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Management of gastroschisis and short bowel: Report of 17 cases
Management of Gastroschisis aqd Short Bowel : Report of 17 Cases By Michel G. Gilbert, Luis F. tvlencia, Subhash R. Puranik, Richard E. Litt, and Donald H. Altman
ASTROSCHISIS is a full-thickness cleft in the abdominal wall through which the intestines eviscerate. The embryology of this defect has been lucidly described by Duhamell and Izant.2 In 1953, Moore and Stokes3 differentiated gastroschisis from ruptured omphalocele by these criteria: (1) extraumbilical abdominal wall defect; (2) normal umbilical cord insertion; and (3) lack of covering sac or its remnant.
G
MATERIALS AND METHODS From January 1963 until December 1971,17 infants with gastroschisis were treated at the Variety Children’s Hospital. There were ten females and seven males; ten weighed 2300 g (5 lb) or less.
PATHOLOGY AND ANATOMICAL
ANOMALIES
The abdominal wall defect was to the right of a normally inserted umbilical cord in all 17 cases. The size of the defect varied from 0.5 to 4 cm in diameter. All patients had part of the small bowel and colon through the defect. In addition, evisceration of the stomach was present in 12 cases, the Fallopian tithes and ovaries in three of the ten females, the bladder in two patients, the liver edge in two patients, and the entire liver in one patient (Fig. IA). In one infant the 0.5 cm abdominal wall defect had constricted the bowel, causing gangrene of two-thirds of the small bowel and one-third of the colon (Fig. IB). Ten patients had rigid edematous, dilated bowel matted together by a greengray fibrinous membrane. This finding indicated a prolonged intrauterine or antenatal evisceration. Seven of these babies were premature. In the remaining seven patients, the evisceration most likely occurred late in intrauterine life From the Variety Children’s Hospital and the University of Miami School of Medicine, Miwni, Fla. Presented at the Third Annual Meeting of the American Pediatric Surgical Association, Hot Springs, Vu., April 13-15, 1972. Michel G. Gilbert, M.D.: Deceased; formerly Chief of Pediatric Surgery and Director of Surgical Seruices, Variety Children’s Hospital, Miami, Fla., and Clinical Assistant Professor of Surgery, University of Miami School of Medicine, Miami, Flu. Luio F.’ Mencia, M.D.: Senior Attending Pediatric Surgeon, Variety Children’s Hospital, Miami, Flu., and Clinical Assistant Professor of Surgery, University of Miami School of Medicine, Miami, Flu. Subhash R. Puranik, M.B., M.S., F.R.C.S. (C.): lnstrucfor in Pediatric Surgery, Variety Children’s Hospital, Miami, Flu. Richard E. Liit, M.D.: Senior Attending Radiologist, Variety Children’s Hospital, Miami, Flu., and Clinical Associate Professor of Radiology and Pediatrics, University of Miami School of Medicine, Miami, Flu. Donald H. Altman, M.D.: Director of Radiology, Variety Chifdren’s Hospitaf, Miami, Flu., and Clinical Professor of Radiology and Pediatrics, Uniuersity of Miami School of Medicine, Miami, Flu. 59s
Journal of Pediatric Surgery, Vol. 7, No. 5 (October-November),
1972
GASTROSCHISIS AND SHORT BOWEL
599
because there was little mesenteric or serosal reaction, and these were called perinatal eviscerations. Bowel function returned more rapidly in these babies. Only three were premature. The bowel length of normal neonates may vary from 298 to 460 crn.‘,§ We have measured bowel length from the pylorus to the anus, using a string along the antimesenteric border in 26 neonates with no intraabdominal disease at postmortem. The small bowel varied from 158 to 390 cm, with an average of 267 cm. The colon varied from 25 to 70 cm, with an average of 50 cm. There was no correlation between body length or body weight and the length of the bowel. We also measured bowel length during the initial surgical procedure in 12 babies with gastroschisis. The small bowel varied from 35 to 130 cm, with an average of 76 cm. The total bowel length varied from 60 to 180 cm, with an average of 113 cm, or about one-third that of the normal neonate. Patients with perinatal gastroschisis had less total bowel shortening (148 cm) as compared with patients with antenatal gastroschisis (87 cm). This “short bowel” was not an irreversible problem. After replacement of the eviscerated mass into the peritoneal cavity, gradual lengthening occurs. For example, one baby was reexplored, at 1 wk of age, due to a leak at the gastrostomy site. The thick
Fig. 1. (A) Evisceration of the entire liver through the defect. Minimal serosal reaction shows it to be a perinatal gastroschisis. The patient died without the surgery. (8) Extensive gangrene of the bowel due to small constricting abdominal defect.
