Sequential sac ligation for giant omphalocele

Sequential By A.R.

Sac Ligation

for Giant Omphalocele

Hong, D.L. Sigalet, F.M. Guttman, Montreal,

0 The authors describe a technique for the management

J.M. Laberge, Quebec

CASE

of

giant omphalocele

and D.P. Croitoru

REPORTS

in which the sac is not resected, but used to sequentially reduce the contents into the abdominal cavity. Three patients were treated in this fashion. Delayed

Three patients have been treated in this fashion; two (both full-term, 2.6 and 3.2 kg) to complete closure, and one (premature. 1.8 kg) for delayed placement of a silo.

primary fascial closure was achieved in two. The third patient not have complete reduction after 3 weeks, and tempo-

Case I

did

rary placement of a silastic sheet was required. The sac remained intact in all three cases, and neither infection nor injury to the abdominal contents occurred. This technique has the advantages of maintaining an intact membrane and avoiding the initial operative placement of a prosthetic silo in these sometimes critically ill neonates. Copyright o 1994 by W. B. Saunders Company INDEX WORDS:

Omphalocele.

M

ANAGEMENT of the patient with a giant omphalocele remains a difficult problem.’ Rapid reduction of the contents of the sac into the abdomen is not always easily accomplished, especially if the liver is herniated through the defect. Standard therapy in most centers involves resection of the sac, and at the same time, placement of a silastic silo that can be used for gradual reduction of the herniated contents. This form of therapy requires an operation and general anesthetic shortly after birth. Two groupsz,3 recently described a variant of Schuster’s technique,” which avoids resection of the omphalocele sac, and adds a silastic sheet over the intact membrane. We describe a modification, in which the prosthetic sheet is eliminated, and the sac itself is used for sequential reduction.

MATERIALS

AND METHODS

All patients were delivered by cesarean section after the diagnosis of giant omphalocele was made with antenatal ultrasound. After transfer to the neonatal intensive care unit, the patients were resuscitated and stabilized as necessary. The following sac ligation technique was used. After cleansing with dilute chlorhexidene, the sac was gently kneaded to release any adherent intestinal or liver tissue. Traction on the cord was applied, and the contents were gently partially reduced. The sac was then twisted and ligated with sterile umbilical ties (Fig 1). In one case, the umbilical cord was positioned at the base of the sac (Fig 2), and the apex of the sac was twisted and ligated with sterile cord clamps (Fig 3). Central venous pressure was monitored continuously during the reduction. In the last case, intragastric pressures were also monitored during the later stages of reduction. The procedure was repeated every 24 to 48 hours until complete reduction was obtained (Fig 2). Desiccation between reductions was prevented with moist gauze and occlusive dressing. Additional support for the sac was provided by suspending it. The infants were sedated with intravenous morphine before each reduction. JournalofPed&ric

Surgery, Vol29, No 3 (March), 1994: pp 413-415

The patient was a full-term girl born by elective cesarean section. The ultrasound diagnosis had been made at 30 weeks’ gestation. The sac was more than 7 cm in diameter at its widest part. Almost all the liver was contained within the sac, as well as most of the intestines. Additional workup showed an atrial septal defect (ASD) and a significant ventricular septal defect (VSD). The patient underwent sequential sac ligation for 7 days. On the second day of life. the patient was intubated to allow the administration of muscle relaxants for the reduction. However, paralysis was found not to be necessary, and sedation with intravenous morphine was employed for the remaining stages. She underwent operative repair of the omphalocele on day X of life. Primary closure of the fascia was obtained. The patient was extubated on the fourth postoperative day and was fed 2 days later. There were no postoperative complications. and she subsequently underwent closure of the VSD at 5 weeks of age. The patient was examined at the l-year follow-up and was found to have well-healed surgical incisions. without evidence of ventral hernia. Case

2

This patient was a boy born at 38 weeks’ gestation after a normal pregnancy. The original diagnosis was made at I? weeks’ gestation. Results of the initial examination showed a giant omphalocele, approximately 6 cm in diameter, containing most of the liver. No other anomalies were noted. Sequential ligation was employed daily, and the patient underwent surgery on the third day of life; primary repair of the defect was achieved. The postoperative course was uncomplicated. The patient was extubated on the third postoperative day and was on a full diet 3 days later. He was discharged home in excellent condition by the third week of life. At the l-month follow-up examination, intact repair was observed, without hernia, and the child was gaining weight appropriately. Case

3

This case demonstrates the pitfalls of management of premature infants with giant omphalocele. This infant boy was born at 36 weeks’ gestation. Spontaneous rupture of the membranes led to labor and delivery by cesarean section. There was initial respiratory distress and acidemia, which improved with supplemental oxygen. Results of the initial examination showed a large omphalocele with most of the abdominal contents (including the liver) in the sac. The

From the Department of Pediattic Surgery. The Montreal Children’s Hospital, Montreal, Quebec. Date accepted: February 15, 1993. Address reprint requests to F.M. Guttman, MD, Department of Pediatric Surgery, The Montreal Children’s Hospital. 2300 Tupper St. Montreal, Quebec, Canada. H3H I P3. Copyright ~11994 by W.B. Saunder.s Cornpart) 0022-346819412903-0013$03.0010 413

HONG ET AL

Fig 1. Patient with a giant omphalocele, just before delayed primary closure after sequential reduction using the sac ligation technique. Note the large amount of redundant sac above the umbilical tie.

