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Gastroschisis and intestinal atresia
Gastroschisis
and Intestinal
By Rajkumar
Shah and Morton
Atresia
M. Woolley
Los Angeles, California @Controversy exists over the best method of treating gastroschisis with concomitant intestinal atresia because the mortality in such patients is still high. We present our experience of 74 neonates with gastroschisis seen over a 17-year period. Four patients (5.5%) had intestinal atresia; 1 had only one small bowel atresia, 2 had double areas of atresia (one in the mid small bowel and the other in the proximal colon), and the remaining 1 had multiple areas of atresia. Atresia with gastroschisis conventionally has been treated by either primary anastmosis or by exteriorization. Three of the patients were treated by replacing the atretic bowel into the abdomen initially and exploring later (1 to 3 weeks) when the bowel appeared much more healthy, making the anastomosis easier and more secure. One patient with extreme jejunal dilatation was treated by creation of a Mikulicz fistula. All 4 of the patients survived the newborn period, but one of them died at 1 year of age due to total parenteral nutrition-induced chronic liver failure. Our experience is presented to emphasize this uncommonly used approach to this complex problem because it seems to be associated with a lower morbidity and mortality than other conventional approaches. Copyright o 1991 by W.B. Saunders Company
these patients the defect was located to the left of the umbilicus. Four (5.4%) had associated intestinal atresia. Twenty-three (31%) underwent primary closure including the fascia, 17 (23%) underwent primary skin closure alone, and the remaining 34 (46%) had a silon chimney placed. The mean duration between placement of the silon and definitive closure was 12 days (range, 5 to 32 days). A gastrostomy tube had been placed in 18 patients (24.3%) all of whom were operated on prior to 1976. A total of 11 neonates died (14.8%). Seven of these deaths occurred before 1976. The causes of death included sepsis due to gangrene of intestine at or soon after birth (4) late sepsis (3; Pseudomonas bronchopneumonia, bowel necrosis of uncertain etiology, and enterocutaneous fistula), necrotizing enterocolitis (2) respiratory distress syndrome (l), and shock (1). The mean age at death was 19 days. Of these, 6 were less than 8 days old, 2 were 22 to 23 days old, and 3 were 47 to 49 days old. Of the patients with associated intestinal atresia, 1 had only one small bowel atresia, 2 had double areas of atresia (one in the mid-small bowel and the other in the proximal colon), and 1 had multiple areas of atresia. A brief description of the management of the patients with atresia is given below.
Case Reports INDEX WORDS:
Gastroschisis;
intestinal atresia.
G
ASTROSCHISIS is a congenital protrusion of part of the abdominal contents through an anterior abdominal wall defect usually located just to the right of the umbilicus. The term, derived from Greek origin, means “belly cleft.” Its location, the absence of a sac, and presence of thickened, foreshortened, matted gut differentiates it from an ompahalocele. An association between gastroschisis and intestinal atresia has been well documented. Recent reports’.’ have indicated a high incidence (23%) of this association. We report a series of neonates with gastroschisis seen over a 17-year period, 4 of whom had associated intestinal atresia (5.4%). MATERIALS
AND
METHODS
In the 17-year period from 1972 to 1989 inclusive, 74 neonates with gastroschisis were admitted to the Childrens Hospital of Los Angeles; 46 (62.1%) were boys and 28 (37.9%) were girls. In two of
From the Division of Pediatric Surgery, Department of Surgery, Children’s Hospital of Los Angeles, and the Depatiment of Surgery, University of Southern California School of Medicine, Los Angeles, CA. Presented at the 23rd Annual Meeting of the Pacific Association of Pediatric Surgeons, Kona, Hawaii, June 3-6, 1990. Address reprint requests to Morton M. Wool&y, MD, 4650 Sunset Blvd, Los Angeles, CA 900276062. Copyright o 1991 by WB. Saunders Company 0022-3468/91/2607-0009$03.00/0 788