600
GILBERT ET AL.
greenish exudate had resolved and the bowel length had increased by onethird. Another baby was reoperated, at 1 mo of life, for intestinal obstruction. There was complete resolution of the exudate and the small bowel had tripled in length, from the original SO cm to 158 cm. All of our patients had nonrotated bowel; we found no Meckel’s diverticulum, but six babies had a broad short appendix that could be mistaken for a Meckel’s. Half of our patients had a marked distention of the distal sigmoid with meconium. Proximally to this, the coIon abruptly narrowed. Enlargement of the duodenum, without any obstructing lesion, was found in three; in the two who survived, the duodenum gradually diminished to a more normal size. Other congenital anomalies consisted only of bilateral cataracts in one and unilateral undescended testicles in two. RADIOLOGIC FINDINGS
We performed a barium enema on nine patients, before 1 mo of age. Six of these demonstrated a relatively large rectum and distal sigmoid, above which the bowel lumen became smaller and the mucosal pattern was radiologically similar to jejunum (Fig. 2). However, microscopic examinations of autopsy specimens of the proximal sigmoid and left colon have shown normal colonic mucosa. Every patient examined had nonrotation of the colon, but it was extremely difficult to identify the cecum, appendix, or ileocecal valve in the neonate. A repeat colon examination, several months later, delineated a normal mucosal pattern and no abnormalities, except for malrotation. Early gastrointestinal examinations revealed delayed transit time. Repeated gastrointestinal series, after I yr of age, have disclosed a normal complement of small bowel. PREOPERATIVE TREATMENT
We advise transfer of these infants in a warm incubator by ambulance or airplane. The referring physician is asked to insert a nasogastric tube and to
Fig. 2. Barium enema at 10 days. Note the dilated sigmoid colon with pattern resembling the valvulae conniventes of the jejunum.
601
GASTROSCHISIS AND SHORT BOWEL
cover the eviscerated bowel with warm, sterile, saline-soaked sponges. Hypothermia is the most serious early complication facing these babies. On arrival in our hospital they are admitted to our intensive-care unit and placed in a crib with an overhead heater. We start intravenous fluids in an upper extremity, and initially give 5 cc/lb of plasma substitute followed by Ringer’s lactate. If blood and gas analysis demonstrates acidosis, this is corrected and the infant taken to the operating room. From 1 to 2 h, ;.I preoyerative preparation is sufficient. We now also start penicillin and gentamicin preoperatively. OPERATIVE
MANAGEMENT
Initially, we obtain a culture from the bowel surface, then wash the skin and viscera with warm aqueous Zephiran solution. The abdominal wall defect may be enlarged, then the loops of bowel are separated and loose portions of the fibrinous peel removed. The abdominal cavity is manually stretched. In eight of our patients, it was possible by stretching the abdominal wall to replace the viscera, and perform a primary one-layer closure with no change in lung compliance. Six of these babies survived the repair, of which one died 3% mo later. Five of these eight patients had an antenatal evisceration. In eight others the abdominal cavity was too small to allow a safe primary closure. One of these babies had a skin flap closure with repair of his ventral hernia at 2 yr of age. In two more babies, Dacron-reinforced silastic patches were sutured to the fascial edges, then to each other, and covered with skin flaps. Both infants died with sepsis. The remaining five patients were all treated by creating an extracelomic tube of silastic sutured to the full-thickness defect. By gradually reducing the viscera every other day, comulete closure was accomplished in B-10 days (Fig. 3). The first of these infants died immediately with sepsis. Since that time we continue the penicillin and gentamicin postoperatively. In addition, we spray the silastic with Neosporin (Burroughs Wellcome) and apply gentamicin ointment to the skin-silastic junction every 4 hr. It is also important to discontinue humidity in the isolette after 24 hr and to move the baby to a clean isolette every 24-48 hr. These measures are important to prevent pseudomonas sepsis. This is our present preferred technique for staged closure. The last four patients treated in this fashion have survived the repair and had no sepsis from using the silastic sheets. One of them died at a second admission of gangrenous enterocolitis unrelated to surgery or original pathology. A gastrostomy tube provides postoperative decompression. The majority of patients with perinatal evisceration can tolerate oral feedings as early as the third to fifth day of life, following a primary closure. In those with staged closure, no feeding is commenced until after the removal of silastic sheeting. The formulas best tolerated by these infants have been Portagen (medium chain triglycerides) or Nutramigen (both Mead Johnson). A prolonged delay in the onset of the effective gastrointestinal function was very common in infants with antenatal evisceration. In these cases, intravenous hyperalimentation was most beneficial.
602
GILBERT ET AL. COMPLICATIONS AND RESULTS
In our series of 17 patients, 16 were operated upon, 11 survived the repair, and nine are alive now (Table 1 and 2). Of the seven patients with perinatal evisceration, a a-lb premature died at 10 hr of age without surgery. The remaining six survived a primary or staged repair and four are alive. One infant died at Wz mo of age from malnutrition due to extensive intestinal gangrene. This was before the availability of intravenous hyperalimentation. Another patient died at a second admission of gangrenous enterocolitis. The ten babies with antenatal evisceration had a high incidence of delayed intestinal function and infections and five died. The nine surviving patients are now entirely asymptomatic and taking a normal diet. None of these patients demonstrate clinical evidence of malabsorbtion from short bowel and they are all in the average percentile for height and weight.
Fig. 3. Our present method of staged closure, just prior to final full-thickness closure. Three stages of previous partial reduction can be seen as the successive ties of the umbilical tape.
Antenatal
2250
1980
1420
1980
3480
4
5
8
7
8’
Silastic to fascia with skin flaps over It Staged closure with silastic tube
Staged closure with silastic tube None
(1) Silastlc to fascia with skin flaps over it. (2) Resection of 7 ileocecal stenosis 9 days postop. Full-thickness primary closure
Primary full-thickness closure Resection of gangrene, end-to-end anstomosis. primary full-thickness ctosure
Surgical Procedure
Delayed
None
None
2wk
None
None
None
Died at 10 days, skin flap necrosis on top andsepsis Survived repair, discharged; died on second admission of gangrenous enterocolltls
Died 3rd day. Increased intraabdominal pressure, sepsis, peritonitis Died on 4th day of pseudomonas sepsis Died at 10 hrwithout any surgery
None
None
None
7 ileocecal stenosis. Died on 17th day from anastomotic leak, peritonitis, and sepsis
None
None
Early from 5th day
Died on Sth.day, possible visceral injury Gangrene. Died on 98th day from malnutrition
Complications and Cause of Death
Delayed
None
I.V. Hyperalimentation
None
Onset of Oral Nutrition
*Considered survivors of initial surgical procedure to reduce the evisceration and close the defect.
