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Management of intestinal atresia complicating gastroschisis
Management of Intestinal Atresia Complicating Gastroschisis By Peter Gornall Birmingham, England 9 In a personal series of 22 patients referred with gastroschisis, five (23%) had intestinal atresia. The records of these patients seen over the past 12 years have been studied in order to elucidate the correct management of their complex problems. The indications for primary anastomosis or stoma formation probably depend upon the degree of damage and dilatation of the preatretic intestine at its initial presentation. In patients with a high jejunal or proximal ileal atresia, primary anastomosis should be attempted, but if the dilatation is severe, intestinal decompression may be required. In patients with distal ileal or colonic atresias, the establishment of a stoma and secondary closure is the t r e a t m e n t of choice. 9 1989 by Grune & Stratton, Inc. INDEX W O R D S : Gastroschisis; intestinal atresia.
I
N THE
MAJORITY
of patients with uncompli-
c a t e d g a s t r o s c h i s i s , t h e i n t e s t i n e a t b i r t h is s h o r t ,
a n d d a m a g e d b y l o n g c o n t a c t w i t h a m n i o t i c fluid. ~ I n a f e w p a t i e n t s , a t r e s i a in t h e e x t r u d e d i n t e s t i n e f u r t h e r complicates the pathology. This study was undertaken with the aim of identifying the problems encountered in t h e s e c a s e s , a n d c o r r e c t s u r g i c a l m a n a g e m e n t
of
some of them. MATERIALS A N D METHODS In the 12-year period from 1976 to 1987 inclusive, 22 patients with gastroschisis were referred to the author. Twenty-one patients, including five with intestinal atresia, had primary closure of the abdomen, and one patient had no operation because all the extruded intestine was gangrenous at the time of presentation. Fifteen patients, including two with intestinal atresia, required postoperative intermittent positive pressure ventilation (IPPV) for between 14 hours and nine days. Of six patients who did not need IPPV, three required continuous positive airways pressure for 7, 24, and 48 hours, and three who had intestinal atresia required no ventilatory support at all. One patient who was severely shocked at the time of presentation died at two days of age. Except for a patient who successfully fed enterally from the fifth day, all the rest required a period of total parenteral nutrition (TPN). The patients without atresias commenced enteral feeds successfully at a mean of 18 days (range, 5 to 56 days), and stayed in the hospital a mean of 40 days (range, 20 to 119 days). The patients with atresias stayed in hospital for a mean of 162 days.
From the Department of Surgery, The Children's Hospital, Ladywood Middleway, Birmingham, England. Presented at the 35th Annual Congress of the British Association of Paediatric Surgeons, Athens, September 21-23, 1988. Address reprint requests to Peter Gornall, MD, Department of Surgery, The Childrens Hospital, Ladywood Middleway, Birmingham B16 8ET, England. 9 1989 by Grune & Stratton, Inc. 0022-3468/89/2406-0003503.00/0 522
CASE REPORTS
Patient 1 A baby girl weighing 2.9 kg presented with all the extruded intestine encased in a greenish fibrinous mass, on the surface of which were four small intestinal stomas. During surgery, a dilated atretic segment of jejunum was found in the abdomen. There was a further atresia of septal type within the mass. The cause of the four stomas was two fistulae--one just proximal to the caecum and one just distal to it. The caecum was excised and three anastomoses were performed. In this case, the serosal surfaces of the bowel were not difficult to follow within the fibrinous mass. The abdomen was closed and the patient required no IPPV. A further operation was performed on day 24 for stenosis of the ileocolic anastomosis. Recovery was slow, with episodes of severe diarrhoea. Duodenal aspirates grew 12 x 106 coliform organisms, and enteral antibiotic treatment improved absorption. She was discharged at 151 days of age and remains well at 11 years of age.
Patient 2 A baby girl weighing 2.6 kg presented with markedly short oedematous intestine, and a distal ileal atresia. Six centimetres of terminal ileum with the caecum and colon were within the abdomen. A primary anastomosis was performed and the abdomen was closed. The patient required five days of IPPV. On 39th and 71st days, operations were performed to relieve intestinal obstruction apparently related to the anastomosis, This was found to be patent on each occasion, but angulated because of the disproportion of the proximal and distal bowel. On the 93rd day, an ileostomy was performed. Since the patient still could not be nourished enterally, on 184th day, this was converted to an end-to-side ileostomy (Bishop-Keep type2). The patient then began to lose very large quantities of gastric aspirate, and TPN became inadequate. She died on 193rd day.
