•
Duodenojejunal Atresia with "Apple-Peel" Small Bowel
•
Pediatric Radiology
A DistinctForm of Intestinal Atresia 1 John C. Leonidas, M.D., Raymond A. Amoury, M.D., Keith W. Ashcraft, M.D., and Richard A. Fellows, M.D. Prenatal occlusion of the superior mesenteric artery results in a distinct type of distal duodenal or proximal jejunal atresia in which the dorsal mesentery is absent and the distal small bowel assumes a spiral configuration around its vascular stalk, strongly resembling an apple peel. In some instances the condition is transmitted genetically as an autosomal recessive disorder. The mortality rate is much higher than in either simple duodenal or jejunal atresia because of deficient collateral circulation to the small intestine. The authors tell of 2 patients who died of necrotizing enterocolitis, a disease related to vascular insufficiency. INDEX TERMS: ties
Arteries, mesenteric· Duodenum, abnormalities· Intestines, abnormali-
Radiology 118: 661-665, March 1976
• HE " APPLE-PEEL" form of intestinal atresia, first described by Santulli and Blanc in 1961 (1), consists of distal duodenal or proximal jejunal atresia with obliteration of the superior mesenteric artery a short distance beyond its origin and absence of the dorsal mesentery. Distal to the atresia, the small intestine is relatively short and small in caliber. It lies free in the peritoneal cavity, having no mesenteric attachment and being only distally connected to the cecum (2-7). Because of the typical spiral arrangement of small-bowel loops around a vascular stalk, the appearance has been described appropriately as "apple-peel" (1, 6), "Christmas-tree" (2), "maypole" (3), or "pagoda" (4) (Fig. 1). The condition is quite rare, with only 34 cases reported in the English literature by the end of 1972 (1-10). It was not described prior to 1961, although a few patients seen before that time have subsequently been recognized as having the apple-peel syndrome (8, 10). Many cases in the past, and perhaps even now, are erroneously diagnosed as duodenojejunal atresia occurring in association with midgut malrotation and prenatal volvulus (7, 8). The peculiar blood supply of the apple-peel small bowel is a constant feature which is of great value in clarifying its pathogenesis. Most of the distal superior mesenteric artery (SMA) is absent, including the straight pre-arterial arcades to the small intestine. The small bowel distal to the site of atresia derives its blood supply from collateral vessels formed by branches of the middle and right colic arteries and ileocolic artery (2, 5, 7). These vessels form a marginal artery which initially runs parallel to the right colon and then becomes the axis for the coiled distal small bowel (Figs. 1, Band 2). It is reasonable to assume that prenatal occlusion of the SMA distal to the origin of the middle colic artery causes aseptic necrosis and disappearance of part of the small bowel along with the dorsal mesentery (2, 6, 7) (Fig. 2).
•
T
Fig 1. CASE II. A. Apple-peel jejunal atresia. The surgical clamp on the left has been placed on the appendix. The small bowel distal to the site of atresia has been eviscerated and presents the characteristic "apple-peel" spiral configuration. B. Close-up of the small bowel, showing the mesenteric defect (upper arrow) and the vascular stalk carrying the collateral circulation just above the distal ileum (lower arrow).
Among the important features of the apple-peel small bowel are its familial incidence-probably through an autosomal recessive mode of inheritance (6,8, 10)-as
1 From the Departments of Radiology (J. C. L., R. A. F.) and Surgery (R. A. A., K. W. A.), Children's Mercy Hospital and the University of Missouri School of Medicine, Kansas City, Mo. Accepted for publication in August 1975. Presented at the Eighteenth Annual Meeting of the Society for Pediatric Radiology, Atlanta, Ga., Sept. 28-29, 1975. sjh
Fig. 2. A. Schematic drawing of the normal distribution of the superior mesenteric artery and its branches. Prenatal occlusion at the point indicated leads to "disappearance" of part of the jejunum and the dorsal mesentery. B. Diagram showing what happens when the circulation to the distal small bowel is maintained by collateral vessels (apple-peel small bowel). Table I: Case Sex F
Summary of Findings
Clinical Findings
Roentgenographic Findings
1 day
Premature {birth weight 1,745 g); bilestained vomitus
Complete duodenal obstruction ("double bubble")
2 mo.
