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Congenital duodenal atresia associated with a separate duodenal diaphragm
Congenital Duodenal Atresia Associated With a Separate Duodenal Diaphragm By Pedro J. Rossello • A case of a complete duodenal diaphragm associated with a separate duodenal atresia is described. This association has not been previously reported. INDEX WO R OS: diaphragm.
Duodenal atresia; duodenal
D
U O DE N A L obstruction is frequently associated with intestinal malrotation, esophageal atresia, bowel atresias, congenital heart disease, and renal anomalies. Similarly, an intrinsic obstruction, associated with annular pancreas or preduodenal portal vein, has been well-described. However, to our knowledge, no description of a complete duodenal atresia, in conjunction with a separate, more proximal duodenal diaphragm, has been reported. CASE REPORT
A 7-day-old female was transferred to the University Childrens' Hospital for persistent, bilious vomiting beginning at one day of age. The typical physical stigmata of Down's syndrome were present. An abdominal film showed a typical "double bubble." There were increased vascular markings and an enlarged cardiac silhouette on the chest film. Further evaluation revealed findings consistent with congenital heart disease. Findings at operation consisted of a proximal dilated duodenal bulb with no distal bowel continuity. The proximal portion was generously mobilized, and a standard retrocolic end-to-side duodenojejunostomy was performed. A Stamm gastrostomy was placed. Her postoperative course was complicated by congestive heart failure requiring digitalization. The child had persistently large nasogastric aspirates and no gas noted in the distal bowel. On the 13th postoperative day, a gastrogaflin upper Gl series showed complete obstruction at the duodenum. She was reexplored on postoperative day 15. The previous duodenojejunostomy was inspected, opened, and found to be widely patent. Attempts to pass probes in a retrograde fashion from the duodenum to the stomach were unsuccessful. A gastrostomy at the antral level, and then a second portion longitudinal duodenotomy were performed. A complete duodenal diaphragm was found distal to the ampullary opening (Fig. I). This was resected. Subsequent histologic examination showed the diaphragm to consist of a muscular strip bordered on both sides with mucosa and submucosa. A silastic tube was passed from the stomach to the duodenum and into the efferent loop of the duodenojejunostomy which was reconstructed. After a protracted course complicated by congestive heart failure, digitalis intoxication, sepsis, atelectasis, and several unsuccessful attempts at feedings, she began full oral feedings after postoperative day 35. A follow-up upper G I series done on postoperative day 45 demonstrated a Journal of Pediatric Surgery, Vol. 13. No.4 (August). 1978
patent anastomosis. She was eventually discharged on oral diet and gaining weight on postoperative day 71.
DISCUSSION
The association of intrinsic duodenal obstruction with extrinsic obstruction is known. Annular pancreas, malrotation with Ladd's bands, and preduodenal portal vein, are all causes of extrinsic obstruction which have been described in association with intrinsic obstruction. 1-3 The "wind sock" type of duodenal diaphragm might lead to the performance of a duodenojejunostomy distal to the obstructing diaphragm." However, in that case, there is bowel continuity, which should alert one to this possibility. Instances of two associated intrinsic obstructions, such as double duodenal diaphragm have been described.' Therapeutically, awareness of th e pathologic anatomy in this case would probably have prevented the need for a second operation in this child. It has been recommended that a catheter be passed through a
Fig. 1.
From the Department of Surgery, University of Puerto Rico School of Medicine, and the University Childrens' Hospital. Sanjuan, Puerto Rico. Address reprint requests 10 Pedro J. Rossetto, M.D.; Director, Experimental Surgery Laboratory, G.p.a. Box 5067, San Juan, Puerto Rico 00936. @ 1978 by Grune & Stratton, Inc. 022--3468/78//304-0013$01.00/0 441
PEDRO J . ROSSELLO
442
gastrostomy into the distal part of the duodenum in cases of duodenal obstruction with continuity of the bowel.' We believe this ma-
neuver should be done routinely in all cases of duodenal obstruction to rule out a more proximal duodenal diaphragm.