Case 1. A full-term female infant was born to a heroin-addicted mother whose pregnancy was complicated by polyhydramnios. Emergency surgery was performed for gastroschisis and a markedly dilated loop of distal jejunum was found that ended abruptly at the point of bowel pertrusion through the defect. The extruded gut was a very short segment, blind at both ends, and was resected. After decompressing the jejunum and resecting it to a level with good peristalsis, a Mikulicz fistula was created because of the marked size disparity in the proximal and distal lumen. Eighteen days later the fistula was closed. Apart from laparotomy for resection of an anastomotic stricture, the child had a steady recovery. Case 2. A 2-kg female product of a 36-week gestation was born with gastroschisis. At surgery it was found that there was an area of atresia at the site of entry and exit of the bowel from the abdomen. The proximal site was in the mid-small bowel and the distal site was in the proximal colon. Because of marked edema and matting of the b owel, the atretic segments were replaced into the abdomen, thereby avoiding the risks of a primary anastomosis and problems of a stoma. Twenty days later, she underwent laparotomy with resection and anastomosis of the atretic segments. At this time the intestines appeared healthier making anastmosis easier and more secure. Oral feeds were initiated after 5 weeks and were tolerated remarkably well. Case 3. A 1,860-g, 35week gestation boy was born by cesarean section in a pregnancy complicated by twins, preeclampsia, and breech presentation. At laparotomy, two atretic segments were seen at the base of the gastroschisis defect (midjejunum and colon). Because of doubtful viability of the gut, the atretic segments were replaced into the abdomen and the defect was repaired. One week later, the child was doing well and the abdomen was reexplored. At this time, intestinal perfusion had markedly improved and the edema had decreased, allowing resection and anastomosis of the atretic segments. Postoperatively the child tolerated feeds very slowly and was discharged 81 days after admission. Journalof Pediatric Surgery, Vol26,
No 7 (July), 1991: pp
788-790
GASTROSCHISIS
AND INTESTINAL
ATRESIA
Case 4. A 2,850-kg female product of an uncomplicated pregnancy was born with gastroschisis. There was marked edema, foreshortening, and matting of the gut, and atresia could not be ruled out. Emergency placement of a silon chimney was done. This was progressively tightened and ultimately removed 2 weeks later when a full-thickness abdominal wall repair was done; however, 3 days later the child developed abdominal wall erythema and induration with systemic sepsis. The abdomen was reexplored and a large length of necrotic ileum was found. Multiple areas of atresia were also identified at this time involving the large and small bowel. There was no terminal ileum and the cecum ended blindly. After resection, only 38 cm of small bowel was left, which was anastomosed to the duodenum proximally and the transverse colon distally. Histological examination of the resected bowel showed hemorrhagic necrosis, ulcers, perforations, severe chronic enteritis and multiple atresias. The postoperative course was complicated by anastomotic leak followed by stricture formation necessitating reexploration and revision of the anastomosis. At this time an area of partial weblike obstruction was also found in the jejunum, which was corrected. The infant was maintained on total parenteral nutrition, which was complicated by severe cholestasis, and ultimately died of chronic liver failure and a severe fungal septicemia at 1 year of age.
DISCUSSION
Gastroschisis was first clearly described by Bernstsein in 1940.3 The incidence of intestinal atresia complicating gastroschisis has been reported to range from 6.8%4 to as high as 23%.‘,’ In a collective review of 200 patients with gastroschisis done in 1977,’ the incidence of atresia was 13%.2 Our experience involving 74 patients from a single hospital has shown an incidence of only 5.5%. Atresias complicating gastroschisis may be single’.‘.4 or multiple’ and may involve the small bowel or the large bowel.‘.2 It has been indicated in the past that the colon is an uncommon site for atresia. However, three of four patients in the present study had involvement of the colon. Weblike obstructions associated with obvious atretic segments have also been reported.