Bloody stools and disten-
Pneumatosis intestinalis, hepatic portal vein gas {Fig. 4)
Age
Operative Findings and Procedures Double duodenal atresia; typical apple-peel small bowel; ma Irotation of the colon End-to-end duodenojejunostomy performed following excision of the third part of the duodenum and gastrostomy Total necrosis of the jejunum without volvuIus, compression, or obstruction Resection and duodenoileostomy Atresia of the proximal jejunurn: apple-peel small bowel with a second atresia in the distal jejunal segment Resection of the proximal part of the distal jejunum; reduction of the diameter of the duodenum and end-to-end duodenojejunostomy; qastrostorny Small bowel marginally viable with segmental ileal necrosis but no volvulus Abdominal exploration
sion
II
M
2 days
Premature (birth weight 1,600 g); bilestained vomitus
Distension of the entire duodenal loop; complete distal duodenal obstructi on
27 days
Bloody stools and distension
Generalized smallbowel distension
662
Course
Autopsy Findings
Total parenteral alimentation complicated by superior vena cava thrombosis and sepsis; persistent diarrhea
Died following the operation
Ischemic necrosis of the resected bowel; pulmonary atelectasis; superior vena cavathrombosis with recanalization
Initially good
Died following the operation
Ischemic necrotizing enteritis; peritonitis; pulmonary edema; intrahepatic cholestasis
Vol. 118
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Pediatric Radiology
well as associated anomalies (most frequently colonic malrotation) (2, 5, 6, 8) and a continuing tendency toward the development of ischemic enteritis. Two infants with apple-peel small bowel recently seen at The Children's Mercy Hospital in Kansas City form the basis of the present report. Similar cases reported in the literature were also reviewed and compared with our own. CASE MATERIAL AND REVIEW OF THE LITERATURE
The clinical, radiographic, and surgical features of our 2 cases are reviewed in TABLE I. Of the 34 patients reported in the literature, 9 were male and 16 were female; the sex of the other 9 patients was not mentioned (1, 4, 6, 9). Of the 24 patients whose birth weight was specified, 14 (58 %) weighed less than 2.5 kg (mean, 2.06 kg), while 10 (42%) weighed more than 2.5 kg (mean, 2.71 kg). No birth weight was stated for the other 10 infants (4, 6, 9), though 4 were described as "premature" (1, 5). No information is available regarding the gestational age of the infants with this syndrome. Comparison of these figures with those of Fonkalsrud et a/. (11) on congenital duodenal atresia and stenosis and deLorimier et a/. (12) on jejuno-ileal atresia and stenosis suggests that an equal sex ratio and a high incidence of prematurity are common in all of these disorders. Four infants with apple-peel small bowel also had a history of maternal polyhydramnios (2, 8). Symptoms and signs have invariably been those of postampullary high intestinal obstruction (gastric retention, bile-stained vomitus, and failure to pass meconium (2, 7, 8). Generalized abdominal distension may be present (5); more often, however, the abdomen is flat or distension is limited to the epigastrium (2, 8). The roentgenographic findings are those of complete duodenal or proximal jejunal obstruction (2, 7, 8). The patient in CASE I of Weitzman and Vanderhoof (2) had dilatation of the proximal duodenum caused by peritoneal bands as well as marked distension of the proximal duodenojejunal loop. The first of our 2 patients exhibited the characteristic "double bubble" of duodenal atresia, and this was confirmed surgically (TABLE I). The abdominal radiograph shown by Zwiren et a/. (7) reveals 3 to 4 upper abdominal gas bubbles with air-fluid levels, consistent with proximal jejunal atresia, and similar findings were present in the patient in our CASE II (Fig. 3). We conclude that the roentgen features of apple-peel small bowel may vary depending on the morphology of the proximal segment and the exact level of the atresia. Weitzman and Vanderhoof report finding a normally rotated microcolon on barium enema study in one of their cases; however, no contrast studies were performed on our patients prior to operation. Surgical exploration has been undertaken in every case of apple-peel small bowel and has uniformly demonstrated complete atresia, involving the distal duo-
Fig. 3. CASE II. Abdominal roentgenogram in the erect position shows an air-fluid level in the stomach. Two more air-fluid levels are evident to the right of the stomach, partially superimposed on each other; these most likely represent the proximal (solid arrow) and distal duodenum (open arrow), respectively.
