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Gastroschisis
Gastroschisis
C. W. Miller, MD, Little Rock, Arkansas
Joe Crumpler,
MD, Little Rock, Arkansas
Gilbert S. Campbell, MD, Little Rock, Arkansas
Gastroschisis is a dramatic congenital defect demanding urgent surgical therapy. This anomaly is characterized by a cleft in the abdominal wall which is extraumbilical in position, normal insertion of the umbilical cord, and absence of any covering sac over the abdominal viscera which are trapped outside the abdominal wall. In recent years, the resurgence of articles on gastroschisis has paralleled the greater success which has been achieved in the management of this relatively rare condition [I]. Recently we have had the opportunity to treat two infants with gastroschisis and believe that the application of basic surgical advances contributed to the success in the management of these infants. Case Reports Case I. The patient (BBP) was a white male infant born at thirty-six weeks’ gestation. Gastroschisis was correctly diagnosed and the infant was transferred to the University of Arkansas Medical Center at two hours of age. The infant’s body weight was 2,600 gm. Initial studies revealed hypothermia and acidosis. There was a 3 cm abdominal wall defect present on the right side which resulted in evisceration of the small and large bowel. After correction of acidosis and hypothermia, the infant was taken to the operating room at six hours of age. The viscera were returned to the abdominal cavity with primary skin closure. During the first two weeks after operation, satisfactory oral intake was not adequate and the infant continued to lose weight. At this time, it was noted that a
From the University of Arkansas Medical Center, Little Rock, Arkansas. Reprint requests should be addressed to Dr Miller, Department of Surgery. University of Arkansas Medical Center, Little Rock, Arkansas 72201. Presented at the Twenty-Fourth Annual Meeting of the Southwestern Surgical Congress, Albuquerque, New Mexico, May 1-4, 1972.
754
small bowel fistula had developed and this complication was managed by discontinuing oral feedings and establishment of parenteral hyperalimentation. This was continued for a period of four weeks when blood cultures were found to be positive for Candida albicans. The intravenous catheter was removed and signs of sepsis abated. The fistula closed and healing of the abdominal wall occurred during hyperalimentation support. Oral feedings were then tolerated. The infant was discharged having a weight of 4,100 gm . Case II. The patient (BGL), a white female infant with a birth weight of 1,900 gm, was delivered approximately one month prematurely and referred to the University of Arkansas Medical Center with the diagnosis of gastroschisis. (Figure 1.) The infant was hypothermic and acidotic upon admission. Physical examination demonstrated a 4 cm abdominal wall defect in the right lower quadrant through which the liver, small and large bowel, and the stomach had eviscerated. The infant was operated upon at twelve hours of age. The surgical procedure consisted of primary skin closure, gastrostomy, and insertion of a Silastic@ catheter in the internal jugular vein for hyperalimentation. The postoperative course was uneventful. Hyperalimentation was used for ten days at which time oral diet was tolerated without difficulty. The abdominal wall healed primarily and the infant was discharged at one month of age with a body weight of 2,300 gm. (Figure 2.) Comments
The treatment of gastroschisis necessitates that the displaced abdominal contents be covered. In earlier years when the viscera were forced into the small abdominal cavity, three major complications were seen: respiratory, cardiovascular, and gastrointestinal. Excessive elevation of intra-abdominal pressure
The American
Journal of Surgery
Gastroschisis
Figure 1. Preoperative appearance tents with absence of any sac.
of eviscerated
con-
in a defenseless anesthetized infant resulted in marked respiratory embarrassment, compression of the vena cava with resultant decrease in return of blood to the heart, and marked compression of an already thickened, edematous, and matted bowel. In 1948, Gross [2] described the two stage treatment of large omphaloceles by closure of skin flaps over the intact sac (making no attempt to crowd the viscera into the small abdominal cavity) and secondary repair of the ventral hernia months later [2]. This technic has contributed to the successful treatment of gastroschisis since from a surgical standpoint gastroschisis and a large omphalocele present similar problems. Skin flap closure can frequently be accomplished after manual enlargement of the small abdominal cavity prior to proper placement of the extra-abdominal viscera. Some gain in the disparity between the small abdominal cavity and the distended matted abdominal viscera can be accomplished by intraoperative decompression including gastrostomy. Schuster’s [3] description of a prosthetic pouch as a temporary cover for eviscerated abdominal contents shows an excellent alternative when Gross’s technic cannot be utilized. Many of these infants are desperately ill by the time they are brought to a medical center and blood gas monitoring pre-, intra-, and postoperatively has been helpful. The thickened distended matted bowel may be slow to develop normal function. Parenteral hyperalimentation, as pioneered by Dudrick [4], proved to be life-saving in our two infants. We now insert a Silastic catheter into the internal jugular vein immediately after repair of gastroschisis, and this catheter
Volume 124, December 1972
Figure 2. closure.