REFERENCES l. Fonkalsrud EW, Oelorimer AA, Hays OM: Congenital atresia and stenosis of the duodenum. Pediatrics 43:79, 1969 2. Merrill JR, Raffensperger JG : Pedi atric annular pancreas. J Pediatr Surg 11:921, 1976
3. Girv an DP , Stephens CA : Congenital intrinsic duodenal obstruction : A twenty year review of its surgical management consequences. J Pediatr Surg 9:833, 1974 4. Rowe MI, Buckner 0, Clatworthy HW : Wind sock of the duodenum. Ann Surg 116:444,1968
Duodenal atresia; duodenal
D
U O DE N A L obstruction is frequently associated with intestinal malrotation, esophageal atresia, bowel atresias, congenital heart disease, and renal anomalies. Similarly, an intrinsic obstruction, associated with annular pancreas or preduodenal portal vein, has been well-described. However, to our knowledge, no description of a complete duodenal atresia, in conjunction with a separate, more proximal duodenal diaphragm, has been reported. CASE REPORT
A 7-day-old female was transferred to the University Childrens' Hospital for persistent, bilious vomiting beginning at one day of age. The typical physical stigmata of Down's syndrome were present. An abdominal film showed a typical "double bubble." There were increased vascular markings and an enlarged cardiac silhouette on the chest film. Further evaluation revealed findings consistent with congenital heart disease. Findings at operation consisted of a proximal dilated duodenal bulb with no distal bowel continuity. The proximal portion was generously mobilized, and a standard retrocolic end-to-side duodenojejunostomy was performed. A Stamm gastrostomy was placed. Her postoperative course was complicated by congestive heart failure requiring digitalization. The child had persistently large nasogastric aspirates and no gas noted in the distal bowel. On the 13th postoperative day, a gastrogaflin upper Gl series showed complete obstruction at the duodenum. She was reexplored on postoperative day 15. The previous duodenojejunostomy was inspected, opened, and found to be widely patent. Attempts to pass probes in a retrograde fashion from the duodenum to the stomach were unsuccessful. A gastrostomy at the antral level, and then a second portion longitudinal duodenotomy were performed. A complete duodenal diaphragm was found distal to the ampullary opening (Fig. I). This was resected. Subsequent histologic examination showed the diaphragm to consist of a muscular strip bordered on both sides with mucosa and submucosa. A silastic tube was passed from the stomach to the duodenum and into the efferent loop of the duodenojejunostomy which was reconstructed. After a protracted course complicated by congestive heart failure, digitalis intoxication, sepsis, atelectasis, and several unsuccessful attempts at feedings, she began full oral feedings after postoperative day 35. A follow-up upper G I series done on postoperative day 45 demonstrated a Journal of Pediatric Surgery, Vol. 13. No.4 (August). 1978
patent anastomosis. She was eventually discharged on oral diet and gaining weight on postoperative day 71.
DISCUSSION
The association of intrinsic duodenal obstruction with extrinsic obstruction is known. Annular pancreas, malrotation with Ladd's bands, and preduodenal portal vein, are all causes of extrinsic obstruction which have been described in association with intrinsic obstruction. 1-3 The "wind sock" type of duodenal diaphragm might lead to the performance of a duodenojejunostomy distal to the obstructing diaphragm." However, in that case, there is bowel continuity, which should alert one to this possibility. Instances of two associated intrinsic obstructions, such as double duodenal diaphragm have been described.' Therapeutically, awareness of th e pathologic anatomy in this case would probably have prevented the need for a second operation in this child. It has been recommended that a catheter be passed through a
Fig. 1.
From the Department of Surgery, University of Puerto Rico School of Medicine, and the University Childrens' Hospital. Sanjuan, Puerto Rico. Address reprint requests 10 Pedro J. Rossetto, M.D.; Director, Experimental Surgery Laboratory, G.p.a. Box 5067, San Juan, Puerto Rico 00936. @ 1978 by Grune & Stratton, Inc. 022--3468/78//304-0013$01.00/0 441
PEDRO J . ROSSELLO
442
gastrostomy into the distal part of the duodenum in cases of duodenal obstruction with continuity of the bowel.' We believe this ma-
neuver should be done routinely in all cases of duodenal obstruction to rule out a more proximal duodenal diaphragm.
REFERENCES l. Fonkalsrud EW, Oelorimer AA, Hays OM: Congenital atresia and stenosis of the duodenum. Pediatrics 43:79, 1969 2. Merrill JR, Raffensperger JG : Pedi atric annular pancreas. J Pediatr Surg 11:921, 1976
3. Girv an DP , Stephens CA : Congenital intrinsic duodenal obstruction : A twenty year review of its surgical management consequences. J Pediatr Surg 9:833, 1974 4. Rowe MI, Buckner 0, Clatworthy HW : Wind sock of the duodenum. Ann Surg 116:444,1968