denum in 2 cases, the proximal jejunum in 13, and an unspecified site in 21. Of the 36 patients reported so far, 19 had malrotation or malfixation of the colon, 9 had other intestinal anomalies (mostly multiple atresias and stenoses), and only 5 had extraintestinal congenital abnormalities; this is similar to the distribution of associated anomalies in jejuno-ileal atresia (12). Patients with duodenal atresia, on the other hand, exhibit a much higher incidence of extra intestinal anomalies, such as Down's syndrome and congenital heart disease (11). A variety of surgical procedures has been performed for correction of the apple-peel small bowel, including end-to-end duodeno- or jejunojejunostomy (with or without resection), end-to-side or side-to-side anastomosis, or some type of "chimney" procedure (6). Of the 32 patients whose postoperative course is known, 20 (62.5 %) died, resulting in a higher mortality rate than that of either duodenal or jejunal atresia (4, 11, 12). The Bishop-Koop anastomosis has been associated with the highest survival rate, although the numbers are too small for a valid comparison. A common cause of postoperative death is anasto-
664
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C.
LEONIDAS AND OTHERS
March 1976
tive period, but extensive ischemic enteritis akin to the syndrome of neonatal necrotizing enterocolitis later developed. Pneumatosis intestinalis and massive accumulation of portal vein gas, seen in our first patient, have not been reported previously in apple-peel small-bowel syndrome (TABLE I; Fig. 4). The genetic implications of the apple-peel small bowel are of great interest. Mishalany and Najjar (8) described 3 siblings (2 boys and 1 girl) and possibly a fourth with this condition, all born of consanguineous parents. Blyth and Dickson (10) discuss 7 index patients belonging to 6 families; 4 of these infants were siblings (1 boy and 3 girls). All of these authors have suggested an autosomal recessive mode of inheritance. Both of our patients were firstborn. DISCUSSION
Fig. 4. CASE I. A. Abdominal roentgenograms obtained 2 months after end-to-end jejunojejunostomy and resection. Note the extensive pneumatosis intestinalis. Arrows indicate linear intramural air. B. Chest roentgenogram obtained a few hours later shows branching lucent areas over the liver, characteristic of hepatic portal vein gas (arrows). Gastric distension is also present.
motic leakage (9 patients) (2, 6), probably caused by poor blood supply to the distal atretic jejunal segment, which is the terminal segment supplied by the vascular axis. Both of our patients survived the initial postopera-
Recent work has proved that jejunal, ileal, and colonic atresia are the result of mechanical injuries to the fetal intestine, primarily vascular accidents (1, 2, 13, 14). The theory of failure of recanalization of the solidstate "epithelial plug" proposed by Tandler in 1900 (15) is probably applicable only to duodenal atresia (2, 16). The morphological features of the apple-peel syndrome lend additional support to the vascular theory of congenital intestinal atresia (1, 2, 7). Because of the high incidence of midgut malrotation, it is tempting to speculate that the syndrome is caused by prenatal volvulus; malrotation is not invariably present, however, and bile has been found in the bowel lumen distal to the atresia (7), indicating that the atresia occurs after the eleventh fetal week at a time when the rotation of the midgut is already complete (7, 17). The familial incidence of the apple-peel small bowel and associated anomalies in a number of cases indicate that it may represent an intrinsic abnormality of growth which predisposes to a prenatal vascular accident (6) such as compression or kinking of the SMA, an intrinsic arterial lesion, or volvulus (6, 7). The level of arterial occlusion corresponds to an area of intense embryologic activity and one or more of the above abnormalities are likely to occur. The presence of additional atresias in our cases emphasizes the importance of proving patency of the intestine distal to the obvious site of atresia at operation by instillation and passage of fluid through its length. Caution is also necessary to avoid positions that may further compromise the already precarious blood supply to the distal atretic segment. Although volvulus is theoretically possible, considering the lack of mesenteric attachments, it has not been reported due to the postoperative development of adhesions which prevent any type of bowel twisting; these were found at re-exploration in both our patients. It is possible, however, that the development of fibrous tissue in this area further compromises blood supply by constricting the course of the mesenteric vessels as they penetrate the intestinal wall.
