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Duodenal atresia secondary to intrauterine midgut strangulation by an omphalocele
Duodenal Atresia Secondary to Intrauterine Midgut Strangulation by an Omphalocele By Hirosada Shigemoto, Yoshihiro Horiya, Toru Isomoto, Yasuhisa Yamamoto, Kaiso Sano and Mitsuko Saito Kurashiki, Japan 9 Presented here is the first instance of duodenal atresia due to intrauterine midgut strangulation and necrosis in a tight omphalocele defect in a newborn infant with myelomeningocele and hypoplasia of both lower extremities. INDEX WORDS: Duodenal atresia; midgut strangulation; omphalocele.
T IS S A I D that early embryologic anomalies are well~locumented causes of duodenal atresia and later intrauterine vascular catastrophes are those of jejunoileal atresias. A few congenital intestinal atresias due to strangulation of the intestine at the navel have been reported in omphalocele and gastroschisis. In these cases the loops of necrotic bowel were short and portions of atresias were only jejunum or ileum, but not duodenum. Our case is the first instance of duodenal atresia due to strangulation of the intestine at the navel in early fetal period in omphalocele.
I
CASE REPORT A female baby with a birth weight of 2,300 g was delivered by cesarean section 5 wk prior to the estimated date of confinement because her mother's pelvic cavity was small. The pregnancy was uncomplicated. At delivery the baby was noted to have infarcted mass (7,5 x 3.0 x 2.5 cm) through a small umbilical defect (1.5 cm in diameter). The base of this infarcted mass was covered by dull reddish, opaque a m n i o n . The center of the mass partly consisted of a cyst like structure (5.0 x 2.5 x 2.0 cm) and the apex was formed by necrotic tissue which was of a gray-color and looked like loops of small intestine 0.4 cm in diameter (Fig. 1). The umbilical cord w a s attached to the left side of infarcted mass (Fig. 2). She had also myelomeningocele and both lower extremities were hypoplastic. A plain erect film of the abdomen revealed two fluid levels, the "double bubble sign" and no evidence of air in the other intestine. A Gastrografin | enema study revealed that the colon ended blindly at the extra-abdominal t u m o r close to the umbilicus. We made the diagnosis of duodenal
From the Department of Surgery, Department of Pediatrics, Kawasaki Medical School, Kurashiki, Japan Address reprint requests to Hirosada Shigemoto, Department of Surgery, Kawasaki Medical School, 577 Matsushima, Kurashiki, Japan. 9 1982 by Grune & Stratton. Inc. 0022-3468/82/1704~9021501.00/0 420
and colonic atresia due to omphalocele strangulation, ruptured myelomeningocele and hypoplasia of both lower extremities. At operation the infarcted mass was excised with the margin of the omphalocele defect at the umbilicus. The infarcted mass involved the entire midgut. There was an infrapapillary duodenal atresia 5 cm from the pyloric ring and the blind end was continuous as a cord into the omphalocele. The colon ended blindly 6 cm from the rectum at the umbilical ring. A side-to--side duodenocolostomy and closure of myelomeningocele were performed. She died of suppurative meningitis on the 16th day postoperatively while being managed by total intravenous nutrition. Histological examiniations revealed that the cystic portion showed intestinal mucosa and muscle layers, but the other portion was homogenous necrotic tissue.
