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A new surgical approach to duplications of the intestine
A New Surgical Approach to Duplications of the Intestine By R.W. Norris, R.J. Brereton, V.M. Wright, and R.E. Cudmore London and Liverpool England 9 T w o unusual intestinal duplications are described, In each case the mesenteric duplication had a separate muscular coat from the normal bowel permitting complete excision of the duplication without interference with the blood supply to the normal intestine. 9 1 9 8 6 by Grune & Stratton, Inc. INDEX W O R D S : Duplication cysts.
HE EMBRYOLOGICAL development of intesti-
T nal duplication systs has been well described I but the surgical significance of the process has not been fully appreciated. This is particularly important when dealing with large mesenteric duplication cysts or with very long tubular duplications of the small bowel. CASE REPORTS
Case 1 In 1979, a 2.5 kg girl was born at term to a 34-year-old primagravida with a ten-year history of infertility. Delivery was by emergency caesarean section because of fetal distress. The infant required external cardiac massage, intracardiac injection of adrenaline, and assisted ventilation. After a few hours, the infant had a convulsion and was subsequently transferred to a neonatal intensive care unit. On the fifth day, she passed a small amount of blood per rectum and was noted to have a distended abdomen, so necrotizing enterocolitis was diagnosed although there were no radiological features of this condition. She returned to the maternity unit at the age of 19 days. When 10 weeks old, she was admitted to the surgical unit at Alder Hey because of severe melena. The hemoglobin was 7.8 g/dL. Radiographs revealed midthoracic vertebral anomalies, so a diagnosis of intestinal duplication was made. At laparotomy, there was a long tubular duplication running through the mesentery of two thirds of the small bowel, arising 4 cm distal to the duodenojejunal flexure and joining the ileum 25 cm from the ileocecal valve (Fig 1). A standard operation, removing duplication and neighboring small bowel, would have necessitated the resection of 130 to 140 cm of normal bowel. On more detailed examination, it was seen that there was a clear plane of cleavage between the normal and the duplicated bowel for all but the distal 10 cm. Using careful dissection between the anterior and posterior mesenteric vessels, the duplicated bowel was removed from the small bowel mesentery. The terminal 10 cm was resected along with the attached normal ileum. There was an ulcer in the duplicated bowel in this region, and the mesentery was very indurated. The ileum was reconstituted by end-to-end anastomosis of interrupted inverting silk sutures. An inversion appendicectomy was performed. The infant was discharged after 10 days. A postoperative technetium scan of the chest was normal, and when last seen at the age of three years she had no gastrointestinal symptoms.
Case 2 In 1981, a 3.5 kg female infant was born at term to a 27-year-old multigravid Indian mother following an uncomplicated pregnancy.
Journal of Pediatric Surgery, Vol 21, No 2 (February), 1986: pp 167-170
The infant was noted to have a distended abdomen at birth, and at the age of 1 week an abdominal mass was easily palpable, filling most of the abdomen. Abdominal radiographs revealed a large well defined soft tissue mass with displacement of the intestines (Fig 2). There was also a left-sided posterior mediastinal mass (Fig 3) but there were no vertebral anomalies. Ultrasound examination confirmed that the lesions were cystic. An intravenous pyelogram was normal apart from inferior displacement of the right kidney. At the age of 9 weeks, the infant was transferred to Queen Elizabeth Hospital for further management. At laparotomy, a large 0.5 kg duplication cyst was found within the small bowel mesentery. In addition, there was a long tubular duplication of the jejunum, arising 20 cm from the duodenojejunal flexure. The proximal 10 cm shared a common seromuscular coat with the jejunum hut the next 20 cm lay separately within the mesentery. Distally, the duplication became progressively more distant from the normal bowel with the final 10 cm ending blindly, suspended by its own mesentery. Both normal bowel and tubular duplication were stretched over the duplication cyst. The tubular duplication and cyst were not communicating (Fig 4). The blood vessels to the normal bowel passed both anterior and posterior to the duplication cyst, so by careful dissection it was possible to develop a plane between the cyst and the vessels. Several of the anterior vessels had to be iigated, but the posterior leash of vessels was left undamaged and the cyst was enucleated without causing small bowel ischemia. The mesentery of the distal tubular duplication was ligated and the duplicated bowel over a 20 cm length was separated from the small bowel mesentery, leaving the posterior mesenteric vessels intact. The next 10 crn was separated by submucosal dissection of the duplication. The proximal 10 cm of tubular duplication could not be separated from the normal bowel, so both were resected together. Intestinal continuity was restored by end-to-end anastomosis using a single layer of interrupted silk sutures. Routine appendicectomy was performed. Histology revealed both cyst and tubular duplication to be lined by small intestinal mucosa. The infant made an uneventful recovery. Following excision of the abdominal component there was rapid enlargement of the left-sided mediastinal cyst (Fig 5). A thoracotomy was performed through the fifth left intercostal space. The cyst was dissected from the chest wall and numerous small feeding vessels required ligation. Inferiorly, the cyst narrowed to a tiny pedicle, which entered the left diaphragm and connected there with another small cyst, which appeared to lie within the diaphragm itself. Following the second operation, the child made a rapid recovery with impressive weight gain. Histology of the mediastinal cyst revealed the lining to be of gastric mucosa. At the age of two years, she is developing normally and is asymptomatic.
