Agenesis of the Dorsal Mesentery Without Jejunoileal Atresia (“Apple Peel Small Bowel”) By James D. Hull ill, John L. Kiesel. Warren H. Proudfoot, and Robert P. Belin
I
NTESTINAL ATRESIA is a relatively uncommon congenital anomaly occurring approximately once in 20,000 live births1 Historically, this anomaly was attributed to developmental failure of recanalization of the intestinal tube during the second month. It is now recognized that recanalization occurs only in the duodenum and that jejunal and ileal atresias are due to mesenteric vascular occlusior? or other mechanical factors. In 1961 Santulli3 first described the variant of small bowel atresia with an anomalous “apple peel” bowel-mesenteric attachment. This congenital small bowel configuration was renamed the “Christmas tree” deformity and was expanded upon by Weitzman and Vanderhoof4 in 1966 with their report of four cases. Subsequently, other reports of jejunal atresia with agenesis of the dorsal mesentery have appeared. 5*eSharma’ reported a case of transmesenteric hernia which was probably agenesis of the dorsal mesentery without jejunoileal atresia but was not recognized as such. The following, however, is a report of a child with agenesis of the dorsal mesentery and the “apple peel” or “Christmas tree” deformity but without small-bowel atresia. CASE REPORT T. E., a 21 z- yr-old previously healthy white male was hospitalized 6 hr after the sudden onset of fever, abdominal cramps, and vomiting. The child was first seen by a surgeon 8 hr after admission at which time he had evidence of generalized peritonitis with greatest tenderness in the right lower quadrant. There was distention, rebound tenderness, absent bowel sounds, and abdominal x-rays demonstrated loops of distended small bowel. Temperature was 40°C. WBC 10,000 and hemoglobin 12.1 g. With a preoperative diagnosis of ruptured appendicitis, celiotomy was performed and an internal transmesenteric hernia was found. The ileum had herniated through a mesenteric defect, and 45 cm of distal ileum had undergone volvulus, strangulation, and infarction. It was obvious that the mesentery was absent from the superior mesenteric artery to the ileocolic vessels, The blood supply to this area was from a marginal vessel. Vessel and bowel were convoluted in a fashion identical to the neonatal “apple peel” or “Christmas tree” deformity associated with atresia. Resection of the infarcted bowel with ileoileostomy and closure of the mesenteric defect were performed. The postoperative course was unremarkable, and at 8 mo postoperatively the child was well.
DISCUSSION
The theory of mesenteric vascular accidents contributing to small-bowel atresias is derived from both clinical and experimental evidence. If it occurs at all, recanalization of the intestinal tube takes place during the second month. In all cases of small-bowel atresia in which the distal bowel or contents could be examined, meconium, squamous epithelial cells, and bile droplets were found.” From the Departments of Surgery and Pediatrics, St. Claire Medical Center, Morehead. Ky, and the Division of Pediatric Surgery, Department of Surgery, University of Kentucky, Lexington, KY. Address for reprint requests: James D. Hull, III. M.D., Cave Run Clinic. Morehead. Ky40351. b 1975 by Grune &iStratton, Inc. Journal of Pediarric Surgery, Vol. 10. No 2 (April). 1975
277
278
HULL
ET AL.
Since these elements do not appear in the stool until the 1lth or 12th wk, the occlusion must have occurred after this time and, therefore, well beyond the time of the theoretical development of the intestinal tube.‘.3.6 Louw and Barnard8 have noted that 40% of patients with intestinal atresia had a V-shaped defect in the mesentery. They postulated that atresias may be due in part to interference with the blood supply to the fetal gut. This was subsequently substantiated with the production of bowel atresias with a mesenteric defect in newborn puppies by inutero ligation of a branch of the mesenteric artery.g Microscopic studies of the atretic segment show evidence of bowel injury and evidence of repair identical to that seen with other vascular occlusions. In 1960, BlandylO reported a case of intestinal obstruction in a newborn caused by internal herniation through a mesenteric defect 4 x 2 inches with marginal vessels to the terminal ileum around the free edge of the defect. This report also noted that congenital mesenteric defects with vascular margination had been reported often. Subsequently, Sharma et al. 7 described an adult with a transmesenteric hernia in which there was a large defect in the mesentery of the small bowel with the mesenteric blood supply running along the margin of the defect. The bowel configuration as depicted in their figures presented with this report seems to be the apple peel or Christmas tree deformity. This appears to be the first report, although not recognized as such, of agenesis of the dorsal mesentery with apple peel or Christmas tree deformity but without intestinal atresia. Therefore, it would seem that small-bowel