‘,4 Of these four patients, one had a weblike partial obstruction of the jejunum. It is important to ensure that these are not overlooked at the time of definitive intestinal repair. The exact etiology of intestinal atresia is still uncertain. It is certainly possible that the atresia may be the result of a pinching effect of the defect on the prolapsed intestine.h Vascular accidents due to an intrauterine volvulus probably account for some of the cases in which the level of atresia is not at the level of the defect. The diagnosis of atresia is usually not a problem, although in one of these cases the extreme matting and edema prevented early recognition. This problem has been reported in the literature,5 although in that case it was a weblike atresia. However, extensive dissection to search for an atresia is not recommended,’ because any occult atresia would manifest later in the patient’s course and can
789
be dealt with at that time, when the intestine is more normal in appearance. The management of intestinal atresia complicating gastroschisis remains controversial. This is chiefly due to its low incidence, which prevents a large experience with the problem. It has been suggested that when the intestine is damaged by preatretic dilatation or by exposure to the amniotic fluid, a temporary proximal ileostomy would be the safest course,1-3 followed by subsequent delayed anastomosis. Muraji et al7 also performed ileostomies on their patients with atresia. This approach carries the disadvantage of enterostomy fluid loss, electrolyte imbalance, skin excoriation, and potential sepsis. This also carries obvious problems if placement of a silon is required. Bypass of the atretic segment has also been used.’ Amoury et al5 have recommended resection and anastomosis as the procedure of choice and recommend exteriorization only when the viability of the gut is in question or if there is a proximal perforation. Due to the unhealthy bowel wall, this not only can be technically difficult but will carry a high risk of anastomotic leakage. The technique of replacing the intestine into the abdomen without either exteriorization or primary anastomosis has been adopted,‘.’ although not frequently. We have adopted this technique in three of the four patients and have been impressed by the appearance of the bowel at the time of reexploration. It has been noted by others that the intestine appears nearly normal within 2 to 4 weeks.‘.’ We have observed the intestine to appear healthier and easier to dissect if the repair is delayed. However, we do not recommend this method if there is massive dilatation of the proximal gut. In such cases a proximal enterostomy must be performed. This would also be required in the rare event of a proximal perforation. Although the technique of replacing atretic bowel necessitates a second operation, it eliminates the problems of a stoma and the risks of a primary anastomosis. An additional advantage is that it may be difficult to decide whether an area of narrowing is due to edema or atresia. Replacing the bowel will result in resolution of the problem if it is one of edema, thereby avoiding unnecessary resection or exteriorization. In patients with atresia in more than one area, there does not appear to be a problem with the midsegment getting distended with enteral secretions, although this is a theoretical possibility. Return of gut motility in all patients with gastroschisis is slow, and advancement of feeds has to be very gradual The prognosis of atresia complicating gastroschisis has been reported to be poor. Amoury et al’ have suggested that the success or failure is related
SHAH AND WOOLLEY
790
more to the length of remaining intestine than to any specific method of treatment. Although this is true to an extent, we feel that delaying the primary anastomosis and avoiding stomas tends to improve the survival by eliminating problems of early anastomotic leaks and sepsis due to enterostomies. All six of the patients with atresia in the series reported by Hollabaugh and Boles’ were treated by resection, and none survived. All four patients in this series survived the neonatal period and the complications of case 4 are
related to the necosis rather than the atresia. The cause of death in case 4 was severe chronic liver failure subsequent to short gut syndrome and total parenteral nutrition-dependency. In conclusion, we feel that replacing atretic bowel in a patient with gastrochisis at the time of the initial operation and anastomosing this 2 to 3 weeks later is a safe and useful approach to dealing with this complex problem.