From the Department of Paediatric Surgery, Queen Elizabeth Hospitalfor Children, London; and Alder Hey Children's Hospital, Liverpool. Address reprint requests to R. J. Brereton, FRCS, Queen Elizabeth Hospital, Hackney Rd, London E2 8PS, England. 9 1986 by Grune & Stratton, Inc. 0022-3468/86/2102~9018503.00/0
167
168
9
NORRIS ET AL
4cm~ ~
Fig 2. Small bowel displaced to the left upper quadrant by a large right-sided abdominal mass in case 2.
Fig 1. A long tubular duplication throughout the small bowel mesentery in case 1.
end of the duplication should be anastomosed to the adjacent normal small bowel so allowing free drainage but after several decades there is a possibility of malignant degeneration within the residual duplication. 8 Basu and Forshall 9 considered that enucleation of a large cystic lesion was rarely feasible for fear of damage to the blood supply of the neighboring bowel, and Drennen 1~stated that such a procedure had never been successfully performed. Lister 4 reported that separation of the duplication from the intestine should not be contemplated as the two structures usually share a
DISCUSSION
Intestinal duplications have been recognized since 17332 and the largest personal series in the literature is of 67 cases collected over a period of 30 years. 3 It is generally agreed that the smaller cystic lesions and short tubular duplications of the small intestine are best treated by complete resection along with the adjacent normal bowel, continuity being restored by end-to-end anastomosis? However, the treatment of large cystic lesions or extensive tubular duplications (more than 35 cm long) remains controversial. The technique of mucosal stripping, devised to deal with very long tubular duplications, has been well described by Wrenn 5 and Lister. 6 In 1964, Bishop and Koop 7 suggested that the distal
Fig 3. case 2.
A medeastinal mass at the lateral border of the heart in
NEW SURGICAL APPROACH TO INTESTINAL DUPLICATIONS
169
Ocm 'common wall
Fig 4. A short tubular duplication and a large mesenteric cyst between the anterior and posterior leash of vessels (vasa recta) in case 2.
common muscular coat, and that attempted separation would render the adjacent bowel ischemic. The blood supply to the small bowel as described in the standard works of anatomy of 40 years ago is incorrect. Various patterns of arterial supply were described by Barlow, 1~and we now realize that the vasa recta from the terminal arcades pass to the wall of the gut either to the anterior or posterior surface, and not in the midline as was frequently described] 2 The terminal branches then anastomose within the wall of
posterior
vessels
the gut and can no longer be regarded as "end arteries. ''12-14 Bianchi 15 made use of these facts in his experiments on longitudinal division of the small bowel prior to intestinal lengthening in short bowel syndrome in pigs. Intestinal lengthening using this technique has now been performed in humans by Boeckman and Traylor 16 and one of the present authors. Our two cases show that in certain circumstances, particularly when there is not a common muscular wall, it is technically possible to remove either a tubular duplication or an enormous cyst from within the small bowel mesentery provided that one understands that the blood supply to the normal bowel passes as two leashes of vessels, one anterior and the other posterior, to the duplication. All of the anterior leash of vessels may be ligated in order to facilitate removal of the duplication provided the posterior vessels are left intact, or alternatively, either anterior or posterior vessels may be ligated provided this is done asynchronously and not more than 1 cm or so of small bowel is totally devascularized. Embryologically, tubular or cystic duplications arise within the leaves of the mesentery and do not interfere with the development of the blood supply to the small bowel. On both embryological and anatomical grounds, it is suggested that when removing duplications of the small bowel far more normal bowel may be preserved than has been realized in the past. REFERENCES
Fig 5. Enlargement of the mediastinal duplication prior t o throaeotomy in case 2.