Postoperative
appearance
with primary
skin
is tunneled subcutaneously and exits above and behind the ear. The basic solution for hyperalimentation in infants requires modification to lessen the likelihood of hyperosmolar problems. (Table I.) The solution is delivered by a continuous infusion TABLE I
Preparation of Pediatric Hyperalimentation Solutions [5] Amount Composition
(ml)
Caloric Value
Unit composition of base solution 5% glucose in 5% fibrin hydrolysate (20 gm) 50% glucose (20 gm) Subtotal Additions to each unit of base solution Sodium (20 mEq) Potassium (25 mEq) Phosphorus (25 mEq) Calcium gluconate (20 mEq) Magnesium (10 mEq) Multiple vitamin infusion Vitamin K Vitamin B12 (added to solution daily or weekly or given intramuscularly) Folic acid Iron Trace elements (added to solution daily or given as 10 ml/kg plasma twice weekly) Subtotal Final solution (given ar rate of 145 ml/kg/day)
400 250 650
160 kcal 500 kcal 660 kcal
10.0 13.0 44.0 1.2 4.0
1.0
1.0 75.0 750
130 kcal/kg/day
755
Miller, Crumpler,
and Campbetl
pump for precise control of the rate of fluid intake. A 0.22~ Millipore filter is used for prevention of contamination which could arise from the nutritional solution and/or tubing. Strict local care is given to the site of entrance of the intravenous catheter, and the tubing is changed daily to avoid external contamination. The use of hyperalimentation is not without its complications, the most common of which is sepsis. The most common organisms are Candida albicans and Micrococcus pyogenes. Removal of the central venous catheter controls most of these infections. Summary
Two infants with gastroschisis have been treated successfully. The application of the following basic principles should lead to improvement in survival for infants with this anomaly.
756
1. Preoperative correction of acidosis and control of hypothermia. 2. Coverage of the eviscerated organs with skin flaps or prosthetic material without tension, and insertion of a gastrostomy tube. 3. Use of hyperalimentation until normal gastrointestinal function returns. References 1. Wesselhoeft C. Porter A, DeLuca F: Treatment of omphalocele and gastroschisis. Amer J Surg 123: 369, 1972. 2. Gross RC: A new method for surgical treatment of large omphaloceles. Surgery 24: 277, 1948. 3. Schuster SR: A new method for the staged repair of large omphaloceles. Surg Gynec Obstet 125: 837, 1967. 4. Wilmore DW, Groff DE, Bishop HC, Dudrick SJ: Total parenteral nutrition in infants with catastrophic gastrointestinal anamolies. J Pediat Surg 4: 181, 1969. 5. Dudrick SJ, Rhoads JE: Intravenous hyperalimentation, chapt 30, p 654. Critical Surgical Illness. Philadelphia, Saunders, 1971.
The American Journal 01 Surgery
C. W. Miller, MD, Little Rock, Arkansas
Joe Crumpler,
MD, Little Rock, Arkansas
Gilbert S. Campbell, MD, Little Rock, Arkansas
Gastroschisis is a dramatic congenital defect demanding urgent surgical therapy. This anomaly is characterized by a cleft in the abdominal wall which is extraumbilical in position, normal insertion of the umbilical cord, and absence of any covering sac over the abdominal viscera which are trapped outside the abdominal wall. In recent years, the resurgence of articles on gastroschisis has paralleled the greater success which has been achieved in the management of this relatively rare condition [I]. Recently we have had the opportunity to treat two infants with gastroschisis and believe that the application of basic surgical advances contributed to the success in the management of these infants. Case Reports Case I. The patient (BBP) was a white male infant born at thirty-six weeks’ gestation. Gastroschisis was correctly diagnosed and the infant was transferred to the University of Arkansas Medical Center at two hours of age. The infant’s body weight was 2,600 gm. Initial studies revealed hypothermia and acidosis. There was a 3 cm abdominal wall defect present on the right side which resulted in evisceration of the small and large bowel. After correction of acidosis and hypothermia, the infant was taken to the operating room at six hours of age. The viscera were returned to the abdominal cavity with primary skin closure. During the first two weeks after operation, satisfactory oral intake was not adequate and the infant continued to lose weight. At this time, it was noted that a
From the University of Arkansas Medical Center, Little Rock, Arkansas. Reprint requests should be addressed to Dr Miller, Department of Surgery. University of Arkansas Medical Center, Little Rock, Arkansas 72201. Presented at the Twenty-Fourth Annual Meeting of the Southwestern Surgical Congress, Albuquerque, New Mexico, May 1-4, 1972.
