Intrauterine
Intussusception
Causing
Intestinal
Atresia
By 0. Adejuyigbe and W.O. Odesanmi l/e-lfe, 0 The patient presented at the age of 2 days with persistent bilious vomiting, failure to pass meconium and urine, and progressive abdominal distension dating from birth, and a clinical diagnosis of intestinal obstruction was made. This was confirmed by plain abdominal radiographs. At laparotomy atresia of the terminal ileum was found. The proximal and distal segments of the bowel ended blindly and there was a V-shaped defect in the adjoining mesentery. The blind ends were resected and an end-toback anastomosis was performed. The lumen of the resected distal segment contained an intussusceptum. 8 1990 by W.B. Saunders Company. INDEX WORDS: Neonatal intestinal obstruction: intrauterine intussusception; intestinal atresia.
I
NTUSSUSCEPTION occuring in intrauterine life is a rarity. To date, less than 30 cases have been adequately documented in the world literature.’ Because of the known tendency of an intussusception to interfere with the blood supply of the involved segment of intestine, intrauterine intassusception had been implicated as a possible cause of congenital intestinal atresia.‘.’ The case of a patient who presented with features of intestinal obstruction and was subsequently found to have intestinal atresia and an intussusception that must have occurred in utero is presented here.
Nigeria
and nasogastric tube suction. Phototherapy was commenced because the serum bilirubin was elevated (7.9 mg%). At laparotomy the findings were congenital atresia of the terminal ileum with the proximal and distal loops ending blindly. The proximal blind loop was grossly distended and of a dark red colour. There was however no evidence of necrosis. There was a small Vshaped defect in the adjoining mesentery with evidenceof mcconium peritonitis in the right lower quadrant of the abdomen. About 12 cm of the proximal lcop and 5 cm of the distal loop were resected and an end-to-back anastomosis was performed to restore gastrointestinal continuity. The child had a smooth postoperative period and was discharged on the 12th postoperative day. He is now 11 months old and his growth and development have been normal.
Pathology Report Examinations of the resected specimens showed gross dilatation of the lumen of the proximal loop while the lumen of the distal loop contained an intussusceptum (Fig 1). Microscopy of the distal bowel loop demonstrated normal intestinal layers with hemorrhagic areas external to the muscular layer. There were also many dilated blood
CASE REPORT Baby A, a boy, was the product of a term pregnancy. His birth weight was 3.5 kg and the l- and Sminute Apgar scores were 10 and 10. He presented at the age of 2 days because of persistent bilious vomiting, failure to pass meconium and urine, all dating from birth, with progressive abdominal distension. There was no history of maternal hydramnios. Physical examination showed a mildly dehydrated term baby with jaundice and no respiratory distress. The abdomen was distended but soft on palpation and apart from the liver that was below the costal margin, no abdominal masses were palpable. The bowel sounds were hyperactive. A plain abdominal x-ray in the supine and erect positions showed marked distension of numerous bowel loops throughout the abdomen with no gas in the pelvis. In the erect position, numerous air-fluid levels were visible in the bowels, the appearance being consistent with a small bowel obstruction. He was started on intravenous fluids
From the Paediatric Surgical Unit, Department of Surgery, and the Department of Morbid Anatomy and Forensic Medicine, University of Ife, Ile-Ife. Nigeria. Address reprint requests to 0. Adejuyigbe. FMCS, Department of Surgery, University of Ife. Ile-Ife. Nigeria. o 1990 by W.B. Saunders Company. 0022-3468/90/2505-oo26$03.00/0
562
Fig 1. (A) Photographof distalbowelloopshowing intussuscep turn within the lumen. (Bl Photograph showing intussuaceptum partially adherent to the outer bowel wall.
hums/
of Pediatric Surgery Vol 25, No 5 (May), 1990:
pp 562-563
NEONATAL INTRAUTERINE
563
INTUSSUSCEPTION
within the muscle layer. In addition, there were areas of mucosal surfaces abutting on each other. These features are consistent with those of intussusception.
vessels
DISCUSSION
Intrauterine bowel intussusception is a rare clinical entity. When intussusception occurs in the fetus and the time elapsed is enough to allow for gangrene and resorption of the intussuscepted portions of the bowel, intestinal atresia will result.lS3 That intestinal atresia may be secondary to prolonged bowel ischemia in utero has long been suspected.’ Laufman et al* demonstrated in 1949 that when isolated sterile devascularized loops of intestine were left in the peritoneal cavity, they converted to fibrous bands or disappeared entirely. This was later con-
firmed by Leuw and Barnard’ in 1955, who were able to produce intestinal atresia in two puppies by ligating the mesenteric vessels supplying a segment of their bowel in utero. Similar results have recently been obtained by Tibboel et a19,” working with the chicken embryo. The case reported here had a V-shaped mesenteric defect between the blindly ending proximal and distal bowel loops. This appearance is similar to those found in experimental animals in which the Mood supply to the bowel had been interfered with in utero. The finding of an intussusceptum in the distal loop suggests that intussusception of the bowel in utero was the primary lesion in this child. The subsequent interference with the blood supply of the intestine then caused the intestinal atresia.