Patient 3 A baby boy weighing 2.2 kg presented with all extruded intestine encased in fibrinous material. Colon emerging from the mass was seen to be very narrow. Dissection showed an atresia just distal to the caecum, which was resected, and an ileocolic anastomosis was made. The abdomen was closed and IPPV was not required. On 30th day, a cutaneous ileostomy was made to relieve intestinal obstruction caused by apparent stenosis of the anastomosis. The patient was slow to thrive with major fluid losses from the stoma. After 90 days, TPN was discontinued, and he was discharged at 121 days of age. The ileostomy was closed at the age of 11 months, and he is well at 3 years of age.
Patient 4 A baby girl weighing 2.77 kg presented with all extruded intestine encased in a mass of fibrinous material. The intestine in the pedicle was very narrow and atresia was suspected, especially as the intra-abdominal upper jejunum was grossly distended. However, dissection of the bowel was abandoned because no serosal layer could be followed accurately in the mass, which was therefore reduced into the abdomen. IPPV was required for five days. During surgery on 32nd day, a jejunal atresia was treated by jejuno-jejunostomy with tapering of the massively dilated proximal bowel. A colonic stenosis was treated by excision and anastomosis. Because of persistent
Journal of Pediatric Surgery, Vol 24, No 6 (June), 1989: pp 522-524
GASTROSCHISIS AND INTESTINAL ATRESIA
523
Table 1. S u m m a r y of Patient Histories Patient No.
Intestinal Abnormalities
IPPV
Intestinal Operations During First Admission
1
2 Jejunal atresia 1 Distal ileal fistula 1 Proximal colonic fistula 1 Distal ileal atresia 1 Proximal colonic atresia 1 Jejunal atresia 1 Colonic stenosis 1 Proximal colonic atresia
None
2
151
5 days None 5 days
4 2 4
193 (died) 121 298
None
1
2 3 4 5
intestinal obstruction, on the 82nd day the jejunal anastomosis was refashioned although it was not apparently narrow, and on 140th
day, an end-to-sidecutaneousjejunostomy (Bishop-Kooptype2) was made. Intestinal motility improvedslowlyand bacterial colonisation of the upper intestine required antibiotic control. On the 222nd day, the jejunostomywas closed. Continuing intestinal inertia was treated with some success with Cisapride.2~The patient was discharged on the 298th days. She is mildly developmentallydelayed. At age 2, her weight is in the tenth centile and she has mild chronic hydrocephalus, probably due to septicaemiafrom an infected TPN catheter.
Patient 5 A baby girl weighing 2.2 kg presented with all small intestine and caecum extruded, and an atresia just distal to the caecum. The descending colon was within the abdomen, and the transverse colon was absent. A colostomy was made and the abdomen closed without
tension. The stoma was rather slow to function, but the patient accepted enteral nourishment from 25th day, and was discharged on the 49th day. At 8 months of age, the colostomywas closed, and a rapid recoverywas made. She is well at 15 months of age. DISCUSSION
The incidence of intestinal atresia in gastroschisis of 23% in this series is the same as that given by Pokorny et al. 3 If total intestinal gangrene is excluded, others have found a lower incidence of 7% to 10%. 4'5 In a recent series of 11 patients diagnosed antenatally, two had intestinal atresias. 6 The cause of the atresias that is simplest to advance is pressure on the intestine from the edge of the defect in the abdominal wall. On occasions, this orifice may be extremely narrow with resulting gangrene of all the extruded intestine. While investigating the inertia of intestine in gastroschisis patients, Tibboel et al] found ischaemic changes which they attribute to contact with urinary products. In chick experimental models, such ischaemic changes have produced atresias. The dilatation proximal to an atresia may worsen the adverse effects of ischaemia on intestinal motility. Raffensperger and Jona 7 also have described laparotomies in a patient with corrected atresia and gastroschisis, in whom intestinal obstruction was due to inertia rather than anastomotic stenosis. However, there may be circumstances in which the intestine is, to a degree, protected from the toxic effects of amniotic fluid. The fibrinous mass enclosing the