Perinatal
Perinatal with total liver evisceration Antenatal
Antenatal
Perinatal with extensive gangrene from small size of defect Antenatal
1980
2210
Antenatal
1980
1
Classification
Birth Weight (grams)
Case No.
Table 1. Summary of Case Histories of Deaths
E
P
%!
$
m o-J
Skin flap cover first; repair hernia at 2 yr (1) Full-thickness primary closure. (2) Lysis of adhesions 1 wk postop. Full-thickness primary closure
Full-thickness
(1) Full-thickness
closure; (2) Closure of gastrostomy leak at 1 wk Full-thickness primary closure
Perinatal
Antenatal
Perinatal
Antenatal
Antenatal
Perinatal
Antenatal
Antenatal
Perinatal
2720
2440
1820
1580
2280
.3080
2840
3170
2380
1
2
3
4
5
8
7
8
9
tube Staged closure with silastic tube
(1) Staged closure with silastic tube (2) Two explorations for ? int. obstr. Staged closure with silastic
primary
primary closure
Surgical Procedure
Classification
Birth Weight (grams)
of Survivors
? intestinal obstruction. Small ventral hernia None
39 days
Delayed Delayed
? intestinal obstruction. Candida and gramnegative sepsis
28 days
Gastrostomy
leak
Small ventral hernia
75 days
None
None
None
None
None
None
? intestinal obstruction
None
Delayed to 2% wk Early, from 3rd day postop. Early, from 4th day Early, from 4th day postop. Early, from 5th day Long delayed
None
Complications
None
I.V. Hyperalimentation
Early
Onset of Oral Nutrition
of Case Histories
Case No.
Table 2. Summary
605
GASTROSCHISIS AND SHORT BOWEL
DISCUSSION The surgical procedure
for gastroschisis must provide a covering for the evisceration, without increasing the intraabdominal pressure. A primary closure should be attempted if the disparity between the evisceration and the peritoneal cavity is not too great. Coverage with skin flaps, a modification of the Gross operation for omphalocele, has been used in many successful cases.Our preferred procedure, where staged closure is necessary, is to create a silassilastic sac as described by Allen and Gilbert. lo*11The dacron-reinforced tic sheeting is simply sewn to the full thickness of the abdominal wall and closed distally. Gradual reduction of the viscera, by progressive ligation of the distal end, can be easily accomplished. Postoperatively, isolation technique and local and systemic antibiotics as already described are absolutely necessary until the prosthesis is removed. This is usually within 8-10 days. Resumption of gastrointestinal function in these infants may be long delayed. During this period, they must be kept in positive nitrogen balance with parenteral alimentation, through a central venous catheter.12*13 We believe that oral Nystatin, while on hyperalimentation, has helped reduce the incidence of candida infection. Since no other significant anomalies are present, a defeatist attitude should never be accepted. Three patients displaying nebulous findings of intestinal obstruction were operated upon. We now treat these infants with a conservative regime of Dulcolax suppositories, intravenous hyperalimentation, and a “tincture of time” until their bowel function returns to normal. SUMMARY
An 8-yr experience with 17 cases of gastroschisis is presented. Survival has increased with refinements of surgical techniques and availability of intravenous hyperalimentation during the period of bowel malfunction. Primary closure is attempted if possible, without causing undue increase of intraabdominal pressure. Where this is not possible, a staged closure is used. A method of staged closure with dacron-reinforced silastic sheets is described. Of the 17 patients, 16 were operated on, 11 survived the repair, and nine are now alive. It was possible to distinguish antenatal from perinatal evisceration from the degree of serosal and mesenteric reaction. Short bowel, megasigmoid, embryonic appendix, and cecum were common observations. In the neonatal period, the radiological pattern of the sigmoid colon resembled that of the jejunum. With time, all changes reverted to normal except the incomplete rotation, which persisted. REFERENCES 1. Duhamel, 8.: Embryology of exomphalos and allied malformations. Arch. Dis. Child. 38:142, 1963. 2. Izant, R. J., Brown, F., and Rothmann, B. F.: Current embryology and treatment of
gastroschisis and omphaIoceIe. Arch. Surg. (Chicago)
93:49,
1966.
