Children with Short Bowel Syndrome: Intestinal Rehabilitation versus Transplant

Clarivet Torres¹, Mohan Parvathi¹, Sona Sehgal^1,2, Artis Krystal¹, Cerezo Carola¹, Pennington Roshnee¹, Higginbotham Karen¹, Anthony Sandler²

¹Gastroenterology, Children's National Medical Center, Washington , DC, United States; ²Pathology, Hôpital Necker-enfants malades, Paris, France; ²Surgery, Childrens National Medical Center, Washington , DC, United States

The treatment of patients with short bowel syndrome (SBS) is long term parenteral nutrition (PN), Intestinal rehabilitation (IR) or transplantation. PN has been the primary treatment for SBS patients but can be associated with severe complications. Infants with SBS have a 3-fold increase in mortality than in controls with the same underlying condition but no SBS (37.5% vs. 13.3%) [1]. IR has improved the outcomes during the last decade [2]. We reviewed the outcomes of 78 patients with SBS treated at the Intestinal Rehabilitation Program (IRP) at Childrens National Medical Center (CNMC), Washington DC, during the past 5.5 years.

Seventy eight SBS patients with 3.5 month median time of PN dependency (1-164 months) were enrolled. Median age was 5 months with a median intestinal length of 40cm. The initial median daily caloric requirements by PN were 100%. Forty eight patients (61%) had hyperbilirubinemia (mean DB of 10.2mg/dl, range: 3–32mg/dl), 20 had liver biopsies (12 portal fibrosis, 5 bridging fibrosis, 3 cirrhosis). Height, weight Z score, platelet, albumin, and bilirubin were obtained at the beginning and end of the study.

All 48 patients with hyperbilirubinemia normalized their bilirubin over a mean time of 10.4 weeks using soy bean intralipid (SBIL). Out of the 78 patients, 39 (81%) reversed their cholestasis while receiving PN, 25 patients had 28 lengthening procedures (9 Bianchi, 19 STEP), and 9 patients had ostomy in continuity due to severe bowel dysmotility. Four were listed for intestinal transplant: 2 were transplanted (one with 4cm of jejunum and the other with 10cm of jejunum), one listed patient was weaned off PN (initial DB of 12mg/dl, 10cm of bowel length and half colon), and the fourth is inactive with no signs of liver disease (initial DB 19mg/dl, 10cm of jejunum joined to his sigmoid colon s/p Bianchi). One died (cardiac anomalies).  Among the 75 remaining patients, the mean caloric requirements by PN decreased from 100% to 9% and 60 (80%) patients were weaned off PN over a median time of 4 months (1-60 months).  All laboratory parameters showed improvement (p< 0.0001). Overall survival was 98.7%.

Patients with SBS treated at CNMC reverse their cholestasis using SBIL effectively and in a shorter time compared with recent reports using Omegaven [3]. With meticulous medical/surgical management, patients with advanced liver disease can improve their liver function and nutritional parameters with the ability to discontinue PN and avoid transplantation. Our IRP at CNMC has shown one of the best survivability rates (98.7%) for patients with SBS with medical and non-transplant surgical treatment. The treatment of SBS PN-dependent patients should be based on medical and non-transplant surgical options. Intestinal transplant should only be considered when those measures fail. {figure1}