DUODENOJEJUNAL ATRESIA
Vol. 118
REFERENCES 1. Santulli TV, Blanc WA: Congenital atresia of the intestine: pathogenesis and treatment. Ann Surg 154:939-948, Dec 1961 . 2. Weitzman JJ, Vanderhoof RS: Jejunal atresia with ageneSIS of the dorsal mesentery. With "Christmas tree" deformity of the small intestine. Am J Surg 111:443-449, Mar 1966 3. Nixon HH, Tawes R: Etiology and treatment of small intestinal atresia: analysis of a series of 127 jejunoileal atresias and comparison with 62 duodenal atreslas, Surgery 69:41-51, Jan 1971 4. Mellish P: Personal communication. [In] Rickham PP, Johnston JH: Neonatal Surgery. London, Butterworths, 1969, Chapt 27, p 316 5. Jimenez FA, Reiner L: Arteriographic findings in congenital abnormalities of the mesentery and intestines. Surg Gynecol Obstet 113:346-352, Sep 1961 6. Dickson JAS: Apple peel small bowel: an uncommon variant of duodenal and jejunal atresia. J Pediatl" Surg 5:595-600, Dec 1970 7. Zwiren GT, Andrews HG, Ahmann P: Jejunal atresia with agenesis of the dorsal mesentery. ("Apple peel small bowel"). J Pediatr Surg 7:414-419, Aug-Sep 1972 8. Mishalany HG, Najjar FB: Familial jejunal atresia: three cases in one family. J Pediatr 73:753-755, Nov 1968 ~. Benson CD, Lloyd JR: Atresia and stenosis of the jejunum and Ileum. [In] Mustard WT, Ravitch MM, Snyder WH Jr, et ai, ed: Pediatric Surgery. Chicago, Year Book, 2d Ed, 1969, Vol 2, Chapt 54, pp 841-851
665
Pediatric Radiology
10. Blyth H, Dickson JAS: Apple peel syndrome (congenital intestinal atresia). A family study of seven index patients. J Med Genet 6:275-277, Sep 1969 11. Fonkalsrud EW, deLorimier AA, Hays DM: Congenital atresia and stenosis of the duodenum. A review compiled from the members of the Surgical Section of the American Academy of Pediatrics. Pediatrics 43:79-83, Jan 1969 12. deLorimier AA, Fonkalsrud EW, Hays DM: Congenital atresia and stenosis of the jejunum and ileum. Surgery 65:819-827, May 1969 13. Blanc WA, Santulli TV, Andersen DH: Pathogenesis of jejunoileal atresia (abst). Am J Dis Child 98:564-565, Nov 1959 14. Louw JH, Barnard CN: Congenital atresia. Observations on its origin. Lancet 2: 1065-1 067, 19 Nov 1955 15. Tandler J: Zur Entwickelungsgeschichte des menschlichen Duodenums im fruhen embryonalstadien. Gegenbaurs Morphol Jahrb 29:187-216, 1900 (cited by Santulli and Blanc, ref. 1) 16. Moutsouris C: The "solid stage" and congenital intestinal atresia. J Pediatr Surg 1:446-450, Oct 1966 17. Snyder WH Jr, Chaffin L: Embryology and pathology of the intestinal tract: presentation of 40 cases of malrotation. Ann Surg 140:368-379, Sep 1954
Department of Radiology Children's Mercy Hospital 24th St. and Gillham Road Kansas City, Mo. 64108
Duodenojejunal Atresia with "Apple-Peel" Small Bowel
•
Pediatric Radiology
A DistinctForm of Intestinal Atresia 1 John C. Leonidas, M.D., Raymond A. Amoury, M.D., Keith W. Ashcraft, M.D., and Richard A. Fellows, M.D. Prenatal occlusion of the superior mesenteric artery results in a distinct type of distal duodenal or proximal jejunal atresia in which the dorsal mesentery is absent and the distal small bowel assumes a spiral configuration around its vascular stalk, strongly resembling an apple peel. In some instances the condition is transmitted genetically as an autosomal recessive disorder. The mortality rate is much higher than in either simple duodenal or jejunal atresia because of deficient collateral circulation to the small intestine. The authors tell of 2 patients who died of necrotizing enterocolitis, a disease related to vascular insufficiency. INDEX TERMS: ties