3. Moore, T. C., and Stokes, G. E.: Gastro-
schisis: Report of two cases treated by modification of Gross operation for omph-
606
GILBERT ET AL.
alocele. Surgery 33:112,1953. 4. Benson, C. D.: Resection and primary anastomosis of jejunum and ileum in the newborn. Ann. Surg. 142:478,1955. 5. Potter, E. L., and Adair, F. L.: Fetal and Neonatal Death (ed. 2). Chicago, University of Chicago Press, 1949,p. 31. 6.Gross, R. E.: A new method for surgical treatment of large omphaloceles. Surgery 24:277,1948. 7. Cook, T. D.: Gastroschisis: A method of treatment. Surgery 46:618,1959. 8. Judd, D. R., Wince, L., and Moore, T. C.: Gastroschisis: Report of 2 cases successfully treated. Surgery 58:1033, 1965. 9. Simpson, R. L., and Caylor, H. D.:
Gastroschisis. Amer. J. Surg. 96:675,1958. 10.Allen, R. G., and Wrenn, E. L.: Silon as a sac in the treatment of omphalocele and gastroschisis. J. Pediat. Surg. 4:3, 1969. 11. Gilbert, M. G., Mencia, L. F., Brown, W. T., and Linn, B. S.: Staged surgical repair of large omphaloceles and gastroschisis. J. Pediat. Surg. 3:702,1968. 12. Wilmore, D. W., and Dudick, S. J.: Growth and development of an infant receiving all nutrients exclusively by vein. JAMA 203:860,1968. 13. Filler, R. M., Eraklis, A. J., Rubin, V. G., and Das, J. 8.: Long-term parenteral nutrition in infants. New Eng. J. Med. 281: 589,1969.
Discussion Dr. E. Lerois (St. Louis): I am pleased that the authors have emphasized the difference between antenatal and perinatal types of gastroschisis. I would like to express our indebtedness to Dr. Thomas Moore for defining the pathology of this problem 15 yr ago and giving us some sense of direction in its surgical management; and also to Dr. Schuster for his fine contribution in the use of prosthetic coverage in omphaloceles and gastroschisis. However, I would like to emphasize that because a patient has gastroschisis, he does not always require a prosthesis in the correction of the problem, nor does he always require parenteral alimentation for survival. During the past 5 yr at Cardinal Glennon Memorial Hospital for Children in St. Louis, we had 20 cases of gastroschisis, with 16 survivals. All of the survivors except two were closed primarily. I believe that all of the perinatal group can be treated by stretching the abdominal wall and primary closure. The antenatal group will require the use of a silon pouch. However, there is a group with sufficient edema and reaction in the intestinal wall to preclude closure of the abdominal wall, although the intestines can be replaced in the peritoneal cavity. In this group, a silon patch will relieve intraabdominal pressure. Closure can be accomplished early or late, depending on the general condition of the infant. The authors have emphasized the early return of ability to eat in most cases after primary closure. Parenteral alimentation should only be used if essential for survival and not as routine treatment. Dr. E. Wrenn (Memphis): I would like to make a point about the definition of gastroschisis. All of the illustrations in the paper seemed to show the defect through the umbilical ring, which suggests that the patients had ruptured omphaloceles rather than gastroschisis. It is my understanding that gastroschisis is a defect outside the umbilical ring. Very few authors seem to make that differentiation, but I feel it is important to separate these tw6 entities since they arise from different embryologic origins. Dr. W. Kiesewetter (Pittsburgh): We have measured the bowel length in nine patients with omphalocele, and agree with the authors that they do have a much shorter bowel. We have also measured intestinal length in 27 newborns with other neonatal conditions, and find a direct relationship between the weight of the child and length of the bowel; in other words, the larger the baby, the longer the bowel. We did not, however, conhm 267 cm as being average. Only one exceeded 267 cm, and most were somewhere between 180 and 220cm. Dr. R. Izant (Cleuefand): It should be mentioned that Dr. Clatworthy pointed out many, many years ago that we should be measuring numbers of vascular arcades in the mesentery, rather than the linear length of bowel. It means more from a functional standpoint and is a much more accurate estimate of bowel length. Further work should be done on this issue.
GASTROSCHISIS AND SHORT BOWEL
007
Dr. S. R. Schuster (Boston): In gastroschisis (contrasted to omphalocele) one is more likely to be able totally to reduce the herniated viscera and effect a single-stage primary repair. This is particularly true for those patients in which the liver is not part of the herniated mass. This easier reduction in gastroschisis patients results because the midgut has already returned to the abdomen during fetal life to claim its right of domain. However, in omphalocele the bowel has never returned. The abdomen has never accommodated to the size necessary for the midgut and possible other viscera, such as liver, spleen, and stomach. Measurement of intestinal length in patients with an antenatal ruptured omphalocele or gastroschisis is highly unreliable. There is usually considerable reaction of the bowel and mesentery with shortening of these structures and actual conglomeration so that separate loops of intestine are sometimes indistinguishable. There is probably more actual length of intestine than one thinks. Dr. Purunik: We agree with most of the observations of Dr. Lewis. In answer to Dr. Wrenn’s question, all of these cases are true gastroschises and satisfy the criteria set forth by Drs. Moore and Stokes. The insertion of the umbilical cord was not seen in all our illustrations because in some cases it was removed prior to obtaining the first picture and in others it was covered in an attempt to demonstrate a different aspect of the pathology. None of these, cases represent a ruptured omphalocele. With regard to bowel length in the neonates, our observations are different from those of Dr. Kiesewetter. We have found no correlation between body weight from 2.3 to 3 kg. Therefore, we do not think that the “short bowel” in gastroschisis is due simply to the low birth weight. We agree with both the points stated by Dr. Schuster. First, the abdominal space in these babies is not actually diminished, but only relatively so, because of the thick, edematous, and matted viscera. The disparity rapidly disappears and the viscera can be replaced into the abdominal cavity within about 8 days, even in the most severe cases, Second, the bowel is probably foreshortened only from the puckering, accordian-like effect to the serosa by the exudate covering it. As the exu&te is absorbed after closure, the bowel attains its real or near-normal length rapidly. This was proved in two of our cases who were reexplored after a short interval.
