- Email: [email protected]
Atresia of the colon at the splenic flexure with absence of the distal colon and ischemic destruction of the proximal colon
CASE REPORTS
Atresia of the Colon at the Splenic Flexure With Absence of the Distal Colon and Ischemic Destruction of the P r o x i m a l Colon By Thomas C. Moore 9 Because of the rarity of ischemic destruction of the colon proximal to a colonic atresia, the author reports a case of splenic flexure colonic atresia associated with absence of the distal colon and ischemic destruction of the proximal colon managed successfully by resection and primary ileo-sigmoidostomy.
I N D EX W O R DS : Colonic atresia.
Atresia of the colon is the rarest of alimentary tract atresias? Colonic atresia, like jejuno-ileal atresias, most likely occurs as a result of an intrauterine vascular accident and, possibly for this reason, shares with jejuno-ileal atresias a remarkable lack of association with major additional multiple malformations. ~-3 The major additional malformation accompanying colonic atresia appears to be gastroschisis. Gastroschisis was observed by Boles et al. 2in 4 of 11 cases of colonic atresia (36%) and may have contributed to ischemia-related intrauterine colonic damage. Like jejuno-ileal atresias, which occur in 14 percent of the cases of gastroschisis,4 colonic atresia may be an integral causeand-effect-related part of the gastroschisis condition. Most colonic atresias may be treated effectively by decompressing eolostomy and subsequent reestablishment of colonic patency or by primary anastomosis following local resection of the atretic segments. Major ischemic damage to the colon proximal to an atresia is quite rare and requires resection. This occurs more frequently when the atresia is at or proximal to the splenic flexure. In one of the cases without gastroschisis reported by Boles et al., 2 concern about the viability of the discolored bulbous end of the cecum led t o resection and ileostomy with subsequent ileocolostomy. Cecal resection with ileostomy was required in two of their four cases with associated gastroschisis due to bulbous changes or gangrene, In the second case of Sturim and Ternberg, a an ascending colon atresia was associated with a 360* torsion and infarction of the proximal colon and distal ileum that was managed by resection and primary ileocolostomy.
CASE REPORT The patient was a 7 lb 3 oz, 36-hr-old white male who was admitted to the UCLA-Harbor General Hospital in December 1971 because of abdominal distention and roentgen evidence of a large intraabdominal accumulation of gas suggesting massive megaeolon (Fig. 1). The patient had a right facial palsy with facial asymmetry on the left with microphthalmia and partial iridial coloboma. The
Journal of Pediatric Surgery, Vol. 13, No. 1 (February),1978
barium enema showed a short segment of rectosigmoid colon that ended blindly. At operation, two atresias at the sigmoid colon were found to be connected by a short fibrous band. The descending and sigmoid colon were missing. The colon proximal to the splenic flexure atresia was massively distended, thickened, and darkly discolored and filled the entire abdominal cavity. The distal ileum and small intestine were not distended and were of normal size. The massively distended, thickened, and ischemically destroyed proximal colon was considered to be nonsalvageable and was resected with the splenic flexure atretic segments. A primary oblique end-to-end anastomosis was carried out between the terminal ileum and small reetosigmoid segment that protruded about 1 cm above the pelvic floor. A gastrostomy was performed. There were no other congenital malformations. The postoperative course was uneventful. The first meconium stool was passed 36 hr following operation and during the next day the infant passed six meconium stools. Intravenous pyelography shortly after operation and at yearly intervals have been normal. The infant was released from the hospital 19 days following operation. He weighed 15 lb at 5 mo of age, 21 lb at 10 too, and 32 lb at 3 yr. Bowel movements have been normal. His facial paralysis and asymmetry have improved significantly. Mental development of the patient has been quite good; indeed, the patient is considered to be mentally gifted by the family pediatrician.
DISCUSSION Primary bowel anastomosis was facilitated in this case because of the lack of ileal and small bowel distention. Small bowel distention was probably avoided by early operation, the discharge of small bowel contents into the massively enlarged and thickened proximal colonic receptacle, and the compressive effect of the massive(y dilated proximal colonic segment on adjacent small intestine. From the appearance of the ischemic, distended, and thickened proximal colon, it is likely that this damage had occurred in utero. Ileorectosigmoidostomy was not associated with untoward effects on bowel movements or on physical and mental growth and development.
From the Division of Pediatric Surgery, UCLA-Harbor General Hospital and the Department of Surgery, University of California, Los Angeles, Calif. ttddress reprint requests to Thomas C. Moore, M.D., Department of Surgery, University of California, Los Angeles, Calif. 9 1978 by Grune & Stratton, Inc. 0022-3468/78/1301-0019501.00/0
89
90
CASE REPORT
Fig, 1. Flat film of the abdomen (A) and upside-down film of the abdomen (B) showing the great air and fluid distention of the massively enlarged iscbemic segment of colon proximal to the splenic flexure atresia.
