GASTROSCHISIS OMPHALOCELE FOLLOW UP

Further Inpatient Care (gastroschisis omphalocele follow up):
• Omphalocele
o Babies with omphalocele usually have rapid return of intestinal function after surgical repair, even if intestinal atresia occurs concomitantly, because no associated gut inflammation is present.
o Babies with giant omphaloceles usually have a protracted hospital course; and overall morbidity and mortality is higher for these patients. Multiple procedures are necessary to obtain closure of the abdominal wall defect.
o Respiratory compromise may complicate the repair and require prolonged support and possibly a tracheotomy. Ventilator management, tracheotomy care, and, ultimately, decannulation require close cooperation by the neonatologist, pulmonologist, and pediatric surgeon.

• Gastroschisis
o Even if primary closure of the abdominal wall defect is obtained, a period of several weeks of intestinal dysfunction (ileus) usually follows, as a result of associated gut inflammation. In this situation, parenteral nutrition is essential, followed by the gradual introduction of enteral feedings. Continuous drip feedings usually are tolerated optimally.
o If reduction of the herniated intestine requires the use of a silo, it usually is removed within 5-7 days. The period of ileus follows, during which the baby requires parenteral nutrition until the gradual return of intestinal function. If this expected recovery does not occur within 3-4 weeks, intestinal obstruction is presumed, and a contrast study is obtained to document intestinal transit.
o If intestinal obstruction is present, a laparotomy must be performed.

Further Outpatient Care:
• After hospital discharge, babies require close follow-up care to assess growth and weight gain
• Patients usually have gastroesophageal reflux and may require medical therapy, but fundoplication should not be necessary.
• Hirschsprung disease (aganglionic megacolon) also may occur. Physicians should be alert to a history of constipation.

Transfer:
The best way to treat the exposed intestines of a baby with gastroschisis who is being transported to a tertiary center includes the application of a moist lap pad. The moist lap pad is placed over the intestines and held directly over the abdominal wall defect with dry Kerlix wrap applied around the baby's torso including the extruded intestine. This prevents traction upon the mesentery. A warm, wet, lap pad placed in a bowel bag with the eviscerated intestine soon becomes a cold, wet, lap pad.

Complications (gastroschisis omphalocele):
• The following case report illustrates some of the complications that a baby with giant omphalocele might experience.
o A 6-week-old male infant from Accra, Ghana, presented with a giant omphalocele. His liver and intestines were contained within the omphalocele sac, and his abdominal cavity was undeveloped and diminutive. The sac was partly covered by skin and partly by granulation tissue.

o An open wound is a metabolic drain. Despite devoted nursing care, the nutritional status of this patient was not good. The granulating portion of the wound was excised, and the abdominal cavity was closed with a patch; however, skin approximation was not possible without extensively undermining the flaps. This was inadvisable because of the risk of wound infection and the desire to avoid too tight a closure of the abdominal cavity, which could compromise the infant's ability to ventilate by limiting diaphragmatic excursion and compromise cardiovascular function by diminishing blood return. The patient was treated with antibiotics and parenteral nutrition. Postoperatively, the wound appeared clean, and the patch incorporated well into the baby's tissue. The patient was weaned from the ventilator, despite copious respiratory secretions.

o Two weeks later, the patch was tightened, approximating the skin flaps. However, this precipitated respiratory failure and ventilator dependency. The patient would not be able to return to his family in Africa if he was ventilator-dependent. While the patient's respiratory status was monitored, his patch became infected, and he became critically ill. The only way to resolve the infection was to remove the patch. The author attempted to stretch the abdominal wall and obtain wound closure without a patch; however, this was not possible because hepatomegaly and soft tissue edema combined to produce a small rigid abdominal cavity. The only recourse was to mobilize flaps and move skin from his flank onto the exposed abdominal viscera, and skin graft the donor wounds.

o The patient's condition improved dramatically once closure of the abdominal cavity was achieved. Again, the author tried to wean him from the ventilator, but his copious secretions and episodes of high fever and drenching sweats prevented this. Finally, it was determined that the patient was experiencing narcotic withdrawal. He had been postoperative for so long, and narcotics had been used liberally to provide postoperative pain relief.

o The need for long-term ventilator assistance was realized, and a tracheotomy was performed. Although wound closure was achieved, a huge ventral (abdominal wall) hernia had been created. The skin flaps were separated from the abdominal viscera, and a new patch was inserted. This added rigidity to his abdominal wall, and, by stabilizing the patient's trunk musculature, movements of his torso, including breathing, were facilitated. The patch gradually separated from the rectus fascia, and this dehiscence required repair. The patient was weaned from the ventilator to pressure support. Discharge from the hospital seemed imminent.

o One night, a low-grade fever developed, and the patient became irritable. A culture was taken, but the patient was not treated with antibiotics. Death occurred within 6 hours. The blood culture grew group B streptococci, but the autopsy was otherwise unremarkable. The initial wound culture grew Pseudomonas species, which was later cultured from the patient's tracheal secretions. The wound infection, which required removal of the patch, was caused by methicillin-resistant Staphylococcus aureus (MRSA).

Prognosis (gastroschisis omphalocele):
• Omphalocele
o Prognosis is dependent upon the severity of the associated problems. Babies with omphalocele are considerably complex, with involvement of many other organ systems.
o Even giant omphaloceles can be closed, although multiple procedures may be necessary.
o The limiting factor for many of these babies, however, is their diminutive thoracic cavities and associated pulmonary hypoplasia and resultant chronic respiratory failure. Even so, lung growth and development continue well into childhood, encouraging optimism regarding the ultimate prognosis.

• Gastroschisis
o Prognosis is dependent mainly upon severity of associated problems, including prematurity, intestinal atresia, short gut, and intestinal inflammatory dysfunction.
o Many pediatric surgeons believe that prognosis has improved because of maternal ultrasound diagnosis and monitoring, which leads to expeditious delivery of babies at tertiary centers.
o Years ago, obtaining primary closure of a baby with gastroschisis was unusual. Usually, it was necessary to use a silo. Now, primary closure is commonly attained.

Patient Education:
• Instruct parents regarding the significance of bilious (green) vomiting, since these babies may develop adhesive small bowel obstruction or midgut volvulus.
• Inform parents that their child's appendix is probably in an unusual location and that a CT scan may be the most reliable way to diagnose acute appendicitis.