ASTROSCHISIS is a full-thickness cleft in the abdominal wall through which the intestines eviscerate. The embryology of this defect has been lucidly described by Duhamell and Izant.2 In 1953, Moore and Stokes3 differentiated gastroschisis from ruptured omphalocele by these criteria: (1) extraumbilical abdominal wall defect; (2) normal umbilical cord insertion; and (3) lack of covering sac or its remnant.
G
MATERIALS AND METHODS From January 1963 until December 1971,17 infants with gastroschisis were treated at the Variety Children’s Hospital. There were ten females and seven males; ten weighed 2300 g (5 lb) or less.
PATHOLOGY AND ANATOMICAL
ANOMALIES
The abdominal wall defect was to the right of a normally inserted umbilical cord in all 17 cases. The size of the defect varied from 0.5 to 4 cm in diameter. All patients had part of the small bowel and colon through the defect. In addition, evisceration of the stomach was present in 12 cases, the Fallopian tithes and ovaries in three of the ten females, the bladder in two patients, the liver edge in two patients, and the entire liver in one patient (Fig. IA). In one infant the 0.5 cm abdominal wall defect had constricted the bowel, causing gangrene of two-thirds of the small bowel and one-third of the colon (Fig. IB). Ten patients had rigid edematous, dilated bowel matted together by a greengray fibrinous membrane. This finding indicated a prolonged intrauterine or antenatal evisceration. Seven of these babies were premature. In the remaining seven patients, the evisceration most likely occurred late in intrauterine life From the Variety Children’s Hospital and the University of Miami School of Medicine, Miwni, Fla. Presented at the Third Annual Meeting of the American Pediatric Surgical Association, Hot Springs, Vu., April 13-15, 1972. Michel G. Gilbert, M.D.: Deceased; formerly Chief of Pediatric Surgery and Director of Surgical Seruices, Variety Children’s Hospital, Miami, Fla., and Clinical Assistant Professor of Surgery, University of Miami School of Medicine, Miami, Flu. Luio F.’ Mencia, M.D.: Senior Attending Pediatric Surgeon, Variety Children’s Hospital, Miami, Flu., and Clinical Assistant Professor of Surgery, University of Miami School of Medicine, Miami, Flu. Subhash R. Puranik, M.B., M.S., F.R.C.S. (C.): lnstrucfor in Pediatric Surgery, Variety Children’s Hospital, Miami, Flu. Richard E. Liit, M.D.: Senior Attending Radiologist, Variety Children’s Hospital, Miami, Flu., and Clinical Associate Professor of Radiology and Pediatrics, University of Miami School of Medicine, Miami, Flu. Donald H. Altman, M.D.: Director of Radiology, Variety Chifdren’s Hospitaf, Miami, Flu., and Clinical Professor of Radiology and Pediatrics, Uniuersity of Miami School of Medicine, Miami, Flu. 59s
Journal of Pediatric Surgery, Vol. 7, No. 5 (October-November),
1972
GASTROSCHISIS AND SHORT BOWEL
599
because there was little mesenteric or serosal reaction, and these were called perinatal eviscerations. Bowel function returned more rapidly in these babies. Only three were premature. The bowel length of normal neonates may vary from 298 to 460 crn.‘,§ We have measured bowel length from the pylorus to the anus, using a string along the antimesenteric border in 26 neonates with no intraabdominal disease at postmortem. The small bowel varied from 158 to 390 cm, with an average of 267 cm. The colon varied from 25 to 70 cm, with an average of 50 cm. There was no correlation between body length or body weight and the length of the bowel. We also measured bowel length during the initial surgical procedure in 12 babies with gastroschisis. The small bowel varied from 35 to 130 cm, with an average of 76 cm. The total bowel length varied from 60 to 180 cm, with an average of 113 cm, or about one-third that of the normal neonate. Patients with perinatal gastroschisis had less total bowel shortening (148 cm) as compared with patients with antenatal gastroschisis (87 cm). This “short bowel” was not an irreversible problem. After replacement of the eviscerated mass into the peritoneal cavity, gradual lengthening occurs. For example, one baby was reexplored, at 1 wk of age, due to a leak at the gastrostomy site. The thick
Fig. 1. (A) Evisceration of the entire liver through the defect. Minimal serosal reaction shows it to be a perinatal gastroschisis. The patient died without the surgery. (8) Extensive gangrene of the bowel due to small constricting abdominal defect.
600
GILBERT ET AL.