DISCUSSION Tandler I (1900) and Forssner 2 (1907) demons t r a t e d t h a t f r o m t h e fifth to t h e t e n t h e m b r y o n i c w e e k s t h e bowel is a solid cord, t h e l u m e n o f w h i c h r e f o r m s by v a c u o l e s w h i c h c o a l e s c e , a n d in cases w h e r e this c o a l e s c e n c e is i n c o m p l e t e , a t r e sia develops. It is b e l i e v e d t h a t d u o d e n a l a t r e s i a is d u e to this d e v e l o p m e n t a l a n o m a l y e a r l y in e m b r y o n i c life. T h e v a s c u l a r a c c i d e n t t h e o r y of L o u w a n d B a r n a r d 3 is b a s e d on t h e b e l i e f t h a t i n t e r r u p t i o n of t h e blood s u p p l y to a s e g m e n t o f t h e sterile fetal bowel m a y l e a d to t h e a r r e s t o f growth, atrophy and even complete disappeara n c e of t h e a f f e c t e d p o r t i o n o f intestine. T h e y listed volvulus, i n t u s s u s c e p t i o n , s t r a n g u l a t i o n o f t h e i n t e s t i n e at t h e navel, f e t a l p e r i t o n i t i s a n d p r i m a r y lesion o f t h e m e s e n t e r i c a r t e r i e s as possible c a u s e s o f j e j u n o i l e a l a t r e s i a s ? '4 C o n g e n i t a l i n t e s t i n a l a t r e s i a s e c o n d a r y to s t r a n g u l a t i o n o f t h e i n t e s t i n e r e s u l t i n g f r o m t h e c o m p r e s s i o n o f its blood s u p p l y in a t i g h t u m b i l i c a l r i n g is a v e r y r a r e o c c u r r e n c e . In 1904, C l o g g 5 first r e p o r t e d a p a t i e n t w i t h ileal a t r e s i a d u e to an i n f a r c t i o n o f t h e i l e u m s e c o n d a r y to i n c a r c e r a t i o n o f t h e b o w e l a n d its m e s e n t e r y in a t i g h t o m p h a l o c e l e d e f e c t . G r o b , 6 N i x o n 7 a n d E1 S h a f i e 8 r e p o r t e d i d e n t i c a l cases. G r o s f e l d 9 also r e p o r t e d a j e j u n a l a t r e s i a d u e to i n t r a u t e r i n e s t r a n g u l a t i o n in a g a s t r o s c h i sis d e f e c t . In t h e s e cases, n e c r o t i c b o w e l was s h o r t a n d t h e p o r t i o n s o f a t r e s i a w e r e j e j u n u m or i l e u m . N o s i m i l a r o b s e r v a t i o n has e v e r b e e n Journal of Pediatric Surgery, Vol. 17, No. 4 (August), 1982
DOUDENAL ATRESIA
421
Fig. 1. The apex of this infarcted mass through a small umbilical defect consisted of necrotic tissue which was of a gray-color and had a form like loops of small intestine.
m a d e concerning duodenal atresias in an o m p h a locele or a gastroschisis defect. O u r present findings on the c o m p l i c a t i o n of o m p h a l o c e l e provides additional support for the vascular accident theory of L o u w and B a r n a r d not only for j e j u n o i l e a l atresias but also for some duodenal atresias.
REFERENCES
1. Tandler J: Zur Entwickelungsgeschichte des menschlichen Duodenum in friihen Embryonalstadien. Morph Jahrb 29:187-216, 1900, cited by Gray SW and Skandalakis JE: Embryology for surgeons. Philadelphia, W.B. Saunders; 1972, pp 147-156 2. Forssner H: Die angeborenen Darm-und Oesophagusatresien, Anat Hefte 34:l-163, 1907, cited by Gray SW and Skandalakis JE: Ibid., pp 147-156 3. Louw JH, Barnard CN: Congenital intestinal atresia, Lancet 2:1065-1067, 1955
Fig. 2. The umbilical cord was attached to the left side of infarcted mass and there was no skin bridge between umbilical cord and this necrotic mass.