Length of First Admission (days)
49
intestine distal to an atresia found in three patients of this series may be similar to the "sac" described by Amoury et a l : and the "ball of granulation tissue" described by Soper. 8 Both of their patients did well, as did the two of ours in whom dissection of intestine from the mass proved possible. In patient 1 the bowel dissected from the mass had an intact serosal layer, and was close in appearance and texture to the postatretic intestine of a neonate with a normal abdominal wall. It is emphasised that dissection within the mass is only required if failure of flow of intraluminally injected saline demonstrates an atresia. So far, no explanation of the formation of this fibrinous mass has been advanced. The intestine that is retained within the abdomen may be in part protected. Currie 9 reported that primary anastomosis worked well in a patient in whom the retained proximal intestine may have suffered less damage than the exposed intestine of patient 4. In the latter, the highly vascular granulation tissue encasing the extruded intestine rendered dissection and precise diagnosis impossible. A month after the mass had been enclosed in the abdomen, dissection was possible, and the atresia and stenosis were discovered. However, the segment of intestine proximal to the atresia was badly damaged, dilated, and inert. It is arguable that a cutaneous jejunostomy should have been performed at that time, as one had to be performed 31/2months later. An alternative at the time of the initial operation would have been decompressive jejunal intubation, as described by Pokorny et al 3 in one of their patients; this was done for 2 weeks, prior to anastomosis, with a good result. Therefore, it is suggested that a careful assessment of the state of the intestine be made in a case of jejunal or upper ileal atresia. Where the intestine proximal to the atresia has been retained within the abdomen, primary anastomosis may be justified. However, if the proximal intestine emerges through the abdominal defect, and has a grossly thickened muscularis as well as severe dilatation, a cutaneous enterostomy may be wiser.S In distal ileal and colonic atresia, and especially
524
PETER GORNALL
where the intestine is damaged both by amniotic exposure and pre-atretic dilatation, a temporary ileostomy or colostomy appears to offer advantages. Pokorny et al, 3 in an impressive series, had three such patients who did well. We felt that our third patient would have done as well as our fifth if an ileostomy had been performed at the initial operation and not a month later. Our second patient had severe intestinal inertia. After the finding of a short, very thickened intestine at the time of presentation, an initial ileostomy would have saved anxieties over the differential diagnosis of intestinal inertia and anastomotic stricture, and she might have survived. Only the patient who died (patient 2), and the
survivor with the longest hospital stay, and worst morbidity (patient 4) required IPPV. If the degree of oedema in damaged proximal intestine is such that the abdomen will be closed only with tension, the prognosis of prolonged intestinal inertia prompts the formation of a temporary ileostomy or colostomy rather than a primary anastomosis. Distension and inertia in the upper jejunum has caused bacterial overgrowth requiring antibiotic control in both patients with high atresias. We would recommend duodenal microbial sampling in all patients with treated high atresias as an aid to nutritional management when malabsorption is suspected.