greenish exudate had resolved and the bowel length had increased by onethird. Another baby was reoperated, at 1 mo of life, for intestinal obstruction. There was complete resolution of the exudate and the small bowel had tripled in length, from the original SO cm to 158 cm. All of our patients had nonrotated bowel; we found no Meckel’s diverticulum, but six babies had a broad short appendix that could be mistaken for a Meckel’s. Half of our patients had a marked distention of the distal sigmoid with meconium. Proximally to this, the coIon abruptly narrowed. Enlargement of the duodenum, without any obstructing lesion, was found in three; in the two who survived, the duodenum gradually diminished to a more normal size. Other congenital anomalies consisted only of bilateral cataracts in one and unilateral undescended testicles in two. RADIOLOGIC FINDINGS
We performed a barium enema on nine patients, before 1 mo of age. Six of these demonstrated a relatively large rectum and distal sigmoid, above which the bowel lumen became smaller and the mucosal pattern was radiologically similar to jejunum (Fig. 2). However, microscopic examinations of autopsy specimens of the proximal sigmoid and left colon have shown normal colonic mucosa. Every patient examined had nonrotation of the colon, but it was extremely difficult to identify the cecum, appendix, or ileocecal valve in the neonate. A repeat colon examination, several months later, delineated a normal mucosal pattern and no abnormalities, except for malrotation. Early gastrointestinal examinations revealed delayed transit time. Repeated gastrointestinal series, after I yr of age, have disclosed a normal complement of small bowel. PREOPERATIVE TREATMENT
We advise transfer of these infants in a warm incubator by ambulance or airplane. The referring physician is asked to insert a nasogastric tube and to
Fig. 2. Barium enema at 10 days. Note the dilated sigmoid colon with pattern resembling the valvulae conniventes of the jejunum.
601
GASTROSCHISIS AND SHORT BOWEL
cover the eviscerated bowel with warm, sterile, saline-soaked sponges. Hypothermia is the most serious early complication facing these babies. On arrival in our hospital they are admitted to our intensive-care unit and placed in a crib with an overhead heater. We start intravenous fluids in an upper extremity, and initially give 5 cc/lb of plasma substitute followed by Ringer’s lactate. If blood and gas analysis demonstrates acidosis, this is corrected and the infant taken to the operating room. From 1 to 2 h, ;.I preoyerative preparation is sufficient. We now also start penicillin and gentamicin preoperatively. OPERATIVE
MANAGEMENT
Initially, we obtain a culture from the bowel surface, then wash the skin and viscera with warm aqueous Zephiran solution. The abdominal wall defect may be enlarged, then the loops of bowel are separated and loose portions of the fibrinous peel removed. The abdominal cavity is manually stretched. In eight of our patients, it was possible by stretching the abdominal wall to replace the viscera, and perform a primary one-layer closure with no change in lung compliance. Six of these babies survived the repair, of which one died 3% mo later. Five of these eight patients had an antenatal evisceration. In eight others the abdominal cavity was too small to allow a safe primary closure. One of these babies had a skin flap closure with repair of his ventral hernia at 2 yr of age. In two more babies, Dacron-reinforced silastic patches were sutured to the fascial edges, then to each other, and covered with skin flaps. Both infants died with sepsis. The remaining five patients were all treated by creating an extracelomic tube of silastic sutured to the full-thickness defect. By gradually reducing the viscera every other day, comulete closure was accomplished in B-10 days (Fig. 3). The first of these infants died immediately with sepsis. Since that time we continue the penicillin and gentamicin postoperatively. In addition, we spray the silastic with Neosporin (Burroughs Wellcome) and apply gentamicin ointment to the skin-silastic junction every 4 hr. It is also important to discontinue humidity in the isolette after 24 hr and to move the baby to a clean isolette every 24-48 hr. These measures are important to prevent pseudomonas sepsis. This is our present preferred technique for staged closure. The last four patients treated in this fashion have survived the repair and had no sepsis from using the silastic sheets. One of them died at a second admission of gangrenous enterocolitis unrelated to surgery or original pathology. A gastrostomy tube provides postoperative decompression. The majority of patients with perinatal evisceration can tolerate oral feedings as early as the third to fifth day of life, following a primary closure. In those with staged closure, no feeding is commenced until after the removal of silastic sheeting. The formulas best tolerated by these infants have been Portagen (medium chain triglycerides) or Nutramigen (both Mead Johnson). A prolonged delay in the onset of the effective gastrointestinal function was very common in infants with antenatal evisceration. In these cases, intravenous hyperalimentation was most beneficial.
602
GILBERT ET AL. COMPLICATIONS AND RESULTS
In our series of 17 patients, 16 were operated upon, 11 survived the repair, and nine are alive now (Table 1 and 2). Of the seven patients with perinatal evisceration, a a-lb premature died at 10 hr of age without surgery. The remaining six survived a primary or staged repair and four are alive. One infant died at Wz mo of age from malnutrition due to extensive intestinal gangrene. This was before the availability of intravenous hyperalimentation. Another patient died at a second admission of gangrenous enterocolitis. The ten babies with antenatal evisceration had a high incidence of delayed intestinal function and infections and five died. The nine surviving patients are now entirely asymptomatic and taking a normal diet. None of these patients demonstrate clinical evidence of malabsorbtion from short bowel and they are all in the average percentile for height and weight.
Fig. 3. Our present method of staged closure, just prior to final full-thickness closure. Three stages of previous partial reduction can be seen as the successive ties of the umbilical tape.
Antenatal
2250
1980
1420
1980
3480
4
5
8
7
8’
Silastic to fascia with skin flaps over It Staged closure with silastic tube
Staged closure with silastic tube None
(1) Silastlc to fascia with skin flaps over it. (2) Resection of 7 ileocecal stenosis 9 days postop. Full-thickness primary closure
Primary full-thickness closure Resection of gangrene, end-to-end anstomosis. primary full-thickness ctosure
Surgical Procedure
Delayed
None
None
2wk
None
None
None
Died at 10 days, skin flap necrosis on top andsepsis Survived repair, discharged; died on second admission of gangrenous enterocolltls
Died 3rd day. Increased intraabdominal pressure, sepsis, peritonitis Died on 4th day of pseudomonas sepsis Died at 10 hrwithout any surgery
None
None
None
7 ileocecal stenosis. Died on 17th day from anastomotic leak, peritonitis, and sepsis
None
None
Early from 5th day
Died on Sth.day, possible visceral injury Gangrene. Died on 98th day from malnutrition
Complications and Cause of Death
Delayed
None
I.V. Hyperalimentation
None
Onset of Oral Nutrition
*Considered survivors of initial surgical procedure to reduce the evisceration and close the defect.