atresias, jejunal atresia with agenesis of the dorsal mesentery, and transmesenteric hernial defects are all variants of the same entity. An in-utero vascular accident involving various degrees of the superior mesenteric artery produces a defect in the mesentery and/ or small bowel. This results clinically in atresia, in atresia with a mesenteric defect, or simply a mesenteric defect. In cases with a mesenteric defect, the attachment of the bowel to a marginal artery may be such that the bowel can outgrow its vascular supply producing the coiling typical of the apple peel. This case as well as Sharma’s7 fit into this apple peel category, even though it previously has been reported only with the associated atresia. The evidence suggests that these are all variants of one disease process. It is important to recognize that the apple peel deformity may occur in the older infant, child, and even adult since the marginal vessel may be the entire blood supply to the small bowel. Inadvertent damage would result in extensive bowel death. SUMMARY
Agenesis of the dorsal mesentery with apple peel or Christmas tree deformity but without small-bowel atresia can occur beyond the neonatal period. The recognition of this entity is imperative as it is also associated with a marginal artery which may be the only blood supply to the majority of small bowel. Preservation of this vessel is necessary to avoid catastrophic bowel death. REFERENCES 1. White JJ, Tecklenberg PL, Esterly JR, et al: Changing concepts in the management of intestinal atresia. Surg Clin N Am 50:863, 1970
2. de Lorimer AA, Fonkalsrud EW, Hays DM: Congenital atresia and stenosis of the jejunum and ileum. Surgery 65:819, 1969
APPLE
PEEL SMALL
BOWEL
3. Santulli TV, Blanc WA: Congenital atresia of the intestine: Pathogenesis and treatment. Ann Surg 154:939, 1961 4. Weitzman JJ, Vanderhoof RS: Jejunal atresia with agenesis of the dorsal mesentery with “christmas tree” deformity of the small intestine. Am J Surg 111:443. 1966 5. Dickson JAS: Apple peel small bowel: An uncommon variant of duodenal and jejunal atresia. J Pediatr Surg 5:59S, 1970 6. Zwiren GT, Andrews HG. Ahmann P: Jejunal atresia with agenesis of the dorsal
279
mesentery
(“apple
peel small bowel”).
J Pediatr
Surg 7:414, 1972 7. Sharma BD, Bhargava KS, Galviya UD: Transmesenteric hernia. Arch Surg 96:306, 1968 8. Louw JH, Barnard CN: Congenital intestinal atresia: Observations on its origin. Lancet 269:1065, 1955 9. Barnard CN: The genesis of intestinal atresia. Surg Forum 7:393, 1956 IO. Blandy JP: Neonatal intestinal obstruction from a congenital hole in the mesentery. Br J Surg 48:133, 1960
I
NTESTINAL ATRESIA is a relatively uncommon congenital anomaly occurring approximately once in 20,000 live births1 Historically, this anomaly was attributed to developmental failure of recanalization of the intestinal tube during the second month. It is now recognized that recanalization occurs only in the duodenum and that jejunal and ileal atresias are due to mesenteric vascular occlusior? or other mechanical factors. In 1961 Santulli3 first described the variant of small bowel atresia with an anomalous “apple peel” bowel-mesenteric attachment. This congenital small bowel configuration was renamed the “Christmas tree” deformity and was expanded upon by Weitzman and Vanderhoof4 in 1966 with their report of four cases. Subsequently, other reports of jejunal atresia with agenesis of the dorsal mesentery have appeared. 5*eSharma’ reported a case of transmesenteric hernia which was probably agenesis of the dorsal mesentery without jejunoileal atresia but was not recognized as such. The following, however, is a report of a child with agenesis of the dorsal mesentery and the “apple peel” or “Christmas tree” deformity but without small-bowel atresia. CASE REPORT T. E., a 21 z- yr-old previously healthy white male was hospitalized 6 hr after the sudden onset of fever, abdominal cramps, and vomiting. The child was first seen by a surgeon 8 hr after admission at which time he had evidence of generalized peritonitis with greatest tenderness in the right lower quadrant. There was distention, rebound tenderness, absent bowel sounds, and abdominal x-rays demonstrated loops of distended small bowel. Temperature was 40°C. WBC 10,000 and hemoglobin 12.1 g. With a preoperative diagnosis of ruptured appendicitis, celiotomy was performed and an internal transmesenteric hernia was found. The ileum had herniated through a mesenteric defect, and 45 cm of distal ileum had undergone volvulus, strangulation, and infarction. It was obvious that the mesentery was absent from the superior mesenteric artery to the ileocolic vessels, The blood supply to this area was from a marginal vessel. Vessel and bowel were convoluted in a fashion identical to the neonatal “apple peel” or “Christmas tree” deformity associated with atresia. Resection of the infarcted bowel with ileoileostomy and closure of the mesenteric defect were performed. The postoperative course was unremarkable, and at 8 mo postoperatively the child was well.