REFERENCES 1. Gomall P: Management of intestinal atresia complicating gastroschisis. J Pediatr Surg 24:522-524,1989 2. Pokorny WJ, Harberg FJ, McGill CW: Gastroschisis complicated by intestinal atresia. J Pediatr Surg 16:261-263,198l 3. Bernstein P: Gastroschisis, a rare teratological condition in the newborn. Arch Pediatr 57:505,1940 4. Lorenzo MD, Yazbeck S, Ducharme JC: Gastroschisis: A 15-year experience. J Pediatr Surg 22:710-712,1987 5. Amoury RA, Aschraft KW, Holder TM: Gastroschisis complicated by intestinal atresia. Surgery 82:373-381,1977
6. Santulli TV, Blank WA: Congenital atresia of the intestine: Pathogenesis and treatment. Ann Surg 154:939-948,196l 7. Muraji T, Tsugawa C, Nishijima E, et al: Gastroschisis: A 17-year experience. J Pediatr Surg 24:343-345,1989 8. Moore TS, Stokes GE: Gastroschisis. Report of two cases treated by a modification of the Gross operation for omphalocele. Surgery 33:112-120,1953 9. Hollabaugh RS, Boles ET: The management of gastroschisis. J Pediatr Surg 8:263-270,1973
and Intestinal
By Rajkumar
Shah and Morton
Atresia
M. Woolley
Los Angeles, California @Controversy exists over the best method of treating gastroschisis with concomitant intestinal atresia because the mortality in such patients is still high. We present our experience of 74 neonates with gastroschisis seen over a 17-year period. Four patients (5.5%) had intestinal atresia; 1 had only one small bowel atresia, 2 had double areas of atresia (one in the mid small bowel and the other in the proximal colon), and the remaining 1 had multiple areas of atresia. Atresia with gastroschisis conventionally has been treated by either primary anastmosis or by exteriorization. Three of the patients were treated by replacing the atretic bowel into the abdomen initially and exploring later (1 to 3 weeks) when the bowel appeared much more healthy, making the anastomosis easier and more secure. One patient with extreme jejunal dilatation was treated by creation of a Mikulicz fistula. All 4 of the patients survived the newborn period, but one of them died at 1 year of age due to total parenteral nutrition-induced chronic liver failure. Our experience is presented to emphasize this uncommonly used approach to this complex problem because it seems to be associated with a lower morbidity and mortality than other conventional approaches. Copyright o 1991 by W.B. Saunders Company
these patients the defect was located to the left of the umbilicus. Four (5.4%) had associated intestinal atresia. Twenty-three (31%) underwent primary closure including the fascia, 17 (23%) underwent primary skin closure alone, and the remaining 34 (46%) had a silon chimney placed. The mean duration between placement of the silon and definitive closure was 12 days (range, 5 to 32 days). A gastrostomy tube had been placed in 18 patients (24.3%) all of whom were operated on prior to 1976. A total of 11 neonates died (14.8%). Seven of these deaths occurred before 1976. The causes of death included sepsis due to gangrene of intestine at or soon after birth (4) late sepsis (3; Pseudomonas bronchopneumonia, bowel necrosis of uncertain etiology, and enterocutaneous fistula), necrotizing enterocolitis (2) respiratory distress syndrome (l), and shock (1). The mean age at death was 19 days. Of these, 6 were less than 8 days old, 2 were 22 to 23 days old, and 3 were 47 to 49 days old. Of the patients with associated intestinal atresia, 1 had only one small bowel atresia, 2 had double areas of atresia (one in the mid-small bowel and the other in the proximal colon), and 1 had multiple areas of atresia. A brief description of the management of the patients with atresia is given below.
Case Reports INDEX WORDS:
Gastroschisis;
intestinal atresia.
G
ASTROSCHISIS is a congenital protrusion of part of the abdominal contents through an anterior abdominal wall defect usually located just to the right of the umbilicus. The term, derived from Greek origin, means “belly cleft.” Its location, the absence of a sac, and presence of thickened, foreshortened, matted gut differentiates it from an ompahalocele. An association between gastroschisis and intestinal atresia has been well documented. Recent reports’.’ have indicated a high incidence (23%) of this association. We report a series of neonates with gastroschisis seen over a 17-year period, 4 of whom had associated intestinal atresia (5.4%). MATERIALS
AND
METHODS
In the 17-year period from 1972 to 1989 inclusive, 74 neonates with gastroschisis were admitted to the Childrens Hospital of Los Angeles; 46 (62.1%) were boys and 28 (37.9%) were girls. In two of
From the Division of Pediatric Surgery, Department of Surgery, Children’s Hospital of Los Angeles, and the Depatiment of Surgery, University of Southern California School of Medicine, Los Angeles, CA. Presented at the 23rd Annual Meeting of the Pacific Association of Pediatric Surgeons, Kona, Hawaii, June 3-6, 1990. Address reprint requests to Morton M. Wool&y, MD, 4650 Sunset Blvd, Los Angeles, CA 900276062. Copyright o 1991 by WB. Saunders Company 0022-3468/91/2607-0009$03.00/0 788