Perinatal
Perinatal with total liver evisceration Antenatal
Antenatal
Perinatal with extensive gangrene from small size of defect Antenatal
1980
2210
Antenatal
1980
1
Classification
Birth Weight (grams)
Case No.
Table 1. Summary of Case Histories of Deaths
E
P
%!
$
m o-J
Skin flap cover first; repair hernia at 2 yr (1) Full-thickness primary closure. (2) Lysis of adhesions 1 wk postop. Full-thickness primary closure
Full-thickness
(1) Full-thickness
closure; (2) Closure of gastrostomy leak at 1 wk Full-thickness primary closure
Perinatal
Antenatal
Perinatal
Antenatal
Antenatal
Perinatal
Antenatal
Antenatal
Perinatal
2720
2440
1820
1580
2280
.3080
2840
3170
2380
1
2
3
4
5
8
7
8
9
tube Staged closure with silastic tube
(1) Staged closure with silastic tube (2) Two explorations for ? int. obstr. Staged closure with silastic
primary
primary closure
Surgical Procedure
Classification
Birth Weight (grams)
of Survivors
? intestinal obstruction. Small ventral hernia None
39 days
Delayed Delayed
? intestinal obstruction. Candida and gramnegative sepsis
28 days
Gastrostomy
leak
Small ventral hernia
75 days
None
None
None
None
None
None
? intestinal obstruction
None
Delayed to 2% wk Early, from 3rd day postop. Early, from 4th day Early, from 4th day postop. Early, from 5th day Long delayed
None
Complications
None
I.V. Hyperalimentation
Early
Onset of Oral Nutrition
of Case Histories
Case No.
Table 2. Summary
605
GASTROSCHISIS AND SHORT BOWEL
DISCUSSION The surgical procedure
for gastroschisis must provide a covering for the evisceration, without increasing the intraabdominal pressure. A primary closure should be attempted if the disparity between the evisceration and the peritoneal cavity is not too great. Coverage with skin flaps, a modification of the Gross operation for omphalocele, has been used in many successful cases.Our preferred procedure, where staged closure is necessary, is to create a silassilastic sac as described by Allen and Gilbert. lo*11The dacron-reinforced tic sheeting is simply sewn to the full thickness of the abdominal wall and closed distally. Gradual reduction of the viscera, by progressive ligation of the distal end, can be easily accomplished. Postoperatively, isolation technique and local and systemic antibiotics as already described are absolutely necessary until the prosthesis is removed. This is usually within 8-10 days. Resumption of gastrointestinal function in these infants may be long delayed. During this period, they must be kept in positive nitrogen balance with parenteral alimentation, through a central venous catheter.12*13 We believe that oral Nystatin, while on hyperalimentation, has helped reduce the incidence of candida infection. Since no other significant anomalies are present, a defeatist attitude should never be accepted. Three patients displaying nebulous findings of intestinal obstruction were operated upon. We now treat these infants with a conservative regime of Dulcolax suppositories, intravenous hyperalimentation, and a “tincture of time” until their bowel function returns to normal. SUMMARY
An 8-yr experience with 17 cases of gastroschisis is presented. Survival has increased with refinements of surgical techniques and availability of intravenous hyperalimentation during the period of bowel malfunction. Primary closure is attempted if possible, without causing undue increase of intraabdominal pressure. Where this is not possible, a staged closure is used. A method of staged closure with dacron-reinforced silastic sheets is described. Of the 17 patients, 16 were operated on, 11 survived the repair, and nine are now alive. It was possible to distinguish antenatal from perinatal evisceration from the degree of serosal and mesenteric reaction. Short bowel, megasigmoid, embryonic appendix, and cecum were common observations. In the neonatal period, the radiological pattern of the sigmoid colon resembled that of the jejunum. With time, all changes reverted to normal except the incomplete rotation, which persisted. REFERENCES 1. Duhamel, 8.: Embryology of exomphalos and allied malformations. Arch. Dis. Child. 38:142, 1963. 2. Izant, R. J., Brown, F., and Rothmann, B. F.: Current embryology and treatment of
gastroschisis and omphaIoceIe. Arch. Surg. (Chicago)
93:49,
1966.