REFERENCES 1. Pavry DR, Marshall DG, Armstrong RF, et al: Intrauterine intussusception: Case report and literature review. Can J Surg 26376378,1983 2. Gherardi GJ, Fisher JH: Atresia of the small intestine produced by intussusception in utero. N Engl J Med 264:229-231,196l 3. Grosveld JL, Clatworthy HW Jr: The nature of ileal atresia due to intrauterine intussusception. Arch Surg 100:714-717,197O 4. Koop CE: Intestinal obstruction in the newborn period, in Levine SZ, Anderson JA, et al (eds): Advances in Pediatrics. Chicago, IL, Year Book, 1953, pp 63-96 5. Leuw JH, Barnard CN: Congenital atresia. Lancet 2:10651067.1955 6. Nixon HH: Intestinal obstruction in the newborn. Arch Dis Child 30:13-22, 1955
7. Ravitch MM: Intussusception, in Paediatric Surgery (ed 3). Ravitch MM, Welch KJ, Benson CD, et al (eds): Chicago, IL, Year Book, 1979, pp 989- 1003 8. Laufman H, Martin WB, Method H, et al: Observations in strangulation obstruction, II. The fate of sterile devascularized intestine in the peritoneal cavity. Arch Surg 59:550-564, 1949 9. Tibboel D, Molenaar JC, Van Nie CJ: New perspective in fetal surgery, the chicken embryo. J Pediatr Surg 14:43&440,1979 10. Tibboel D, Van Nie CJ, Molenaar JC: The effects of temporary general hypoxia and local ischaemia on, the development of the intestines: An experimental study. J Pediatr Surg 1557-62, 1980
Intussusception
Causing
Intestinal
Atresia
By 0. Adejuyigbe and W.O. Odesanmi l/e-lfe, 0 The patient presented at the age of 2 days with persistent bilious vomiting, failure to pass meconium and urine, and progressive abdominal distension dating from birth, and a clinical diagnosis of intestinal obstruction was made. This was confirmed by plain abdominal radiographs. At laparotomy atresia of the terminal ileum was found. The proximal and distal segments of the bowel ended blindly and there was a V-shaped defect in the adjoining mesentery. The blind ends were resected and an end-toback anastomosis was performed. The lumen of the resected distal segment contained an intussusceptum. 8 1990 by W.B. Saunders Company. INDEX WORDS: Neonatal intestinal obstruction: intrauterine intussusception; intestinal atresia.
I
NTUSSUSCEPTION occuring in intrauterine life is a rarity. To date, less than 30 cases have been adequately documented in the world literature.’ Because of the known tendency of an intussusception to interfere with the blood supply of the involved segment of intestine, intrauterine intassusception had been implicated as a possible cause of congenital intestinal atresia.‘.’ The case of a patient who presented with features of intestinal obstruction and was subsequently found to have intestinal atresia and an intussusception that must have occurred in utero is presented here.
Nigeria
and nasogastric tube suction. Phototherapy was commenced because the serum bilirubin was elevated (7.9 mg%). At laparotomy the findings were congenital atresia of the terminal ileum with the proximal and distal loops ending blindly. The proximal blind loop was grossly distended and of a dark red colour. There was however no evidence of necrosis. There was a small Vshaped defect in the adjoining mesentery with evidenceof mcconium peritonitis in the right lower quadrant of the abdomen. About 12 cm of the proximal lcop and 5 cm of the distal loop were resected and an end-to-back anastomosis was performed to restore gastrointestinal continuity. The child had a smooth postoperative period and was discharged on the 12th postoperative day. He is now 11 months old and his growth and development have been normal.