REFERENCES
1. TibboelD, Raine P, McNee M, et al: Developmentalaspects of gastroschisis. J Pediatr Surg 21:865-869, 1986 2. BishopHC, KoopCE: Management of meconiumileus: resection, roux-en-yanastomosisand ileostomyirrigation with pancreatic enzymes. Ann Surg 145:410-414, 1957. 2a. CohenNP, BoothIW, Parashar K, et al: Successfulmanagement of idiopathic intestinal pseudo-obstruction with Cisapride. J Pediatr Surg 23:229-230, 1988 3. PokornyWJ, Harberg FJ, McGill CW: Gastroschisis complicated by intestinal atresia. J Pediatr Surg 16:261-263,1981 4. Di Lorenzo M, Yazbeck S, Ducharme J-C: Gastroschisis: A 15-year experience. J Pediatr Surg 22:710-712, 1987
5. AmouryRA, AshcraftKW, HolderTM: Gastroschisiscomplicated by intestinal atresia. Surgery 82:373-381, 1977 6. BondSJ, Harrison MR, Filly RA, et al: Severityof intestinal damage in gastroschisis:correlation with prenatal sonographicfindings. J Pediatr Surg 23:520-525, 1988 7. Raffensperger JG, Jona JZ: Gastroschisis. Surg Gynecol Obstet 138:230-234, 1974 8. SoperRT: Discussionof BinningtonHB, Keating JP, Ternberg JL: Gastroschisis. Arch Surg 108:455-459,1974 9. Currie ABM: Gastroschisis. J R Coil Surg Edinb 18:37-41, 1973
I
N THE
MAJORITY
of patients with uncompli-
c a t e d g a s t r o s c h i s i s , t h e i n t e s t i n e a t b i r t h is s h o r t ,
a n d d a m a g e d b y l o n g c o n t a c t w i t h a m n i o t i c fluid. ~ I n a f e w p a t i e n t s , a t r e s i a in t h e e x t r u d e d i n t e s t i n e f u r t h e r complicates the pathology. This study was undertaken with the aim of identifying the problems encountered in t h e s e c a s e s , a n d c o r r e c t s u r g i c a l m a n a g e m e n t
of
some of them. MATERIALS A N D METHODS In the 12-year period from 1976 to 1987 inclusive, 22 patients with gastroschisis were referred to the author. Twenty-one patients, including five with intestinal atresia, had primary closure of the abdomen, and one patient had no operation because all the extruded intestine was gangrenous at the time of presentation. Fifteen patients, including two with intestinal atresia, required postoperative intermittent positive pressure ventilation (IPPV) for between 14 hours and nine days. Of six patients who did not need IPPV, three required continuous positive airways pressure for 7, 24, and 48 hours, and three who had intestinal atresia required no ventilatory support at all. One patient who was severely shocked at the time of presentation died at two days of age. Except for a patient who successfully fed enterally from the fifth day, all the rest required a period of total parenteral nutrition (TPN). The patients without atresias commenced enteral feeds successfully at a mean of 18 days (range, 5 to 56 days), and stayed in the hospital a mean of 40 days (range, 20 to 119 days). The patients with atresias stayed in hospital for a mean of 162 days.
From the Department of Surgery, The Children's Hospital, Ladywood Middleway, Birmingham, England. Presented at the 35th Annual Congress of the British Association of Paediatric Surgeons, Athens, September 21-23, 1988. Address reprint requests to Peter Gornall, MD, Department of Surgery, The Childrens Hospital, Ladywood Middleway, Birmingham B16 8ET, England. 9 1989 by Grune & Stratton, Inc. 0022-3468/89/2406-0003503.00/0 522
CASE REPORTS
Patient 1 A baby girl weighing 2.9 kg presented with all the extruded intestine encased in a greenish fibrinous mass, on the surface of which were four small intestinal stomas. During surgery, a dilated atretic segment of jejunum was found in the abdomen. There was a further atresia of septal type within the mass. The cause of the four stomas was two fistulae--one just proximal to the caecum and one just distal to it. The caecum was excised and three anastomoses were performed. In this case, the serosal surfaces of the bowel were not difficult to follow within the fibrinous mass. The abdomen was closed and the patient required no IPPV. A further operation was performed on day 24 for stenosis of the ileocolic anastomosis. Recovery was slow, with episodes of severe diarrhoea. Duodenal aspirates grew 12 x 106 coliform organisms, and enteral antibiotic treatment improved absorption. She was discharged at 151 days of age and remains well at 11 years of age.
Patient 2 A baby girl weighing 2.6 kg presented with markedly short oedematous intestine, and a distal ileal atresia. Six centimetres of terminal ileum with the caecum and colon were within the abdomen. A primary anastomosis was performed and the abdomen was closed. The patient required five days of IPPV. On 39th and 71st days, operations were performed to relieve intestinal obstruction apparently related to the anastomosis, This was found to be patent on each occasion, but angulated because of the disproportion of the proximal and distal bowel. On the 93rd day, an ileostomy was performed. Since the patient still could not be nourished enterally, on 184th day, this was converted to an end-to-side ileostomy (Bishop-Keep type2). The patient then began to lose very large quantities of gastric aspirate, and TPN became inadequate. She died on 193rd day.