Arteries, mesenteric· Duodenum, abnormalities· Intestines, abnormali-
Radiology 118: 661-665, March 1976
• HE " APPLE-PEEL" form of intestinal atresia, first described by Santulli and Blanc in 1961 (1), consists of distal duodenal or proximal jejunal atresia with obliteration of the superior mesenteric artery a short distance beyond its origin and absence of the dorsal mesentery. Distal to the atresia, the small intestine is relatively short and small in caliber. It lies free in the peritoneal cavity, having no mesenteric attachment and being only distally connected to the cecum (2-7). Because of the typical spiral arrangement of small-bowel loops around a vascular stalk, the appearance has been described appropriately as "apple-peel" (1, 6), "Christmas-tree" (2), "maypole" (3), or "pagoda" (4) (Fig. 1). The condition is quite rare, with only 34 cases reported in the English literature by the end of 1972 (1-10). It was not described prior to 1961, although a few patients seen before that time have subsequently been recognized as having the apple-peel syndrome (8, 10). Many cases in the past, and perhaps even now, are erroneously diagnosed as duodenojejunal atresia occurring in association with midgut malrotation and prenatal volvulus (7, 8). The peculiar blood supply of the apple-peel small bowel is a constant feature which is of great value in clarifying its pathogenesis. Most of the distal superior mesenteric artery (SMA) is absent, including the straight pre-arterial arcades to the small intestine. The small bowel distal to the site of atresia derives its blood supply from collateral vessels formed by branches of the middle and right colic arteries and ileocolic artery (2, 5, 7). These vessels form a marginal artery which initially runs parallel to the right colon and then becomes the axis for the coiled distal small bowel (Figs. 1, Band 2). It is reasonable to assume that prenatal occlusion of the SMA distal to the origin of the middle colic artery causes aseptic necrosis and disappearance of part of the small bowel along with the dorsal mesentery (2, 6, 7) (Fig. 2).
•
T
Fig 1. CASE II. A. Apple-peel jejunal atresia. The surgical clamp on the left has been placed on the appendix. The small bowel distal to the site of atresia has been eviscerated and presents the characteristic "apple-peel" spiral configuration. B. Close-up of the small bowel, showing the mesenteric defect (upper arrow) and the vascular stalk carrying the collateral circulation just above the distal ileum (lower arrow).
Among the important features of the apple-peel small bowel are its familial incidence-probably through an autosomal recessive mode of inheritance (6,8, 10)-as
1 From the Departments of Radiology (J. C. L., R. A. F.) and Surgery (R. A. A., K. W. A.), Children's Mercy Hospital and the University of Missouri School of Medicine, Kansas City, Mo. Accepted for publication in August 1975. Presented at the Eighteenth Annual Meeting of the Society for Pediatric Radiology, Atlanta, Ga., Sept. 28-29, 1975. sjh
Fig. 2. A. Schematic drawing of the normal distribution of the superior mesenteric artery and its branches. Prenatal occlusion at the point indicated leads to "disappearance" of part of the jejunum and the dorsal mesentery. B. Diagram showing what happens when the circulation to the distal small bowel is maintained by collateral vessels (apple-peel small bowel). Table I: Case Sex F
Summary of Findings
Clinical Findings
Roentgenographic Findings
1 day
Premature {birth weight 1,745 g); bilestained vomitus
Complete duodenal obstruction ("double bubble")
2 mo.