Case 1. A full-term female infant was born to a heroin-addicted mother whose pregnancy was complicated by polyhydramnios. Emergency surgery was performed for gastroschisis and a markedly dilated loop of distal jejunum was found that ended abruptly at the point of bowel pertrusion through the defect. The extruded gut was a very short segment, blind at both ends, and was resected. After decompressing the jejunum and resecting it to a level with good peristalsis, a Mikulicz fistula was created because of the marked size disparity in the proximal and distal lumen. Eighteen days later the fistula was closed. Apart from laparotomy for resection of an anastomotic stricture, the child had a steady recovery. Case 2. A 2-kg female product of a 36-week gestation was born with gastroschisis. At surgery it was found that there was an area of atresia at the site of entry and exit of the bowel from the abdomen. The proximal site was in the mid-small bowel and the distal site was in the proximal colon. Because of marked edema and matting of the b owel, the atretic segments were replaced into the abdomen, thereby avoiding the risks of a primary anastomosis and problems of a stoma. Twenty days later, she underwent laparotomy with resection and anastomosis of the atretic segments. At this time the intestines appeared healthier making anastmosis easier and more secure. Oral feeds were initiated after 5 weeks and were tolerated remarkably well. Case 3. A 1,860-g, 35week gestation boy was born by cesarean section in a pregnancy complicated by twins, preeclampsia, and breech presentation. At laparotomy, two atretic segments were seen at the base of the gastroschisis defect (midjejunum and colon). Because of doubtful viability of the gut, the atretic segments were replaced into the abdomen and the defect was repaired. One week later, the child was doing well and the abdomen was reexplored. At this time, intestinal perfusion had markedly improved and the edema had decreased, allowing resection and anastomosis of the atretic segments. Postoperatively the child tolerated feeds very slowly and was discharged 81 days after admission. Journalof Pediatric Surgery, Vol26,
No 7 (July), 1991: pp
788-790
GASTROSCHISIS
AND INTESTINAL
ATRESIA
Case 4. A 2,850-kg female product of an uncomplicated pregnancy was born with gastroschisis. There was marked edema, foreshortening, and matting of the gut, and atresia could not be ruled out. Emergency placement of a silon chimney was done. This was progressively tightened and ultimately removed 2 weeks later when a full-thickness abdominal wall repair was done; however, 3 days later the child developed abdominal wall erythema and induration with systemic sepsis. The abdomen was reexplored and a large length of necrotic ileum was found. Multiple areas of atresia were also identified at this time involving the large and small bowel. There was no terminal ileum and the cecum ended blindly. After resection, only 38 cm of small bowel was left, which was anastomosed to the duodenum proximally and the transverse colon distally. Histological examination of the resected bowel showed hemorrhagic necrosis, ulcers, perforations, severe chronic enteritis and multiple atresias. The postoperative course was complicated by anastomotic leak followed by stricture formation necessitating reexploration and revision of the anastomosis. At this time an area of partial weblike obstruction was also found in the jejunum, which was corrected. The infant was maintained on total parenteral nutrition, which was complicated by severe cholestasis, and ultimately died of chronic liver failure and a severe fungal septicemia at 1 year of age.
DISCUSSION
Gastroschisis was first clearly described by Bernstsein in 1940.3 The incidence of intestinal atresia complicating gastroschisis has been reported to range from 6.8%4 to as high as 23%.‘,’ In a collective review of 200 patients with gastroschisis done in 1977,’ the incidence of atresia was 13%.2 Our experience involving 74 patients from a single hospital has shown an incidence of only 5.5%. Atresias complicating gastroschisis may be single’.‘.4 or multiple’ and may involve the small bowel or the large bowel.‘.2 It has been indicated in the past that the colon is an uncommon site for atresia. However, three of four patients in the present study had involvement of the colon. Weblike obstructions associated with obvious atretic segments have also been reported.‘,4 Of these four patients, one had a weblike partial obstruction of the jejunum. It is important to ensure that these are not overlooked at the time of definitive intestinal repair. The exact etiology of intestinal atresia is still uncertain. It is certainly possible that the atresia may be the result of a pinching effect of the defect on the prolapsed intestine.h Vascular accidents due to an intrauterine volvulus probably account for some of the cases in which the level of atresia is not at the level of the defect. The diagnosis of atresia is usually not a problem, although in one of these cases the extreme matting and edema prevented early recognition. This problem has been reported in the literature,5 although in that case it was a weblike atresia. However, extensive dissection to search for an atresia is not recommended,’ because any occult atresia would manifest later in the patient’s course and can