Patient 3 A baby boy weighing 2.2 kg presented with all extruded intestine encased in fibrinous material. Colon emerging from the mass was seen to be very narrow. Dissection showed an atresia just distal to the caecum, which was resected, and an ileocolic anastomosis was made. The abdomen was closed and IPPV was not required. On 30th day, a cutaneous ileostomy was made to relieve intestinal obstruction caused by apparent stenosis of the anastomosis. The patient was slow to thrive with major fluid losses from the stoma. After 90 days, TPN was discontinued, and he was discharged at 121 days of age. The ileostomy was closed at the age of 11 months, and he is well at 3 years of age.
Patient 4 A baby girl weighing 2.77 kg presented with all extruded intestine encased in a mass of fibrinous material. The intestine in the pedicle was very narrow and atresia was suspected, especially as the intra-abdominal upper jejunum was grossly distended. However, dissection of the bowel was abandoned because no serosal layer could be followed accurately in the mass, which was therefore reduced into the abdomen. IPPV was required for five days. During surgery on 32nd day, a jejunal atresia was treated by jejuno-jejunostomy with tapering of the massively dilated proximal bowel. A colonic stenosis was treated by excision and anastomosis. Because of persistent
Journal of Pediatric Surgery, Vol 24, No 6 (June), 1989: pp 522-524
GASTROSCHISIS AND INTESTINAL ATRESIA
523
Table 1. S u m m a r y of Patient Histories Patient No.
Intestinal Abnormalities
IPPV
Intestinal Operations During First Admission
1
2 Jejunal atresia 1 Distal ileal fistula 1 Proximal colonic fistula 1 Distal ileal atresia 1 Proximal colonic atresia 1 Jejunal atresia 1 Colonic stenosis 1 Proximal colonic atresia
None
2
151
5 days None 5 days
4 2 4
193 (died) 121 298
None
1
2 3 4 5
intestinal obstruction, on the 82nd day the jejunal anastomosis was refashioned although it was not apparently narrow, and on 140th
day, an end-to-sidecutaneousjejunostomy (Bishop-Kooptype2) was made. Intestinal motility improvedslowlyand bacterial colonisation of the upper intestine required antibiotic control. On the 222nd day, the jejunostomywas closed. Continuing intestinal inertia was treated with some success with Cisapride.2~The patient was discharged on the 298th days. She is mildly developmentallydelayed. At age 2, her weight is in the tenth centile and she has mild chronic hydrocephalus, probably due to septicaemiafrom an infected TPN catheter.
Patient 5 A baby girl weighing 2.2 kg presented with all small intestine and caecum extruded, and an atresia just distal to the caecum. The descending colon was within the abdomen, and the transverse colon was absent. A colostomy was made and the abdomen closed without
tension. The stoma was rather slow to function, but the patient accepted enteral nourishment from 25th day, and was discharged on the 49th day. At 8 months of age, the colostomywas closed, and a rapid recoverywas made. She is well at 15 months of age. DISCUSSION
The incidence of intestinal atresia in gastroschisis of 23% in this series is the same as that given by Pokorny et al. 3 If total intestinal gangrene is excluded, others have found a lower incidence of 7% to 10%. 4'5 In a recent series of 11 patients diagnosed antenatally, two had intestinal atresias. 6 The cause of the atresias that is simplest to advance is pressure on the intestine from the edge of the defect in the abdominal wall. On occasions, this orifice may be extremely narrow with resulting gangrene of all the extruded intestine. While investigating the inertia of intestine in gastroschisis patients, Tibboel et al] found ischaemic changes which they attribute to contact with urinary products. In chick experimental models, such ischaemic changes have produced atresias. The dilatation proximal to an atresia may worsen the adverse effects of ischaemia on intestinal motility. Raffensperger and Jona 7 also have described laparotomies in a patient with corrected atresia and gastroschisis, in whom intestinal obstruction was due to inertia rather than anastomotic stenosis. However, there may be circumstances in which the intestine is, to a degree, protected from the toxic effects of amniotic fluid. The fibrinous mass enclosing the
Length of First Admission (days)
49