Pathology Report Examinations of the resected specimens showed gross dilatation of the lumen of the proximal loop while the lumen of the distal loop contained an intussusceptum (Fig 1). Microscopy of the distal bowel loop demonstrated normal intestinal layers with hemorrhagic areas external to the muscular layer. There were also many dilated blood
CASE REPORT Baby A, a boy, was the product of a term pregnancy. His birth weight was 3.5 kg and the l- and Sminute Apgar scores were 10 and 10. He presented at the age of 2 days because of persistent bilious vomiting, failure to pass meconium and urine, all dating from birth, with progressive abdominal distension. There was no history of maternal hydramnios. Physical examination showed a mildly dehydrated term baby with jaundice and no respiratory distress. The abdomen was distended but soft on palpation and apart from the liver that was below the costal margin, no abdominal masses were palpable. The bowel sounds were hyperactive. A plain abdominal x-ray in the supine and erect positions showed marked distension of numerous bowel loops throughout the abdomen with no gas in the pelvis. In the erect position, numerous air-fluid levels were visible in the bowels, the appearance being consistent with a small bowel obstruction. He was started on intravenous fluids
From the Paediatric Surgical Unit, Department of Surgery, and the Department of Morbid Anatomy and Forensic Medicine, University of Ife, Ile-Ife. Nigeria. Address reprint requests to 0. Adejuyigbe. FMCS, Department of Surgery, University of Ife. Ile-Ife. Nigeria. o 1990 by W.B. Saunders Company. 0022-3468/90/2505-oo26$03.00/0
562
Fig 1. (A) Photographof distalbowelloopshowing intussuscep turn within the lumen. (Bl Photograph showing intussuaceptum partially adherent to the outer bowel wall.
hums/
of Pediatric Surgery Vol 25, No 5 (May), 1990:
pp 562-563
NEONATAL INTRAUTERINE
563
INTUSSUSCEPTION
within the muscle layer. In addition, there were areas of mucosal surfaces abutting on each other. These features are consistent with those of intussusception.
vessels
DISCUSSION
Intrauterine bowel intussusception is a rare clinical entity. When intussusception occurs in the fetus and the time elapsed is enough to allow for gangrene and resorption of the intussuscepted portions of the bowel, intestinal atresia will result.lS3 That intestinal atresia may be secondary to prolonged bowel ischemia in utero has long been suspected.’ Laufman et al* demonstrated in 1949 that when isolated sterile devascularized loops of intestine were left in the peritoneal cavity, they converted to fibrous bands or disappeared entirely. This was later con-
firmed by Leuw and Barnard’ in 1955, who were able to produce intestinal atresia in two puppies by ligating the mesenteric vessels supplying a segment of their bowel in utero. Similar results have recently been obtained by Tibboel et a19,” working with the chicken embryo. The case reported here had a V-shaped mesenteric defect between the blindly ending proximal and distal bowel loops. This appearance is similar to those found in experimental animals in which the Mood supply to the bowel had been interfered with in utero. The finding of an intussusceptum in the distal loop suggests that intussusception of the bowel in utero was the primary lesion in this child. The subsequent interference with the blood supply of the intestine then caused the intestinal atresia.
REFERENCES 1. Pavry DR, Marshall DG, Armstrong RF, et al: Intrauterine intussusception: Case report and literature review. Can J Surg 26376378,1983 2. Gherardi GJ, Fisher JH: Atresia of the small intestine produced by intussusception in utero. N Engl J Med 264:229-231,196l 3. Grosveld JL, Clatworthy HW Jr: The nature of ileal atresia due to intrauterine intussusception. Arch Surg 100:714-717,197O 4. Koop CE: Intestinal obstruction in the newborn period, in Levine SZ, Anderson JA, et al (eds): Advances in Pediatrics. Chicago, IL, Year Book, 1953, pp 63-96 5. Leuw JH, Barnard CN: Congenital atresia. Lancet 2:10651067.1955 6. Nixon HH: Intestinal obstruction in the newborn. Arch Dis Child 30:13-22, 1955
7. Ravitch MM: Intussusception, in Paediatric Surgery (ed 3). Ravitch MM, Welch KJ, Benson CD, et al (eds): Chicago, IL, Year Book, 1979, pp 989- 1003 8. Laufman H, Martin WB, Method H, et al: Observations in strangulation obstruction, II. The fate of sterile devascularized intestine in the peritoneal cavity. Arch Surg 59:550-564, 1949 9. Tibboel D, Molenaar JC, Van Nie CJ: New perspective in fetal surgery, the chicken embryo. J Pediatr Surg 14:43&440,1979 10. Tibboel D, Van Nie CJ, Molenaar JC: The effects of temporary general hypoxia and local ischaemia on, the development of the intestines: An experimental study. J Pediatr Surg 1557-62, 1980