DISCUSSION
The theory of mesenteric vascular accidents contributing to small-bowel atresias is derived from both clinical and experimental evidence. If it occurs at all, recanalization of the intestinal tube takes place during the second month. In all cases of small-bowel atresia in which the distal bowel or contents could be examined, meconium, squamous epithelial cells, and bile droplets were found.” From the Departments of Surgery and Pediatrics, St. Claire Medical Center, Morehead. Ky, and the Division of Pediatric Surgery, Department of Surgery, University of Kentucky, Lexington, KY. Address for reprint requests: James D. Hull, III. M.D., Cave Run Clinic. Morehead. Ky40351. b 1975 by Grune &iStratton, Inc. Journal of Pediarric Surgery, Vol. 10. No 2 (April). 1975
277
278
HULL
ET AL.
Since these elements do not appear in the stool until the 1lth or 12th wk, the occlusion must have occurred after this time and, therefore, well beyond the time of the theoretical development of the intestinal tube.‘.3.6 Louw and Barnard8 have noted that 40% of patients with intestinal atresia had a V-shaped defect in the mesentery. They postulated that atresias may be due in part to interference with the blood supply to the fetal gut. This was subsequently substantiated with the production of bowel atresias with a mesenteric defect in newborn puppies by inutero ligation of a branch of the mesenteric artery.g Microscopic studies of the atretic segment show evidence of bowel injury and evidence of repair identical to that seen with other vascular occlusions. In 1960, BlandylO reported a case of intestinal obstruction in a newborn caused by internal herniation through a mesenteric defect 4 x 2 inches with marginal vessels to the terminal ileum around the free edge of the defect. This report also noted that congenital mesenteric defects with vascular margination had been reported often. Subsequently, Sharma et al. 7 described an adult with a transmesenteric hernia in which there was a large defect in the mesentery of the small bowel with the mesenteric blood supply running along the margin of the defect. The bowel configuration as depicted in their figures presented with this report seems to be the apple peel or Christmas tree deformity. This appears to be the first report, although not recognized as such, of agenesis of the dorsal mesentery with apple peel or Christmas tree deformity but without intestinal atresia. Therefore, it would seem that small-bowel atresias, jejunal atresia with agenesis of the dorsal mesentery, and transmesenteric hernial defects are all variants of the same entity. An in-utero vascular accident involving various degrees of the superior mesenteric artery produces a defect in the mesentery and/ or small bowel. This results clinically in atresia, in atresia with a mesenteric defect, or simply a mesenteric defect. In cases with a mesenteric defect, the attachment of the bowel to a marginal artery may be such that the bowel can outgrow its vascular supply producing the coiling typical of the apple peel. This case as well as Sharma’s7 fit into this apple peel category, even though it previously has been reported only with the associated atresia. The evidence suggests that these are all variants of one disease process. It is important to recognize that the apple peel deformity may occur in the older infant, child, and even adult since the marginal vessel may be the entire blood supply to the small bowel. Inadvertent damage would result in extensive bowel death. SUMMARY
Agenesis of the dorsal mesentery with apple peel or Christmas tree deformity but without small-bowel atresia can occur beyond the neonatal period. The recognition of this entity is imperative as it is also associated with a marginal artery which may be the only blood supply to the majority of small bowel. Preservation of this vessel is necessary to avoid catastrophic bowel death. REFERENCES 1. White JJ, Tecklenberg PL, Esterly JR, et al: Changing concepts in the management of intestinal atresia. Surg Clin N Am 50:863, 1970
2. de Lorimer AA, Fonkalsrud EW, Hays DM: Congenital atresia and stenosis of the jejunum and ileum. Surgery 65:819, 1969
APPLE
PEEL SMALL
BOWEL
3. Santulli TV, Blanc WA: Congenital atresia of the intestine: Pathogenesis and treatment. Ann Surg 154:939, 1961 4. Weitzman JJ, Vanderhoof RS: Jejunal atresia with agenesis of the dorsal mesentery with “christmas tree” deformity of the small intestine. Am J Surg 111:443. 1966 5. Dickson JAS: Apple peel small bowel: An uncommon variant of duodenal and jejunal atresia. J Pediatr Surg 5:59S, 1970 6. Zwiren GT, Andrews HG. Ahmann P: Jejunal atresia with agenesis of the dorsal
279
mesentery
(“apple
peel small bowel”).
J Pediatr
Surg 7:414, 1972 7. Sharma BD, Bhargava KS, Galviya UD: Transmesenteric hernia. Arch Surg 96:306, 1968 8. Louw JH, Barnard CN: Congenital intestinal atresia: Observations on its origin. Lancet 269:1065, 1955 9. Barnard CN: The genesis of intestinal atresia. Surg Forum 7:393, 1956 IO. Blandy JP: Neonatal intestinal obstruction from a congenital hole in the mesentery. Br J Surg 48:133, 1960