intestine distal to an atresia found in three patients of this series may be similar to the "sac" described by Amoury et a l : and the "ball of granulation tissue" described by Soper. 8 Both of their patients did well, as did the two of ours in whom dissection of intestine from the mass proved possible. In patient 1 the bowel dissected from the mass had an intact serosal layer, and was close in appearance and texture to the postatretic intestine of a neonate with a normal abdominal wall. It is emphasised that dissection within the mass is only required if failure of flow of intraluminally injected saline demonstrates an atresia. So far, no explanation of the formation of this fibrinous mass has been advanced. The intestine that is retained within the abdomen may be in part protected. Currie 9 reported that primary anastomosis worked well in a patient in whom the retained proximal intestine may have suffered less damage than the exposed intestine of patient 4. In the latter, the highly vascular granulation tissue encasing the extruded intestine rendered dissection and precise diagnosis impossible. A month after the mass had been enclosed in the abdomen, dissection was possible, and the atresia and stenosis were discovered. However, the segment of intestine proximal to the atresia was badly damaged, dilated, and inert. It is arguable that a cutaneous jejunostomy should have been performed at that time, as one had to be performed 31/2months later. An alternative at the time of the initial operation would have been decompressive jejunal intubation, as described by Pokorny et al 3 in one of their patients; this was done for 2 weeks, prior to anastomosis, with a good result. Therefore, it is suggested that a careful assessment of the state of the intestine be made in a case of jejunal or upper ileal atresia. Where the intestine proximal to the atresia has been retained within the abdomen, primary anastomosis may be justified. However, if the proximal intestine emerges through the abdominal defect, and has a grossly thickened muscularis as well as severe dilatation, a cutaneous enterostomy may be wiser.S In distal ileal and colonic atresia, and especially
524
PETER GORNALL
where the intestine is damaged both by amniotic exposure and pre-atretic dilatation, a temporary ileostomy or colostomy appears to offer advantages. Pokorny et al, 3 in an impressive series, had three such patients who did well. We felt that our third patient would have done as well as our fifth if an ileostomy had been performed at the initial operation and not a month later. Our second patient had severe intestinal inertia. After the finding of a short, very thickened intestine at the time of presentation, an initial ileostomy would have saved anxieties over the differential diagnosis of intestinal inertia and anastomotic stricture, and she might have survived. Only the patient who died (patient 2), and the
survivor with the longest hospital stay, and worst morbidity (patient 4) required IPPV. If the degree of oedema in damaged proximal intestine is such that the abdomen will be closed only with tension, the prognosis of prolonged intestinal inertia prompts the formation of a temporary ileostomy or colostomy rather than a primary anastomosis. Distension and inertia in the upper jejunum has caused bacterial overgrowth requiring antibiotic control in both patients with high atresias. We would recommend duodenal microbial sampling in all patients with treated high atresias as an aid to nutritional management when malabsorption is suspected.
REFERENCES
1. TibboelD, Raine P, McNee M, et al: Developmentalaspects of gastroschisis. J Pediatr Surg 21:865-869, 1986 2. BishopHC, KoopCE: Management of meconiumileus: resection, roux-en-yanastomosisand ileostomyirrigation with pancreatic enzymes. Ann Surg 145:410-414, 1957. 2a. CohenNP, BoothIW, Parashar K, et al: Successfulmanagement of idiopathic intestinal pseudo-obstruction with Cisapride. J Pediatr Surg 23:229-230, 1988 3. PokornyWJ, Harberg FJ, McGill CW: Gastroschisis complicated by intestinal atresia. J Pediatr Surg 16:261-263,1981 4. Di Lorenzo M, Yazbeck S, Ducharme J-C: Gastroschisis: A 15-year experience. J Pediatr Surg 22:710-712, 1987
5. AmouryRA, AshcraftKW, HolderTM: Gastroschisiscomplicated by intestinal atresia. Surgery 82:373-381, 1977 6. BondSJ, Harrison MR, Filly RA, et al: Severityof intestinal damage in gastroschisis:correlation with prenatal sonographicfindings. J Pediatr Surg 23:520-525, 1988 7. Raffensperger JG, Jona JZ: Gastroschisis. Surg Gynecol Obstet 138:230-234, 1974 8. SoperRT: Discussionof BinningtonHB, Keating JP, Ternberg JL: Gastroschisis. Arch Surg 108:455-459,1974 9. Currie ABM: Gastroschisis. J R Coil Surg Edinb 18:37-41, 1973