1. Fallon M, Gordon ARG, Lendrum A: Mediastinal cysts of fore-gut origin associated with vertebral abnormalities. Br J Surg 41:520-533, 1954
170
2. Calder J: Two examples of children born with preternatural conformations of the guts. Medical Essays and Observations, Edinburgh 1:203-206, 1733 3. Gross RE: The Surgery of Infancy and Childhood. Philadelphia, Saunders, 1953, pp 221 245 4. Lister J. Rickham PP: Duplications of the alimentary tract, in Rickham PP, Lister J, Irving 1 (eds): Neonatal Surgery, (ed 2). London, Butterworth, 1978, pp 401-410 5. Wrenn EL: Tubular duplications of the small intestine. Surgery 52:494-498, 1962 6. Lister J: Duplications of the alimentary tract, in Rob C, Smith R, Operative Surgery, Nixon HH (consultant ed): Paediatric Surgery, (ed 3). London, Butterworth, 1978, pp 79 83 7. Bishop HC, Koop CE: Surgical management of duplications of the alimentary tract. Am J Surg 107:434-441, 1964 8. Orr MM, Edwards A J: Neoplastic change in duplications of the alimentary tract. Br J Surg 62:269 274, 1975
NORRIS ET AL
9. Basu R, Forshall I, Rickham PP: Duplications of the alimentary tract. Br J Surg 47:477-484, 1960 10. Drennen E: lleocecal cysts. Arch Surg 22:105-120, 1931 11. Barlow TE: Variations in the blood supply of the upper jejunum. Br J Surg 43:473-475, 1956 12. Doran FSA: The intramural blood supply of the upper jejunum in man. J Anat 84:283-286, 1950 13. Ross JA: Some observations on the course of arteries. MD Thesis, University of Edinburgh, 1947 14. Ross JA: Vascular patterns of small and large intestines compared. Br J Surg 39:33(~333, 1952 15. Bianchi A: Intestinal loop lengthening--a technique for increasing small intestinal length. J Pediatr Surg 15:145-151, 1980 16. Boeckman CR, Traylor R: Bowel lengthening for short gut syndrome. J Pediatr Surg 16:996 997, 1981
HE EMBRYOLOGICAL development of intesti-
T nal duplication systs has been well described I but the surgical significance of the process has not been fully appreciated. This is particularly important when dealing with large mesenteric duplication cysts or with very long tubular duplications of the small bowel. CASE REPORTS
Case 1 In 1979, a 2.5 kg girl was born at term to a 34-year-old primagravida with a ten-year history of infertility. Delivery was by emergency caesarean section because of fetal distress. The infant required external cardiac massage, intracardiac injection of adrenaline, and assisted ventilation. After a few hours, the infant had a convulsion and was subsequently transferred to a neonatal intensive care unit. On the fifth day, she passed a small amount of blood per rectum and was noted to have a distended abdomen, so necrotizing enterocolitis was diagnosed although there were no radiological features of this condition. She returned to the maternity unit at the age of 19 days. When 10 weeks old, she was admitted to the surgical unit at Alder Hey because of severe melena. The hemoglobin was 7.8 g/dL. Radiographs revealed midthoracic vertebral anomalies, so a diagnosis of intestinal duplication was made. At laparotomy, there was a long tubular duplication running through the mesentery of two thirds of the small bowel, arising 4 cm distal to the duodenojejunal flexure and joining the ileum 25 cm from the ileocecal valve (Fig 1). A standard operation, removing duplication and neighboring small bowel, would have necessitated the resection of 130 to 140 cm of normal bowel. On more detailed examination, it was seen that there was a clear plane of cleavage between the normal and the duplicated bowel for all but the distal 10 cm. Using careful dissection between the anterior and posterior mesenteric vessels, the duplicated bowel was removed from the small bowel mesentery. The terminal 10 cm was resected along with the attached normal ileum. There was an ulcer in the duplicated bowel in this region, and the mesentery was very indurated. The ileum was reconstituted by end-to-end anastomosis of interrupted inverting silk sutures. An inversion appendicectomy was performed. The infant was discharged after 10 days. A postoperative technetium scan of the chest was normal, and when last seen at the age of three years she had no gastrointestinal symptoms.