REFERENCES
1. Freeman NV: Congenital atresia and stenosis of the colon. Br J Surg 53:595, 1966 2. Boles ET Jr, Vassy LE, Ralston M: Atresia of the colon. J Pediatr Surg 11:69, 1976
3. Sturim HS, Ternberg JL: Congenital atresia of the colon. Surgery 59:458, 1966 4. Moore TC: Gastroschisis and omphalocele: Clinical differences. Surgery (in press)
Atresia of the Colon at the Splenic Flexure With Absence of the Distal Colon and Ischemic Destruction of the P r o x i m a l Colon By Thomas C. Moore 9 Because of the rarity of ischemic destruction of the colon proximal to a colonic atresia, the author reports a case of splenic flexure colonic atresia associated with absence of the distal colon and ischemic destruction of the proximal colon managed successfully by resection and primary ileo-sigmoidostomy.
I N D EX W O R DS : Colonic atresia.
Atresia of the colon is the rarest of alimentary tract atresias? Colonic atresia, like jejuno-ileal atresias, most likely occurs as a result of an intrauterine vascular accident and, possibly for this reason, shares with jejuno-ileal atresias a remarkable lack of association with major additional multiple malformations. ~-3 The major additional malformation accompanying colonic atresia appears to be gastroschisis. Gastroschisis was observed by Boles et al. 2in 4 of 11 cases of colonic atresia (36%) and may have contributed to ischemia-related intrauterine colonic damage. Like jejuno-ileal atresias, which occur in 14 percent of the cases of gastroschisis,4 colonic atresia may be an integral causeand-effect-related part of the gastroschisis condition. Most colonic atresias may be treated effectively by decompressing eolostomy and subsequent reestablishment of colonic patency or by primary anastomosis following local resection of the atretic segments. Major ischemic damage to the colon proximal to an atresia is quite rare and requires resection. This occurs more frequently when the atresia is at or proximal to the splenic flexure. In one of the cases without gastroschisis reported by Boles et al., 2 concern about the viability of the discolored bulbous end of the cecum led t o resection and ileostomy with subsequent ileocolostomy. Cecal resection with ileostomy was required in two of their four cases with associated gastroschisis due to bulbous changes or gangrene, In the second case of Sturim and Ternberg, a an ascending colon atresia was associated with a 360* torsion and infarction of the proximal colon and distal ileum that was managed by resection and primary ileocolostomy.
CASE REPORT The patient was a 7 lb 3 oz, 36-hr-old white male who was admitted to the UCLA-Harbor General Hospital in December 1971 because of abdominal distention and roentgen evidence of a large intraabdominal accumulation of gas suggesting massive megaeolon (Fig. 1). The patient had a right facial palsy with facial asymmetry on the left with microphthalmia and partial iridial coloboma. The
Journal of Pediatric Surgery, Vol. 13, No. 1 (February),1978
barium enema showed a short segment of rectosigmoid colon that ended blindly. At operation, two atresias at the sigmoid colon were found to be connected by a short fibrous band. The descending and sigmoid colon were missing. The colon proximal to the splenic flexure atresia was massively distended, thickened, and darkly discolored and filled the entire abdominal cavity. The distal ileum and small intestine were not distended and were of normal size. The massively distended, thickened, and ischemically destroyed proximal colon was considered to be nonsalvageable and was resected with the splenic flexure atretic segments. A primary oblique end-to-end anastomosis was carried out between the terminal ileum and small reetosigmoid segment that protruded about 1 cm above the pelvic floor. A gastrostomy was performed. There were no other congenital malformations. The postoperative course was uneventful. The first meconium stool was passed 36 hr following operation and during the next day the infant passed six meconium stools. Intravenous pyelography shortly after operation and at yearly intervals have been normal. The infant was released from the hospital 19 days following operation. He weighed 15 lb at 5 mo of age, 21 lb at 10 too, and 32 lb at 3 yr. Bowel movements have been normal. His facial paralysis and asymmetry have improved significantly. Mental development of the patient has been quite good; indeed, the patient is considered to be mentally gifted by the family pediatrician.
DISCUSSION Primary bowel anastomosis was facilitated in this case because of the lack of ileal and small bowel distention. Small bowel distention was probably avoided by early operation, the discharge of small bowel contents into the massively enlarged and thickened proximal colonic receptacle, and the compressive effect of the massive(y dilated proximal colonic segment on adjacent small intestine. From the appearance of the ischemic, distended, and thickened proximal colon, it is likely that this damage had occurred in utero. Ileorectosigmoidostomy was not associated with untoward effects on bowel movements or on physical and mental growth and development.
From the Division of Pediatric Surgery, UCLA-Harbor General Hospital and the Department of Surgery, University of California, Los Angeles, Calif. ttddress reprint requests to Thomas C. Moore, M.D., Department of Surgery, University of California, Los Angeles, Calif. 9 1978 by Grune & Stratton, Inc. 0022-3468/78/1301-0019501.00/0
89
90
CASE REPORT
Fig, 1. Flat film of the abdomen (A) and upside-down film of the abdomen (B) showing the great air and fluid distention of the massively enlarged iscbemic segment of colon proximal to the splenic flexure atresia.
REFERENCES
1. Freeman NV: Congenital atresia and stenosis of the colon. Br J Surg 53:595, 1966 2. Boles ET Jr, Vassy LE, Ralston M: Atresia of the colon. J Pediatr Surg 11:69, 1976
3. Sturim HS, Ternberg JL: Congenital atresia of the colon. Surgery 59:458, 1966 4. Moore TC: Gastroschisis and omphalocele: Clinical differences. Surgery (in press)