by caesarean section at 28 weeks gestation who developed an acute perforation of the ileum due to intussusception 11 days after birth. This was treated successfully by resection of the affected segment of ileum and by performing a temporary ileostomy followed by end-to-end anastomosis. She made a complete recovery. This case also demonstrates the difficulty of early diagnosis of intussusception in the neonatal period. Key words: intrauterine intussusception, perforation.
Intussusception is rare under the age of three months, with an incidence of 0.3%in the neonatal period (1). It is exceedingly uncommon for intussusception to present in the first few days of life, when it is considered to have occurred in utero. To our knowledge, this is only the third published case report of intrauterine intussusception presenting as acute perforation (2, 3). CASE HISTORY A girl weighing 1 500 g was delivered by caesarean section at 28 weeks gestation to a 19 year old mother. The baby was cyanosed at birth. One minute after birth, an endotracheal tube was inserted and mechanical ventilation was commenced. Following this, she improved rapidly and showed signs of spontaneous respiratory effort from 1.5 min. The infant's apgar scores were six at 1 and nine at 5 min, respectively. Intravenous fluid were commenced and an umbilical catheter was inserted. Chest X-ray revealed a ground-glass appearance and a diagnosis of Idiopathic Respiratory Distress Syndrome was made. Culture of a vaginal swab from the mother revealed Gardnerella infection and although no organisms were grown from swabs and blood cultures from the baby, treatment was commenced prophylactically with Piperacillin, Gentamycin and Metronidazole. O n day one, the mean arterial blood pressure fell below 30 mmHg and a dopamine infusion was commenced. O n day four, abdominal distension and bilious vomiting were noted and an abdominal Xray revealed distended loops of bowel. Total parenteral nutrition was commenced. By day six, she had passed a small amount of meconium. Following this, she developed a temperature of 38°C and a leucocytosis of 31.8x1O9/1. O n day 10, she was intubated again and ventilated because of respiratory acidosis associated with generalised deterioration. By day 11, her abdomen was more distended and there was quite marked abdominal tenderness on palpation accompanied by guarding but no rebound tenderness. No bowel sounds were audible. Abdominal X-rays revealed grossly distended loops of bowel. O n the lateral decubitus film, free gas was noted in the peritoneal cavity. A diagnosis of intestinal perforation was made and she was taken t o the operating theatre. At laparotomy, a congenital intussusception was found in the midileum and in addition, there were two perforations, one at the base of the intussusceptum and the other at the tip of
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Acta Paediatr Scand 80
the intussuscipiens. The intussusception was reduced, revealing an atretic segment of ileum, which was excised and both ends were brought out as ileostomies. Eighteen days after the initial operation, continuity of the gut was restored by end-to-end anastomosis over a transanastomotic stent which was brought out through the skin. Thirteen days later, a tubogram was performed and this demonstrated no leak from the site of the anastomosis. Following this, the tube was removed. Histological examination of the resected segment of bowel revealed full thickness necrosis of the ileum due to intussusception.
DISCUSSION Our patient was initially thought to have necrotizing enterocolitis (NEC) because of multiple perinatal risk factors, including prematurity, low birthweight, low initial Apgar score and respiratory distress requiring ventilatory support (4).She also had some important clinical findings compatible with NEC, including abdominal distention, and bile stained vomiting. The clinical course evolved gradually with increasing abdominal pain and distension accompanied by increasing evidence of small bowel obstruction on abdominal X-rays. There was no sign of pneumatosis intestinalis, the hallmark of NEC. However, on day 11, a pneumoperitoneum became evident on the abdominal films. It is possible that the ileal atresia could have been responsible for the abdominal symptoms in the beginning and that the intussusception developed at a later stage. In retrospect, knowing the operative findings, a barium enema may have indicated the diagnosis at an earlier stage and might have led to operation before intestinal perforat ion. At laparotomy, if simple reduction fails or there is any doubt about the viability of the gut, a resection with primary end-to-end anastomosis intussusception should be resected. End to end anastomosis should be performed to restore continuity, or if it is felt that a primary anastomosis is too risky as in this case, then a defunctioning ileostomy can be performed initially followed by re-anastomosis at a later date. REFERENCES 1. 'Pavri DR, Marshall DG, Armstrong RF, Gorodzinsky FP. Intrauterine intussusception: Case report and literature review. Can J Surg 1983; 26: 376-78. 2. Reed DN, Polley TZ, Rees MA. Jejunal atresia secondary to intrauterine intussusception, presenting as acute perforation. Can J Surg 1987; 30: 203-04. 3. Nixon HH. Intestinal obstruction in the newborn. Arch Dis Child 1955; 30: 13-22. 4. Stine MJ, Harris H. Intussusception in a premature infant simulating neonatal necrotizing enterocolitis. Am J Dis Child 1982; 136: 76-77.
(M. P. S.) Department of Surgery Royal Postgraduate Medical School Hammersmith Hospital Du Cane Road London W12 OHS U.K.
CASE REPORT Intrauterine lntussusception and Ileal Atresia Presenting as Acute Perforation S. B. KELLY and M. P. SINGH From the Paediatric Surgery Unit, Royal Postgraduate Medical School, Hammersmith Hospital, London, U.K.