Bloody stools and disten-
Pneumatosis intestinalis, hepatic portal vein gas {Fig. 4)
Age
Operative Findings and Procedures Double duodenal atresia; typical apple-peel small bowel; ma Irotation of the colon End-to-end duodenojejunostomy performed following excision of the third part of the duodenum and gastrostomy Total necrosis of the jejunum without volvuIus, compression, or obstruction Resection and duodenoileostomy Atresia of the proximal jejunurn: apple-peel small bowel with a second atresia in the distal jejunal segment Resection of the proximal part of the distal jejunum; reduction of the diameter of the duodenum and end-to-end duodenojejunostomy; qastrostorny Small bowel marginally viable with segmental ileal necrosis but no volvulus Abdominal exploration
sion
II
M
2 days
Premature (birth weight 1,600 g); bilestained vomitus
Distension of the entire duodenal loop; complete distal duodenal obstructi on
27 days
Bloody stools and distension
Generalized smallbowel distension
662
Course
Autopsy Findings
Total parenteral alimentation complicated by superior vena cava thrombosis and sepsis; persistent diarrhea
Died following the operation
Ischemic necrosis of the resected bowel; pulmonary atelectasis; superior vena cavathrombosis with recanalization
Initially good
Died following the operation
Ischemic necrotizing enteritis; peritonitis; pulmonary edema; intrahepatic cholestasis
Vol. 118
DUODENOJEJUNAL ATRESIA
663
Pediatric Radiology
well as associated anomalies (most frequently colonic malrotation) (2, 5, 6, 8) and a continuing tendency toward the development of ischemic enteritis. Two infants with apple-peel small bowel recently seen at The Children's Mercy Hospital in Kansas City form the basis of the present report. Similar cases reported in the literature were also reviewed and compared with our own. CASE MATERIAL AND REVIEW OF THE LITERATURE
The clinical, radiographic, and surgical features of our 2 cases are reviewed in TABLE I. Of the 34 patients reported in the literature, 9 were male and 16 were female; the sex of the other 9 patients was not mentioned (1, 4, 6, 9). Of the 24 patients whose birth weight was specified, 14 (58 %) weighed less than 2.5 kg (mean, 2.06 kg), while 10 (42%) weighed more than 2.5 kg (mean, 2.71 kg). No birth weight was stated for the other 10 infants (4, 6, 9), though 4 were described as "premature" (1, 5). No information is available regarding the gestational age of the infants with this syndrome. Comparison of these figures with those of Fonkalsrud et a/. (11) on congenital duodenal atresia and stenosis and deLorimier et a/. (12) on jejuno-ileal atresia and stenosis suggests that an equal sex ratio and a high incidence of prematurity are common in all of these disorders. Four infants with apple-peel small bowel also had a history of maternal polyhydramnios (2, 8). Symptoms and signs have invariably been those of postampullary high intestinal obstruction (gastric retention, bile-stained vomitus, and failure to pass meconium (2, 7, 8). Generalized abdominal distension may be present (5); more often, however, the abdomen is flat or distension is limited to the epigastrium (2, 8). The roentgenographic findings are those of complete duodenal or proximal jejunal obstruction (2, 7, 8). The patient in CASE I of Weitzman and Vanderhoof (2) had dilatation of the proximal duodenum caused by peritoneal bands as well as marked distension of the proximal duodenojejunal loop. The first of our 2 patients exhibited the characteristic "double bubble" of duodenal atresia, and this was confirmed surgically (TABLE I). The abdominal radiograph shown by Zwiren et a/. (7) reveals 3 to 4 upper abdominal gas bubbles with air-fluid levels, consistent with proximal jejunal atresia, and similar findings were present in the patient in our CASE II (Fig. 3). We conclude that the roentgen features of apple-peel small bowel may vary depending on the morphology of the proximal segment and the exact level of the atresia. Weitzman and Vanderhoof report finding a normally rotated microcolon on barium enema study in one of their cases; however, no contrast studies were performed on our patients prior to operation. Surgical exploration has been undertaken in every case of apple-peel small bowel and has uniformly demonstrated complete atresia, involving the distal duo-
Fig. 3. CASE II. Abdominal roentgenogram in the erect position shows an air-fluid level in the stomach. Two more air-fluid levels are evident to the right of the stomach, partially superimposed on each other; these most likely represent the proximal (solid arrow) and distal duodenum (open arrow), respectively.