3. Moore, T. C., and Stokes, G. E.: Gastro-
schisis: Report of two cases treated by modification of Gross operation for omph-
606
GILBERT ET AL.
alocele. Surgery 33:112,1953. 4. Benson, C. D.: Resection and primary anastomosis of jejunum and ileum in the newborn. Ann. Surg. 142:478,1955. 5. Potter, E. L., and Adair, F. L.: Fetal and Neonatal Death (ed. 2). Chicago, University of Chicago Press, 1949,p. 31. 6.Gross, R. E.: A new method for surgical treatment of large omphaloceles. Surgery 24:277,1948. 7. Cook, T. D.: Gastroschisis: A method of treatment. Surgery 46:618,1959. 8. Judd, D. R., Wince, L., and Moore, T. C.: Gastroschisis: Report of 2 cases successfully treated. Surgery 58:1033, 1965. 9. Simpson, R. L., and Caylor, H. D.:
Gastroschisis. Amer. J. Surg. 96:675,1958. 10.Allen, R. G., and Wrenn, E. L.: Silon as a sac in the treatment of omphalocele and gastroschisis. J. Pediat. Surg. 4:3, 1969. 11. Gilbert, M. G., Mencia, L. F., Brown, W. T., and Linn, B. S.: Staged surgical repair of large omphaloceles and gastroschisis. J. Pediat. Surg. 3:702,1968. 12. Wilmore, D. W., and Dudick, S. J.: Growth and development of an infant receiving all nutrients exclusively by vein. JAMA 203:860,1968. 13. Filler, R. M., Eraklis, A. J., Rubin, V. G., and Das, J. 8.: Long-term parenteral nutrition in infants. New Eng. J. Med. 281: 589,1969.
Discussion Dr. E. Lerois (St. Louis): I am pleased that the authors have emphasized the difference between antenatal and perinatal types of gastroschisis. I would like to express our indebtedness to Dr. Thomas Moore for defining the pathology of this problem 15 yr ago and giving us some sense of direction in its surgical management; and also to Dr. Schuster for his fine contribution in the use of prosthetic coverage in omphaloceles and gastroschisis. However, I would like to emphasize that because a patient has gastroschisis, he does not always require a prosthesis in the correction of the problem, nor does he always require parenteral alimentation for survival. During the past 5 yr at Cardinal Glennon Memorial Hospital for Children in St. Louis, we had 20 cases of gastroschisis, with 16 survivals. All of the survivors except two were closed primarily. I believe that all of the perinatal group can be treated by stretching the abdominal wall and primary closure. The antenatal group will require the use of a silon pouch. However, there is a group with sufficient edema and reaction in the intestinal wall to preclude closure of the abdominal wall, although the intestines can be replaced in the peritoneal cavity. In this group, a silon patch will relieve intraabdominal pressure. Closure can be accomplished early or late, depending on the general condition of the infant. The authors have emphasized the early return of ability to eat in most cases after primary closure. Parenteral alimentation should only be used if essential for survival and not as routine treatment. Dr. E. Wrenn (Memphis): I would like to make a point about the definition of gastroschisis. All of the illustrations in the paper seemed to show the defect through the umbilical ring, which suggests that the patients had ruptured omphaloceles rather than gastroschisis. It is my understanding that gastroschisis is a defect outside the umbilical ring. Very few authors seem to make that differentiation, but I feel it is important to separate these tw6 entities since they arise from different embryologic origins. Dr. W. Kiesewetter (Pittsburgh): We have measured the bowel length in nine patients with omphalocele, and agree with the authors that they do have a much shorter bowel. We have also measured intestinal length in 27 newborns with other neonatal conditions, and find a direct relationship between the weight of the child and length of the bowel; in other words, the larger the baby, the longer the bowel. We did not, however, conhm 267 cm as being average. Only one exceeded 267 cm, and most were somewhere between 180 and 220cm. Dr. R. Izant (Cleuefand): It should be mentioned that Dr. Clatworthy pointed out many, many years ago that we should be measuring numbers of vascular arcades in the mesentery, rather than the linear length of bowel. It means more from a functional standpoint and is a much more accurate estimate of bowel length. Further work should be done on this issue.
GASTROSCHISIS AND SHORT BOWEL
007
Dr. S. R. Schuster (Boston): In gastroschisis (contrasted to omphalocele) one is more likely to be able totally to reduce the herniated viscera and effect a single-stage primary repair. This is particularly true for those patients in which the liver is not part of the herniated mass. This easier reduction in gastroschisis patients results because the midgut has already returned to the abdomen during fetal life to claim its right of domain. However, in omphalocele the bowel has never returned. The abdomen has never accommodated to the size necessary for the midgut and possible other viscera, such as liver, spleen, and stomach. Measurement of intestinal length in patients with an antenatal ruptured omphalocele or gastroschisis is highly unreliable. There is usually considerable reaction of the bowel and mesentery with shortening of these structures and actual conglomeration so that separate loops of intestine are sometimes indistinguishable. There is probably more actual length of intestine than one thinks. Dr. Purunik: We agree with most of the observations of Dr. Lewis. In answer to Dr. Wrenn’s question, all of these cases are true gastroschises and satisfy the criteria set forth by Drs. Moore and Stokes. The insertion of the umbilical cord was not seen in all our illustrations because in some cases it was removed prior to obtaining the first picture and in others it was covered in an attempt to demonstrate a different aspect of the pathology. None of these, cases represent a ruptured omphalocele. With regard to bowel length in the neonates, our observations are different from those of Dr. Kiesewetter. We have found no correlation between body weight from 2.3 to 3 kg. Therefore, we do not think that the “short bowel” in gastroschisis is due simply to the low birth weight. We agree with both the points stated by Dr. Schuster. First, the abdominal space in these babies is not actually diminished, but only relatively so, because of the thick, edematous, and matted viscera. The disparity rapidly disappears and the viscera can be replaced into the abdominal cavity within about 8 days, even in the most severe cases, Second, the bowel is probably foreshortened only from the puckering, accordian-like effect to the serosa by the exudate covering it. As the exu&te is absorbed after closure, the bowel attains its real or near-normal length rapidly. This was proved in two of our cases who were reexplored after a short interval.