Case 2 In 1981, a 3.5 kg female infant was born at term to a 27-year-old multigravid Indian mother following an uncomplicated pregnancy.
Journal of Pediatric Surgery, Vol 21, No 2 (February), 1986: pp 167-170
The infant was noted to have a distended abdomen at birth, and at the age of 1 week an abdominal mass was easily palpable, filling most of the abdomen. Abdominal radiographs revealed a large well defined soft tissue mass with displacement of the intestines (Fig 2). There was also a left-sided posterior mediastinal mass (Fig 3) but there were no vertebral anomalies. Ultrasound examination confirmed that the lesions were cystic. An intravenous pyelogram was normal apart from inferior displacement of the right kidney. At the age of 9 weeks, the infant was transferred to Queen Elizabeth Hospital for further management. At laparotomy, a large 0.5 kg duplication cyst was found within the small bowel mesentery. In addition, there was a long tubular duplication of the jejunum, arising 20 cm from the duodenojejunal flexure. The proximal 10 cm shared a common seromuscular coat with the jejunum hut the next 20 cm lay separately within the mesentery. Distally, the duplication became progressively more distant from the normal bowel with the final 10 cm ending blindly, suspended by its own mesentery. Both normal bowel and tubular duplication were stretched over the duplication cyst. The tubular duplication and cyst were not communicating (Fig 4). The blood vessels to the normal bowel passed both anterior and posterior to the duplication cyst, so by careful dissection it was possible to develop a plane between the cyst and the vessels. Several of the anterior vessels had to be iigated, but the posterior leash of vessels was left undamaged and the cyst was enucleated without causing small bowel ischemia. The mesentery of the distal tubular duplication was ligated and the duplicated bowel over a 20 cm length was separated from the small bowel mesentery, leaving the posterior mesenteric vessels intact. The next 10 crn was separated by submucosal dissection of the duplication. The proximal 10 cm of tubular duplication could not be separated from the normal bowel, so both were resected together. Intestinal continuity was restored by end-to-end anastomosis using a single layer of interrupted silk sutures. Routine appendicectomy was performed. Histology revealed both cyst and tubular duplication to be lined by small intestinal mucosa. The infant made an uneventful recovery. Following excision of the abdominal component there was rapid enlargement of the left-sided mediastinal cyst (Fig 5). A thoracotomy was performed through the fifth left intercostal space. The cyst was dissected from the chest wall and numerous small feeding vessels required ligation. Inferiorly, the cyst narrowed to a tiny pedicle, which entered the left diaphragm and connected there with another small cyst, which appeared to lie within the diaphragm itself. Following the second operation, the child made a rapid recovery with impressive weight gain. Histology of the mediastinal cyst revealed the lining to be of gastric mucosa. At the age of two years, she is developing normally and is asymptomatic.
From the Department of Paediatric Surgery, Queen Elizabeth Hospitalfor Children, London; and Alder Hey Children's Hospital, Liverpool. Address reprint requests to R. J. Brereton, FRCS, Queen Elizabeth Hospital, Hackney Rd, London E2 8PS, England. 9 1986 by Grune & Stratton, Inc. 0022-3468/86/2102~9018503.00/0
167
168
9
NORRIS ET AL
4cm~ ~
Fig 2. Small bowel displaced to the left upper quadrant by a large right-sided abdominal mass in case 2.
Fig 1. A long tubular duplication throughout the small bowel mesentery in case 1.
end of the duplication should be anastomosed to the adjacent normal small bowel so allowing free drainage but after several decades there is a possibility of malignant degeneration within the residual duplication. 8 Basu and Forshall 9 considered that enucleation of a large cystic lesion was rarely feasible for fear of damage to the blood supply of the neighboring bowel, and Drennen 1~stated that such a procedure had never been successfully performed. Lister 4 reported that separation of the duplication from the intestine should not be contemplated as the two structures usually share a
DISCUSSION
Intestinal duplications have been recognized since 17332 and the largest personal series in the literature is of 67 cases collected over a period of 30 years. 3 It is generally agreed that the smaller cystic lesions and short tubular duplications of the small intestine are best treated by complete resection along with the adjacent normal bowel, continuity being restored by end-to-end anastomosis? However, the treatment of large cystic lesions or extensive tubular duplications (more than 35 cm long) remains controversial. The technique of mucosal stripping, devised to deal with very long tubular duplications, has been well described by Wrenn 5 and Lister. 6 In 1964, Bishop and Koop 7 suggested that the distal
Fig 3. case 2.