denum in 2 cases, the proximal jejunum in 13, and an unspecified site in 21. Of the 36 patients reported so far, 19 had malrotation or malfixation of the colon, 9 had other intestinal anomalies (mostly multiple atresias and stenoses), and only 5 had extraintestinal congenital abnormalities; this is similar to the distribution of associated anomalies in jejuno-ileal atresia (12). Patients with duodenal atresia, on the other hand, exhibit a much higher incidence of extra intestinal anomalies, such as Down's syndrome and congenital heart disease (11). A variety of surgical procedures has been performed for correction of the apple-peel small bowel, including end-to-end duodeno- or jejunojejunostomy (with or without resection), end-to-side or side-to-side anastomosis, or some type of "chimney" procedure (6). Of the 32 patients whose postoperative course is known, 20 (62.5 %) died, resulting in a higher mortality rate than that of either duodenal or jejunal atresia (4, 11, 12). The Bishop-Koop anastomosis has been associated with the highest survival rate, although the numbers are too small for a valid comparison. A common cause of postoperative death is anasto-
664
JOHN
C.
LEONIDAS AND OTHERS
March 1976
tive period, but extensive ischemic enteritis akin to the syndrome of neonatal necrotizing enterocolitis later developed. Pneumatosis intestinalis and massive accumulation of portal vein gas, seen in our first patient, have not been reported previously in apple-peel small-bowel syndrome (TABLE I; Fig. 4). The genetic implications of the apple-peel small bowel are of great interest. Mishalany and Najjar (8) described 3 siblings (2 boys and 1 girl) and possibly a fourth with this condition, all born of consanguineous parents. Blyth and Dickson (10) discuss 7 index patients belonging to 6 families; 4 of these infants were siblings (1 boy and 3 girls). All of these authors have suggested an autosomal recessive mode of inheritance. Both of our patients were firstborn. DISCUSSION
Fig. 4. CASE I. A. Abdominal roentgenograms obtained 2 months after end-to-end jejunojejunostomy and resection. Note the extensive pneumatosis intestinalis. Arrows indicate linear intramural air. B. Chest roentgenogram obtained a few hours later shows branching lucent areas over the liver, characteristic of hepatic portal vein gas (arrows). Gastric distension is also present.
motic leakage (9 patients) (2, 6), probably caused by poor blood supply to the distal atretic jejunal segment, which is the terminal segment supplied by the vascular axis. Both of our patients survived the initial postopera-
Recent work has proved that jejunal, ileal, and colonic atresia are the result of mechanical injuries to the fetal intestine, primarily vascular accidents (1, 2, 13, 14). The theory of failure of recanalization of the solidstate "epithelial plug" proposed by Tandler in 1900 (15) is probably applicable only to duodenal atresia (2, 16). The morphological features of the apple-peel syndrome lend additional support to the vascular theory of congenital intestinal atresia (1, 2, 7). Because of the high incidence of midgut malrotation, it is tempting to speculate that the syndrome is caused by prenatal volvulus; malrotation is not invariably present, however, and bile has been found in the bowel lumen distal to the atresia (7), indicating that the atresia occurs after the eleventh fetal week at a time when the rotation of the midgut is already complete (7, 17). The familial incidence of the apple-peel small bowel and associated anomalies in a number of cases indicate that it may represent an intrinsic abnormality of growth which predisposes to a prenatal vascular accident (6) such as compression or kinking of the SMA, an intrinsic arterial lesion, or volvulus (6, 7). The level of arterial occlusion corresponds to an area of intense embryologic activity and one or more of the above abnormalities are likely to occur. The presence of additional atresias in our cases emphasizes the importance of proving patency of the intestine distal to the obvious site of atresia at operation by instillation and passage of fluid through its length. Caution is also necessary to avoid positions that may further compromise the already precarious blood supply to the distal atretic segment. Although volvulus is theoretically possible, considering the lack of mesenteric attachments, it has not been reported due to the postoperative development of adhesions which prevent any type of bowel twisting; these were found at re-exploration in both our patients. It is possible, however, that the development of fibrous tissue in this area further compromises blood supply by constricting the course of the mesenteric vessels as they penetrate the intestinal wall.
