Support for Short Bowel Syndrome Goes Beyond the N/IICU

Baby boy A was born via emergency cesarean section at 26 weeks EGA to a 41-year-old G1P0 mother with pre-eclampsia at a Philadelphia suburban community hospital. At a birth weight of only 710 grams, he was intubated, given surfactant, and transferred to the NICU. By day of life 22, he was just approaching tolerance of full volume maternal breast milk OG feeds when he developed massive abdominal distention and bilious emesis. An exploratory laparotomy revealed necrotizing enterocolitis (NEC) totalis. After intraoperative discussion with his mother, he underwent near total small bowel resection, leaving him with a duodenostomy just proximal to the ligament of Treitz and a mucous fistula that was 10 cm proximal to the ileocecal valve. He was transferred to Children’s Hospital of Philadelphia’s Newborn/Infant Intensive Care Unit (N/IICU) for further management.

Within days of his transfer, the N/IICU team consulted Children’s Hospital’s Intestinal Rehabilitation Program (IRP). The IRP team followed him closely throughout the hospitalization, providing guidance to optimize a gradual transition from parenteral to enteral nutrition. The distal bowel was gradually conditioned by refeeding the duodenostomy contents into the mucous fistula until the intestine was placed back in continuity on DOL 56. Diarrhea was managed with a combination of elemental formula, pectin, loperamide, and cholestyramine.

The baby’s N/IICU course was complicated by parenteral nutrition-associated liver disease, which was treated with reductions in intravenous fat emulsion and Omegaven. His conjugated hyperbilirubinemia resolved shortly after initiating these therapies, and his liver synthetic function was maintained despite longstanding parenteral nutrition dependence. Central venous access sites were preserved in collaboration with the Interventional Radiology team, by rewiring existing central venous catheters without repeated vessel cannulation, thus avoiding thrombosis.

He was eventually transitioned to a floor dedicated to children with multisystem complexities, where he was jointly managed by the IRP and the Integrated Care Service (ICS). This allowed for good continuity of care and continued intensive pediatric expertise to attend to his global needs and complications of prematurity. His mother was able to room with him and become comfortable with his care before he was discharged to home with parenteral nutrition. He was seen every few weeks in the outpatient IRP clinic, where he was followed by the same familiar clinicians who cared for him in the inpatient setting.

PN was weaned off at 21 months of age. He is now an active 4-year-old who attends preschool and is thriving on a regular diet, supplemented with a minimal amount of overnight gastrostomy feeds.


Necrotizing enterocolitis (NEC) leading to short bowel syndrome (SBS) is one of the most devastating complications of prematurity. With the help of innovative collaborative programs such as the IRP and the ICS, our N/IICU is able to provide state-of-the-art care through discharge and beyond.

The IRP, created in 2009, provides comprehensive care to children with intestinal failure. Intestinal failure occurs when the intestine’s absorptive capacity is inadequate to meet the patient’s nutrition, hydration, electrolyte, and growth requirements, resulting in a need for parenteral nutrition for greater than 90 days. This may be due to an intrinsic intestinal abnormality or SBS. SBS occurs in 24.5/100,000 of all live births and in 353/100,000 of all premature (<37 weeks) live births, with 80 percent of pediatric SBS occurring in the neonatal period, most commonly from NEC and gastroschisis with intestinal atresias.

SBS is a life-threatening condition, but with the development of programs such as the IRP, the mortality rate (mainly from bloodstream infections and parenteral nutrition-associated liver disease) has dropped from between 20 percent and 40 percent in the 1990s to less than 10 percent today. The IRP is a multidisciplinary team that includes a gastroenterologist, surgeon, nurse, interventional radiologist, and social worker as well as dietitians and psychologists. The team collaborates with the neonatal and neosurgical teams to use a variety of medications and parenteral and enteral nutrition manipulations to foster intestinal adaptation and limit liver disease. From a surgical standpoint, the IRP offers a range of operations when medical options are limited, including bowel lengthening surgery.

“Over time, many children with intestinal failure can experience intestinal adaptation, where the bowels grow and gradually gain enough function to permit independence from intravenous nutrition,” says Tina Bales, MD, pediatric gastroenterologist and medical director of the IRP. But for those patients with extreme cases of intestinal failure, the IRP also provides referral to the nation’s best intestinal transplantation centers and helps families coordinate and finance travel, nursing, medical care, and housing while visiting these centers.

The IRP works closely with our institution’s ICS, an inpatient general pediatrics team dedicated to the care of medically complex patients. Annique Hogan, MD, medical director of the ICS, says, “These patients often have multiple specialists involved in their care, require recurrent inpatient admissions, and benefit from an inpatient medical home model.”

The ICS team consists of a small and select group of attending physicians, along with residents, nurses, dietitians, occupational therapists, physical therapists, speech therapists, a social worker, and a nurse case manager who offer experience and expertise caring for medically complex children. This often involves developing and implementing comprehensive care plans, assessing effectiveness of the plan during an inpatient stay, and preparing for discharge to home.

For infants in our N/IICU who are medically appropriate and would benefit from having an inpatient medical home, the ICS team performs an inpatient consult in the N/IICU and meets the family prior to transfer to ensure a safe transition. Infants with hypotonia, genetic syndromes, and SBS are among the infants who are likely to benefit from a transfer to ICS if medically appropriate.

Originally published September 2016 in Neonatology Update.