789
be dealt with at that time, when the intestine is more normal in appearance. The management of intestinal atresia complicating gastroschisis remains controversial. This is chiefly due to its low incidence, which prevents a large experience with the problem. It has been suggested that when the intestine is damaged by preatretic dilatation or by exposure to the amniotic fluid, a temporary proximal ileostomy would be the safest course,1-3 followed by subsequent delayed anastomosis. Muraji et al7 also performed ileostomies on their patients with atresia. This approach carries the disadvantage of enterostomy fluid loss, electrolyte imbalance, skin excoriation, and potential sepsis. This also carries obvious problems if placement of a silon is required. Bypass of the atretic segment has also been used.’ Amoury et al5 have recommended resection and anastomosis as the procedure of choice and recommend exteriorization only when the viability of the gut is in question or if there is a proximal perforation. Due to the unhealthy bowel wall, this not only can be technically difficult but will carry a high risk of anastomotic leakage. The technique of replacing the intestine into the abdomen without either exteriorization or primary anastomosis has been adopted,‘.’ although not frequently. We have adopted this technique in three of the four patients and have been impressed by the appearance of the bowel at the time of reexploration. It has been noted by others that the intestine appears nearly normal within 2 to 4 weeks.‘.’ We have observed the intestine to appear healthier and easier to dissect if the repair is delayed. However, we do not recommend this method if there is massive dilatation of the proximal gut. In such cases a proximal enterostomy must be performed. This would also be required in the rare event of a proximal perforation. Although the technique of replacing atretic bowel necessitates a second operation, it eliminates the problems of a stoma and the risks of a primary anastomosis. An additional advantage is that it may be difficult to decide whether an area of narrowing is due to edema or atresia. Replacing the bowel will result in resolution of the problem if it is one of edema, thereby avoiding unnecessary resection or exteriorization. In patients with atresia in more than one area, there does not appear to be a problem with the midsegment getting distended with enteral secretions, although this is a theoretical possibility. Return of gut motility in all patients with gastroschisis is slow, and advancement of feeds has to be very gradual The prognosis of atresia complicating gastroschisis has been reported to be poor. Amoury et al’ have suggested that the success or failure is related
SHAH AND WOOLLEY
790
more to the length of remaining intestine than to any specific method of treatment. Although this is true to an extent, we feel that delaying the primary anastomosis and avoiding stomas tends to improve the survival by eliminating problems of early anastomotic leaks and sepsis due to enterostomies. All six of the patients with atresia in the series reported by Hollabaugh and Boles’ were treated by resection, and none survived. All four patients in this series survived the neonatal period and the complications of case 4 are
related to the necosis rather than the atresia. The cause of death in case 4 was severe chronic liver failure subsequent to short gut syndrome and total parenteral nutrition-dependency. In conclusion, we feel that replacing atretic bowel in a patient with gastrochisis at the time of the initial operation and anastomosing this 2 to 3 weeks later is a safe and useful approach to dealing with this complex problem.
REFERENCES 1. Gomall P: Management of intestinal atresia complicating gastroschisis. J Pediatr Surg 24:522-524,1989 2. Pokorny WJ, Harberg FJ, McGill CW: Gastroschisis complicated by intestinal atresia. J Pediatr Surg 16:261-263,198l 3. Bernstein P: Gastroschisis, a rare teratological condition in the newborn. Arch Pediatr 57:505,1940 4. Lorenzo MD, Yazbeck S, Ducharme JC: Gastroschisis: A 15-year experience. J Pediatr Surg 22:710-712,1987 5. Amoury RA, Aschraft KW, Holder TM: Gastroschisis complicated by intestinal atresia. Surgery 82:373-381,1977
6. Santulli TV, Blank WA: Congenital atresia of the intestine: Pathogenesis and treatment. Ann Surg 154:939-948,196l 7. Muraji T, Tsugawa C, Nishijima E, et al: Gastroschisis: A 17-year experience. J Pediatr Surg 24:343-345,1989 8. Moore TS, Stokes GE: Gastroschisis. Report of two cases treated by a modification of the Gross operation for omphalocele. Surgery 33:112-120,1953 9. Hollabaugh RS, Boles ET: The management of gastroschisis. J Pediatr Surg 8:263-270,1973