4. Louw JH: Jejunal atresia and stenosis. J Pediat Surg 1:8-23, 1966 5. Clogg HS: Congenital intestinal atresia. Lancet 2:1770-1774, 1904 6. Grob M: Conservative treatment of exomphalos. Arch Dis Child 38:148-150, 1963 7. Nixon HH, Tawes R: Etiology and treatment of small intestinal atresia: Analysis of a series of 127 jejunoileal atresias and comparison with 62 duodenal atresias. Surgery 69:41-51, 1971 8. El Shafie M, Waag KL, Spits L: Ileal atresia secondary to antenatal strangulation by a ruptured ompbalocele. J Pediat Surg 7:64-65, 1972 9. Grosfeld JL, Clatworthy HW: Intrauterine midgut strangulation in a gastroschisis defect. Surgery 67:519-521, 1970
T IS S A I D that early embryologic anomalies are well~locumented causes of duodenal atresia and later intrauterine vascular catastrophes are those of jejunoileal atresias. A few congenital intestinal atresias due to strangulation of the intestine at the navel have been reported in omphalocele and gastroschisis. In these cases the loops of necrotic bowel were short and portions of atresias were only jejunum or ileum, but not duodenum. Our case is the first instance of duodenal atresia due to strangulation of the intestine at the navel in early fetal period in omphalocele.
I
CASE REPORT A female baby with a birth weight of 2,300 g was delivered by cesarean section 5 wk prior to the estimated date of confinement because her mother's pelvic cavity was small. The pregnancy was uncomplicated. At delivery the baby was noted to have infarcted mass (7,5 x 3.0 x 2.5 cm) through a small umbilical defect (1.5 cm in diameter). The base of this infarcted mass was covered by dull reddish, opaque a m n i o n . The center of the mass partly consisted of a cyst like structure (5.0 x 2.5 x 2.0 cm) and the apex was formed by necrotic tissue which was of a gray-color and looked like loops of small intestine 0.4 cm in diameter (Fig. 1). The umbilical cord w a s attached to the left side of infarcted mass (Fig. 2). She had also myelomeningocele and both lower extremities were hypoplastic. A plain erect film of the abdomen revealed two fluid levels, the "double bubble sign" and no evidence of air in the other intestine. A Gastrografin | enema study revealed that the colon ended blindly at the extra-abdominal t u m o r close to the umbilicus. We made the diagnosis of duodenal
From the Department of Surgery, Department of Pediatrics, Kawasaki Medical School, Kurashiki, Japan Address reprint requests to Hirosada Shigemoto, Department of Surgery, Kawasaki Medical School, 577 Matsushima, Kurashiki, Japan. 9 1982 by Grune & Stratton. Inc. 0022-3468/82/1704~9021501.00/0 420
and colonic atresia due to omphalocele strangulation, ruptured myelomeningocele and hypoplasia of both lower extremities. At operation the infarcted mass was excised with the margin of the omphalocele defect at the umbilicus. The infarcted mass involved the entire midgut. There was an infrapapillary duodenal atresia 5 cm from the pyloric ring and the blind end was continuous as a cord into the omphalocele. The colon ended blindly 6 cm from the rectum at the umbilical ring. A side-to--side duodenocolostomy and closure of myelomeningocele were performed. She died of suppurative meningitis on the 16th day postoperatively while being managed by total intravenous nutrition. Histological examiniations revealed that the cystic portion showed intestinal mucosa and muscle layers, but the other portion was homogenous necrotic tissue.