A medeastinal mass at the lateral border of the heart in
NEW SURGICAL APPROACH TO INTESTINAL DUPLICATIONS
169
Ocm 'common wall
Fig 4. A short tubular duplication and a large mesenteric cyst between the anterior and posterior leash of vessels (vasa recta) in case 2.
common muscular coat, and that attempted separation would render the adjacent bowel ischemic. The blood supply to the small bowel as described in the standard works of anatomy of 40 years ago is incorrect. Various patterns of arterial supply were described by Barlow, 1~and we now realize that the vasa recta from the terminal arcades pass to the wall of the gut either to the anterior or posterior surface, and not in the midline as was frequently described] 2 The terminal branches then anastomose within the wall of
posterior
vessels
the gut and can no longer be regarded as "end arteries. ''12-14 Bianchi 15 made use of these facts in his experiments on longitudinal division of the small bowel prior to intestinal lengthening in short bowel syndrome in pigs. Intestinal lengthening using this technique has now been performed in humans by Boeckman and Traylor 16 and one of the present authors. Our two cases show that in certain circumstances, particularly when there is not a common muscular wall, it is technically possible to remove either a tubular duplication or an enormous cyst from within the small bowel mesentery provided that one understands that the blood supply to the normal bowel passes as two leashes of vessels, one anterior and the other posterior, to the duplication. All of the anterior leash of vessels may be ligated in order to facilitate removal of the duplication provided the posterior vessels are left intact, or alternatively, either anterior or posterior vessels may be ligated provided this is done asynchronously and not more than 1 cm or so of small bowel is totally devascularized. Embryologically, tubular or cystic duplications arise within the leaves of the mesentery and do not interfere with the development of the blood supply to the small bowel. On both embryological and anatomical grounds, it is suggested that when removing duplications of the small bowel far more normal bowel may be preserved than has been realized in the past. REFERENCES
Fig 5. Enlargement of the mediastinal duplication prior t o throaeotomy in case 2.
1. Fallon M, Gordon ARG, Lendrum A: Mediastinal cysts of fore-gut origin associated with vertebral abnormalities. Br J Surg 41:520-533, 1954
170
2. Calder J: Two examples of children born with preternatural conformations of the guts. Medical Essays and Observations, Edinburgh 1:203-206, 1733 3. Gross RE: The Surgery of Infancy and Childhood. Philadelphia, Saunders, 1953, pp 221 245 4. Lister J. Rickham PP: Duplications of the alimentary tract, in Rickham PP, Lister J, Irving 1 (eds): Neonatal Surgery, (ed 2). London, Butterworth, 1978, pp 401-410 5. Wrenn EL: Tubular duplications of the small intestine. Surgery 52:494-498, 1962 6. Lister J: Duplications of the alimentary tract, in Rob C, Smith R, Operative Surgery, Nixon HH (consultant ed): Paediatric Surgery, (ed 3). London, Butterworth, 1978, pp 79 83 7. Bishop HC, Koop CE: Surgical management of duplications of the alimentary tract. Am J Surg 107:434-441, 1964 8. Orr MM, Edwards A J: Neoplastic change in duplications of the alimentary tract. Br J Surg 62:269 274, 1975
NORRIS ET AL
9. Basu R, Forshall I, Rickham PP: Duplications of the alimentary tract. Br J Surg 47:477-484, 1960 10. Drennen E: lleocecal cysts. Arch Surg 22:105-120, 1931 11. Barlow TE: Variations in the blood supply of the upper jejunum. Br J Surg 43:473-475, 1956 12. Doran FSA: The intramural blood supply of the upper jejunum in man. J Anat 84:283-286, 1950 13. Ross JA: Some observations on the course of arteries. MD Thesis, University of Edinburgh, 1947 14. Ross JA: Vascular patterns of small and large intestines compared. Br J Surg 39:33(~333, 1952 15. Bianchi A: Intestinal loop lengthening--a technique for increasing small intestinal length. J Pediatr Surg 15:145-151, 1980 16. Boeckman CR, Traylor R: Bowel lengthening for short gut syndrome. J Pediatr Surg 16:996 997, 1981