ABSTRACT. Kelly, S. B. and Singh, M. P. (Paediatric Surgery Unit, Royal Postgraduate Medical School, Hammersmith Hospital, London, U.K.). Intrauterine intussusception and ileal atresia presenting as acute perforation. Acta Paediatr Scand 80: 1233,1991. A case is reported of a girl born by caesarean section at 28 weeks gestation who developed an acute perforation of the ileum due to intussusception 11 days after birth. This was treated successfully by resection of the affected segment of ileum and by performing a temporary ileostomy followed by end-to-end anastomosis. She made a complete recovery. This case also demonstrates the difficulty of early diagnosis of intussusception in the neonatal period. Key words: intrauterine intussusception, perforation.
Intussusception is rare under the age of three months, with an incidence of 0.3%in the neonatal period (1). It is exceedingly uncommon for intussusception to present in the first few days of life, when it is considered to have occurred in utero. To our knowledge, this is only the third published case report of intrauterine intussusception presenting as acute perforation (2, 3). CASE HISTORY A girl weighing 1 500 g was delivered by caesarean section at 28 weeks gestation to a 19 year old mother. The baby was cyanosed at birth. One minute after birth, an endotracheal tube was inserted and mechanical ventilation was commenced. Following this, she improved rapidly and showed signs of spontaneous respiratory effort from 1.5 min. The infant's apgar scores were six at 1 and nine at 5 min, respectively. Intravenous fluid were commenced and an umbilical catheter was inserted. Chest X-ray revealed a ground-glass appearance and a diagnosis of Idiopathic Respiratory Distress Syndrome was made. Culture of a vaginal swab from the mother revealed Gardnerella infection and although no organisms were grown from swabs and blood cultures from the baby, treatment was commenced prophylactically with Piperacillin, Gentamycin and Metronidazole. O n day one, the mean arterial blood pressure fell below 30 mmHg and a dopamine infusion was commenced. O n day four, abdominal distension and bilious vomiting were noted and an abdominal Xray revealed distended loops of bowel. Total parenteral nutrition was commenced. By day six, she had passed a small amount of meconium. Following this, she developed a temperature of 38°C and a leucocytosis of 31.8x1O9/1. O n day 10, she was intubated again and ventilated because of respiratory acidosis associated with generalised deterioration. By day 11, her abdomen was more distended and there was quite marked abdominal tenderness on palpation accompanied by guarding but no rebound tenderness. No bowel sounds were audible. Abdominal X-rays revealed grossly distended loops of bowel. O n the lateral decubitus film, free gas was noted in the peritoneal cavity. A diagnosis of intestinal perforation was made and she was taken t o the operating theatre. At laparotomy, a congenital intussusception was found in the midileum and in addition, there were two perforations, one at the base of the intussusceptum and the other at the tip of
1234 S. B. KeNv and M . P.Sinnh
Acta Paediatr Scand 80
the intussuscipiens. The intussusception was reduced, revealing an atretic segment of ileum, which was excised and both ends were brought out as ileostomies. Eighteen days after the initial operation, continuity of the gut was restored by end-to-end anastomosis over a transanastomotic stent which was brought out through the skin. Thirteen days later, a tubogram was performed and this demonstrated no leak from the site of the anastomosis. Following this, the tube was removed. Histological examination of the resected segment of bowel revealed full thickness necrosis of the ileum due to intussusception.
DISCUSSION Our patient was initially thought to have necrotizing enterocolitis (NEC) because of multiple perinatal risk factors, including prematurity, low birthweight, low initial Apgar score and respiratory distress requiring ventilatory support (4).She also had some important clinical findings compatible with NEC, including abdominal distention, and bile stained vomiting. The clinical course evolved gradually with increasing abdominal pain and distension accompanied by increasing evidence of small bowel obstruction on abdominal X-rays. There was no sign of pneumatosis intestinalis, the hallmark of NEC. However, on day 11, a pneumoperitoneum became evident on the abdominal films. It is possible that the ileal atresia could have been responsible for the abdominal symptoms in the beginning and that the intussusception developed at a later stage. In retrospect, knowing the operative findings, a barium enema may have indicated the diagnosis at an earlier stage and might have led to operation before intestinal perforat ion. At laparotomy, if simple reduction fails or there is any doubt about the viability of the gut, a resection with primary end-to-end anastomosis intussusception should be resected. End to end anastomosis should be performed to restore continuity, or if it is felt that a primary anastomosis is too risky as in this case, then a defunctioning ileostomy can be performed initially followed by re-anastomosis at a later date. REFERENCES 1. 'Pavri DR, Marshall DG, Armstrong RF, Gorodzinsky FP. Intrauterine intussusception: Case report and literature review. Can J Surg 1983; 26: 376-78. 2. Reed DN, Polley TZ, Rees MA. Jejunal atresia secondary to intrauterine intussusception, presenting as acute perforation. Can J Surg 1987; 30: 203-04. 3. Nixon HH. Intestinal obstruction in the newborn. Arch Dis Child 1955; 30: 13-22. 4. Stine MJ, Harris H. Intussusception in a premature infant simulating neonatal necrotizing enterocolitis. Am J Dis Child 1982; 136: 76-77.
(M. P. S.) Department of Surgery Royal Postgraduate Medical School Hammersmith Hospital Du Cane Road London W12 OHS U.K.