abdominal wall defect was estimated at 6 cm in diameter, and the maximum diameter of the sac itself was 12 cm. The cord was located at the right inferolateral base of the sac. The cord was ligated, and sequential ligation of the sac using the apex was begun. Approximately 6 hours after the third reduction, the patient became mottled, and respiratory acidosis developed, as well as several apneic spells. He was intubated and maintained on ventilatory support. The cord clamps that had been used for the last reduction were removed, and the patient’s condition improved. Over the next 20 days, the sac was slowly reduced in the same fashion. During this period, the patient had several episodes of fever and was treated empirically with intravenous antibiotics. Results of cultures remained negative. The sac remained intact during this period, and several aspirates of fluid from underneath the sac were also negative for culture. By the 23rd day of life, further reduction became impossible because the remaining sac was adherent to the liver. The patient then underwent surgery. Fascia1 closure was possible initially; however, during dissection of the sac from the liver, and of the fascial edges, the liver became greatly enlarged and congested, and the fascia could no longer be brought together. The defect was finally closed with a silastic patch approximately 4 cm in diameter, and the patient was returned to the neonatal intensive care unit in critical condition. His postoperative course was complicated by severe total body edema and pulmonary congestion requiring very high ventilatory pressures. As

Fig 3. Same infant (as in Fig 2) after partial reduction; note nested cord clamps on apex of the sac.

the edema worsened, intraabdominal pressure increased. The silastic patch was removed, and a silo was placed to provide additional room for the abdominal contents. Oliguria and acute renal failure developed, which required hemodialysis. This resolved, and the hemodialysis was stopped. Additional management problems included large pleural e&ions requiring closed thoracostomy, and a fungal urinary tract infection necessitating systemic amphotericin B. Sixteen days after placement the silo was opened, and the contents were found to be covered by a thick fibrin peel. The next day the silo was removed completely, and the wound was dressed with saline gauze packing. Dressing changes were continued, and the wound surface was allowed to granulate in. The wound finally closed 3 months after the original operation. The patient’s condition improved slowly, and nasogastric feeding was begun 4 weeks after the first operation. The patient was weaned from the ventilator shortly after the wound closed and is now on supplemental oxygen. Although now feeding, gastroesophageal reflux remains a problem. DISCUSSION

Fig 2. Premature infant with cord originating from the base of the omphalocele.

Since Gross” and Schuster’s4 description of the management of giant omphaloceles, many of the problems still remain. Primary closure is complicated by several factors, including a small abdominal cavity, sutures tearing through because of tension, and impaired venous return, with potentially drastic effects on the intestine and kidneys. Respiratory insufficiency from both the mechanical effects of the reduction and from pulmonary hypoplasia (as has been demonstrated by Hershenson et a15)may occur. Methods of treatment have varied from minimal management by escharization of the sac with various agents, popularized by Grob,h to leaving the amniotic sac intact and pulling undermined skin over the sac

SEQUENTIAL

SAC LIGATION

415

FOR GIANT OMPHALOCELE

(Gross’). Schustefl modified this by introducing the use of prosthetic sheeting. Most surgeons currently resect the sac before performing the repair, primarily or staged. The proposed advantage is that anomalies of the intestinal tract such as malrotation or obstruction can be dealt with, or at least noted for future intervention and repair. Staged return of the abdominal contents, especially the liver, is a definite benefit. Acute reduction often results in kinking of the junction of the hepatic veins and the inferior vena cava. This results in engorgement of the liver, compromising the reduction and sometimes resulting in hypovolemia.’ The importance of monitoring intraabdominal pressure during reduction is stressed by all investigators.7-y The infolding of the amniotic sac, without resection, and gradual reduction with a prosthesis, as described by de Lorimier et al,? is a modification of Schuster’s technique4 for intact omphaloceles in that the prosthesis is sutured directly to the skin without incising it. Yukomori et al” described the use of essentially the same technique, in which the prosthesis is sutured under local anesthesia in the neonatal intensive care unit. However, the application of a silo shortly after birth has the disadvantage of limiting the reduction period to 7 to 10 days, after which the prosthesis often dehisces or becomes infected. In our modification, the sac itself is used to begin the reduction of contents into the abdominal cavity, and no prosthesis is placed. The advantage is that this can be accomplished in the neonatal nursery with simple intravenous sedation, and no surgery is required. If

complete reduction of the contents cannot be achieved because of adherence to the liver, a silo can be placed. Our experience with this technique demonstrates that the sac is strong and allows aggressive reduction of the contents, similar to that achieved with a silo. The sac remained intact in all three cases and did not become infected. Dehiscence, rupture, or injury to the intraabdominal contents did not occur. Traction on the sac stretches the abdominal wall and brings the fascia together. Although two of the three patients required ventilatory support during the reduction process, the complications encountered in these patients do not necessarily relate to the technique itself; rather, they may be associated with the overall anomaly. The third case exemplifies the risk of rapid reduction of a giant omphalocele. Despite progressive reduction for 21 days, the additional acute reduction in the operating room resulted in obstruction of the hepatic venous outflow and severe hepatic engorgement. The attempt to gain fascial closure was abandoned, but too late to prevent further complications. The major advantage of this technique is that it obviates surgery while beginning treatment in newborn infants, many of whom are at high risk because of concomitant respiratory distress syndrome or associated anomalies. In addition, allowing the membrane to remain intact may actually lessen the risk of infection. Sequential sac ligation should be considered in patients in whom immediate closure is not possible.

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7. Wesley JR. Drongowski R, Coran AC: Intragastric pressure measurement: A guide for reduction and closure of the silastic chimney in omphalocele and gastroschisis. J Pediatr Surg 16:264270, 1981 8. Lacey SR, Bruce J, Brooks SP, et al: The relative merits of various methods of indirect measurement of intra-abdominal pressure as a guide to closure of abdominal wall defects. J Pediatr Surg22:1207-1211.1987 9. Yaster M, Scherer TLR, Stone MM, et al: Prediction of successful primary closure of congenital abdominal wall defects using intraoperative measurements. J Pediatr Surg ?4:1217-1220. 1989