DUODENOJEJUNAL ATRESIA
Vol. 118
REFERENCES 1. Santulli TV, Blanc WA: Congenital atresia of the intestine: pathogenesis and treatment. Ann Surg 154:939-948, Dec 1961 . 2. Weitzman JJ, Vanderhoof RS: Jejunal atresia with ageneSIS of the dorsal mesentery. With "Christmas tree" deformity of the small intestine. Am J Surg 111:443-449, Mar 1966 3. Nixon HH, Tawes R: Etiology and treatment of small intestinal atresia: analysis of a series of 127 jejunoileal atresias and comparison with 62 duodenal atreslas, Surgery 69:41-51, Jan 1971 4. Mellish P: Personal communication. [In] Rickham PP, Johnston JH: Neonatal Surgery. London, Butterworths, 1969, Chapt 27, p 316 5. Jimenez FA, Reiner L: Arteriographic findings in congenital abnormalities of the mesentery and intestines. Surg Gynecol Obstet 113:346-352, Sep 1961 6. Dickson JAS: Apple peel small bowel: an uncommon variant of duodenal and jejunal atresia. J Pediatl" Surg 5:595-600, Dec 1970 7. Zwiren GT, Andrews HG, Ahmann P: Jejunal atresia with agenesis of the dorsal mesentery. ("Apple peel small bowel"). J Pediatr Surg 7:414-419, Aug-Sep 1972 8. Mishalany HG, Najjar FB: Familial jejunal atresia: three cases in one family. J Pediatr 73:753-755, Nov 1968 ~. Benson CD, Lloyd JR: Atresia and stenosis of the jejunum and Ileum. [In] Mustard WT, Ravitch MM, Snyder WH Jr, et ai, ed: Pediatric Surgery. Chicago, Year Book, 2d Ed, 1969, Vol 2, Chapt 54, pp 841-851
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Pediatric Radiology
10. Blyth H, Dickson JAS: Apple peel syndrome (congenital intestinal atresia). A family study of seven index patients. J Med Genet 6:275-277, Sep 1969 11. Fonkalsrud EW, deLorimier AA, Hays DM: Congenital atresia and stenosis of the duodenum. A review compiled from the members of the Surgical Section of the American Academy of Pediatrics. Pediatrics 43:79-83, Jan 1969 12. deLorimier AA, Fonkalsrud EW, Hays DM: Congenital atresia and stenosis of the jejunum and ileum. Surgery 65:819-827, May 1969 13. Blanc WA, Santulli TV, Andersen DH: Pathogenesis of jejunoileal atresia (abst). Am J Dis Child 98:564-565, Nov 1959 14. Louw JH, Barnard CN: Congenital atresia. Observations on its origin. Lancet 2: 1065-1 067, 19 Nov 1955 15. Tandler J: Zur Entwickelungsgeschichte des menschlichen Duodenums im fruhen embryonalstadien. Gegenbaurs Morphol Jahrb 29:187-216, 1900 (cited by Santulli and Blanc, ref. 1) 16. Moutsouris C: The "solid stage" and congenital intestinal atresia. J Pediatr Surg 1:446-450, Oct 1966 17. Snyder WH Jr, Chaffin L: Embryology and pathology of the intestinal tract: presentation of 40 cases of malrotation. Ann Surg 140:368-379, Sep 1954
Department of Radiology Children's Mercy Hospital 24th St. and Gillham Road Kansas City, Mo. 64108