DISCUSSION Tandler I (1900) and Forssner 2 (1907) demons t r a t e d t h a t f r o m t h e fifth to t h e t e n t h e m b r y o n i c w e e k s t h e bowel is a solid cord, t h e l u m e n o f w h i c h r e f o r m s by v a c u o l e s w h i c h c o a l e s c e , a n d in cases w h e r e this c o a l e s c e n c e is i n c o m p l e t e , a t r e sia develops. It is b e l i e v e d t h a t d u o d e n a l a t r e s i a is d u e to this d e v e l o p m e n t a l a n o m a l y e a r l y in e m b r y o n i c life. T h e v a s c u l a r a c c i d e n t t h e o r y of L o u w a n d B a r n a r d 3 is b a s e d on t h e b e l i e f t h a t i n t e r r u p t i o n of t h e blood s u p p l y to a s e g m e n t o f t h e sterile fetal bowel m a y l e a d to t h e a r r e s t o f growth, atrophy and even complete disappeara n c e of t h e a f f e c t e d p o r t i o n o f intestine. T h e y listed volvulus, i n t u s s u s c e p t i o n , s t r a n g u l a t i o n o f t h e i n t e s t i n e at t h e navel, f e t a l p e r i t o n i t i s a n d p r i m a r y lesion o f t h e m e s e n t e r i c a r t e r i e s as possible c a u s e s o f j e j u n o i l e a l a t r e s i a s ? '4 C o n g e n i t a l i n t e s t i n a l a t r e s i a s e c o n d a r y to s t r a n g u l a t i o n o f t h e i n t e s t i n e r e s u l t i n g f r o m t h e c o m p r e s s i o n o f its blood s u p p l y in a t i g h t u m b i l i c a l r i n g is a v e r y r a r e o c c u r r e n c e . In 1904, C l o g g 5 first r e p o r t e d a p a t i e n t w i t h ileal a t r e s i a d u e to an i n f a r c t i o n o f t h e i l e u m s e c o n d a r y to i n c a r c e r a t i o n o f t h e b o w e l a n d its m e s e n t e r y in a t i g h t o m p h a l o c e l e d e f e c t . G r o b , 6 N i x o n 7 a n d E1 S h a f i e 8 r e p o r t e d i d e n t i c a l cases. G r o s f e l d 9 also r e p o r t e d a j e j u n a l a t r e s i a d u e to i n t r a u t e r i n e s t r a n g u l a t i o n in a g a s t r o s c h i sis d e f e c t . In t h e s e cases, n e c r o t i c b o w e l was s h o r t a n d t h e p o r t i o n s o f a t r e s i a w e r e j e j u n u m or i l e u m . N o s i m i l a r o b s e r v a t i o n has e v e r b e e n Journal of Pediatric Surgery, Vol. 17, No. 4 (August), 1982
DOUDENAL ATRESIA
421
Fig. 1. The apex of this infarcted mass through a small umbilical defect consisted of necrotic tissue which was of a gray-color and had a form like loops of small intestine.
m a d e concerning duodenal atresias in an o m p h a locele or a gastroschisis defect. O u r present findings on the c o m p l i c a t i o n of o m p h a l o c e l e provides additional support for the vascular accident theory of L o u w and B a r n a r d not only for j e j u n o i l e a l atresias but also for some duodenal atresias.
REFERENCES
1. Tandler J: Zur Entwickelungsgeschichte des menschlichen Duodenum in friihen Embryonalstadien. Morph Jahrb 29:187-216, 1900, cited by Gray SW and Skandalakis JE: Embryology for surgeons. Philadelphia, W.B. Saunders; 1972, pp 147-156 2. Forssner H: Die angeborenen Darm-und Oesophagusatresien, Anat Hefte 34:l-163, 1907, cited by Gray SW and Skandalakis JE: Ibid., pp 147-156 3. Louw JH, Barnard CN: Congenital intestinal atresia, Lancet 2:1065-1067, 1955
Fig. 2. The umbilical cord was attached to the left side of infarcted mass and there was no skin bridge between umbilical cord and this necrotic mass.
4. Louw JH: Jejunal atresia and stenosis. J Pediat Surg 1:8-23, 1966 5. Clogg HS: Congenital intestinal atresia. Lancet 2:1770-1774, 1904 6. Grob M: Conservative treatment of exomphalos. Arch Dis Child 38:148-150, 1963 7. Nixon HH, Tawes R: Etiology and treatment of small intestinal atresia: Analysis of a series of 127 jejunoileal atresias and comparison with 62 duodenal atresias. Surgery 69:41-51, 1971 8. El Shafie M, Waag KL, Spits L: Ileal atresia secondary to antenatal strangulation by a ruptured ompbalocele. J Pediat Surg 7:64-65, 1972 9. Grosfeld JL, Clatworthy HW: Intrauterine midgut strangulation in a gastroschisis defect. Surgery 67:519-521, 1970