754
small bowel fistula had developed and this complication was managed by discontinuing oral feedings and establishment of parenteral hyperalimentation. This was continued for a period of four weeks when blood cultures were found to be positive for Candida albicans. The intravenous catheter was removed and signs of sepsis abated. The fistula closed and healing of the abdominal wall occurred during hyperalimentation support. Oral feedings were then tolerated. The infant was discharged having a weight of 4,100 gm . Case II. The patient (BGL), a white female infant with a birth weight of 1,900 gm, was delivered approximately one month prematurely and referred to the University of Arkansas Medical Center with the diagnosis of gastroschisis. (Figure 1.) The infant was hypothermic and acidotic upon admission. Physical examination demonstrated a 4 cm abdominal wall defect in the right lower quadrant through which the liver, small and large bowel, and the stomach had eviscerated. The infant was operated upon at twelve hours of age. The surgical procedure consisted of primary skin closure, gastrostomy, and insertion of a Silastic@ catheter in the internal jugular vein for hyperalimentation. The postoperative course was uneventful. Hyperalimentation was used for ten days at which time oral diet was tolerated without difficulty. The abdominal wall healed primarily and the infant was discharged at one month of age with a body weight of 2,300 gm. (Figure 2.) Comments
The treatment of gastroschisis necessitates that the displaced abdominal contents be covered. In earlier years when the viscera were forced into the small abdominal cavity, three major complications were seen: respiratory, cardiovascular, and gastrointestinal. Excessive elevation of intra-abdominal pressure
The American
Journal of Surgery
Gastroschisis
Figure 1. Preoperative appearance tents with absence of any sac.
of eviscerated
con-
in a defenseless anesthetized infant resulted in marked respiratory embarrassment, compression of the vena cava with resultant decrease in return of blood to the heart, and marked compression of an already thickened, edematous, and matted bowel. In 1948, Gross [2] described the two stage treatment of large omphaloceles by closure of skin flaps over the intact sac (making no attempt to crowd the viscera into the small abdominal cavity) and secondary repair of the ventral hernia months later [2]. This technic has contributed to the successful treatment of gastroschisis since from a surgical standpoint gastroschisis and a large omphalocele present similar problems. Skin flap closure can frequently be accomplished after manual enlargement of the small abdominal cavity prior to proper placement of the extra-abdominal viscera. Some gain in the disparity between the small abdominal cavity and the distended matted abdominal viscera can be accomplished by intraoperative decompression including gastrostomy. Schuster’s [3] description of a prosthetic pouch as a temporary cover for eviscerated abdominal contents shows an excellent alternative when Gross’s technic cannot be utilized. Many of these infants are desperately ill by the time they are brought to a medical center and blood gas monitoring pre-, intra-, and postoperatively has been helpful. The thickened distended matted bowel may be slow to develop normal function. Parenteral hyperalimentation, as pioneered by Dudrick [4], proved to be life-saving in our two infants. We now insert a Silastic catheter into the internal jugular vein immediately after repair of gastroschisis, and this catheter
Volume 124, December 1972
Figure 2. closure.
Postoperative
appearance
with primary
skin
is tunneled subcutaneously and exits above and behind the ear. The basic solution for hyperalimentation in infants requires modification to lessen the likelihood of hyperosmolar problems. (Table I.) The solution is delivered by a continuous infusion TABLE I
Preparation of Pediatric Hyperalimentation Solutions [5] Amount Composition
(ml)
Caloric Value
Unit composition of base solution 5% glucose in 5% fibrin hydrolysate (20 gm) 50% glucose (20 gm) Subtotal Additions to each unit of base solution Sodium (20 mEq) Potassium (25 mEq) Phosphorus (25 mEq) Calcium gluconate (20 mEq) Magnesium (10 mEq) Multiple vitamin infusion Vitamin K Vitamin B12 (added to solution daily or weekly or given intramuscularly) Folic acid Iron Trace elements (added to solution daily or given as 10 ml/kg plasma twice weekly) Subtotal Final solution (given ar rate of 145 ml/kg/day)
400 250 650
160 kcal 500 kcal 660 kcal
10.0 13.0 44.0 1.2 4.0
1.0
1.0 75.0 750
130 kcal/kg/day
755
Miller, Crumpler,
and Campbetl
pump for precise control of the rate of fluid intake. A 0.22~ Millipore filter is used for prevention of contamination which could arise from the nutritional solution and/or tubing. Strict local care is given to the site of entrance of the intravenous catheter, and the tubing is changed daily to avoid external contamination. The use of hyperalimentation is not without its complications, the most common of which is sepsis. The most common organisms are Candida albicans and Micrococcus pyogenes. Removal of the central venous catheter controls most of these infections. Summary
Two infants with gastroschisis have been treated successfully. The application of the following basic principles should lead to improvement in survival for infants with this anomaly.
756
1. Preoperative correction of acidosis and control of hypothermia. 2. Coverage of the eviscerated organs with skin flaps or prosthetic material without tension, and insertion of a gastrostomy tube. 3. Use of hyperalimentation until normal gastrointestinal function returns. References 1. Wesselhoeft C. Porter A, DeLuca F: Treatment of omphalocele and gastroschisis. Amer J Surg 123: 369, 1972. 2. Gross RC: A new method for surgical treatment of large omphaloceles. Surgery 24: 277, 1948. 3. Schuster SR: A new method for the staged repair of large omphaloceles. Surg Gynec Obstet 125: 837, 1967. 4. Wilmore DW, Groff DE, Bishop HC, Dudrick SJ: Total parenteral nutrition in infants with catastrophic gastrointestinal anamolies. J Pediat Surg 4: 181, 1969. 5. Dudrick SJ, Rhoads JE: Intravenous hyperalimentation, chapt 30, p 654. Critical Surgical Illness. Philadelphia, Saunders, 1971.
The American Journal 01 Surgery