Long-term evolution of a medical-surgical unit for intestinal rehabilitation
Alba Bueno1, Javier J. Serradilla1, Ane Miren AM Andrés1, Esther E. Ramos2, Alba A. Sánchez1, Alida A. Alcolea2, Francisco F. Hernández1,3, Esteban E. Frauca4, Gerardo G. Prieto2, Manuel M. López-Santamaría1.
1Pediatric Surgery, La Paz University Hospital, Madrid, Spain; 2Pediatric Gastroenterology and Intestinal Rehabilitation Unit, La Paz University Hospital, Madrid, Spain; 3ERN-Transplantchild, IdiPaz, Madrid, Spain; 4Pediatric Hepatology, La Paz University Hospital, Madrid, Spain
Introduction: The complications of intestinal failure (IF) in the childhood are serious and potentially fatal. We study the evolution and achievements of an established Intestinal Rehabilitation Unit.
Patients and Methods: We retrospectively reviewed all patients referred to our Unit from 1997 to 2020; we analyzed demographic and clinical data, diagnostic and therapeutic strategies implemented and long-term evolution. We look for prognostic factors depending on the time of the program, age at first assessment, etiology and associated complications.
Results: 302 children were evaluated (median age at arrival 4.9 months, range 0-18yo). 84% came from other institutions. 69% had short bowel syndrome (SBS), 17% motility disorders and 10% stubborn diarrhea. Within SBS, necrotizing enterocolitis (36%), volvulus (22%), atresia (18%) and gastroschisis (14%) stood out. Of these, 40 had ultra-short bowel (<10 cm).
On arrival, venous access, liver damage (by analysis, biopsy and in recent years fibroscan) and residual bowel length and function were assessed. All were dependent on parenteral nutrition (PN), 44.4% had liver damage (24.1% moderate or severe), and 29.3% had thrombosis of a major venous access (11.4% more than one). Those with SBS described an average of 3 previous surgeries.
Enteral and PN was optimized, using SMOFlipid® recently; Teduglutide® was introduced since 2017 in 13 cases to improve intestinal adaptation; 440 laparotomies were performed in 151 patients: intestinal resections, stomas, enteroplasties, adhesiolysis and transit reconstruction, as well as intestinal elongation techniques (10 STEPS and 3 Bianchis). We used refeeding in 5 infants before stoma closure successfully. Placement and care of venous accesses were optimized working with interventional radiologists and skilled nursing, and severe abdominal wall complications were treated (9 VAC systems). A patient association was also created and a psychologist was included in the multidisciplinary team.
After an average follow-up of 6.1 years (0-24), 73.8% of the patients were alive: 39.2% achieved complete digestive autonomy (1.7% in process), 15% were adapted after an intestinal transplant (IT) (2.3% currently listed) and 13.7% were partially adapted (home PN). In 1% transplantation is contraindicated and they have not adapted. 26% of the patients have died (9% waiting for an).
The group of stubborn diarrhoea presented greater survival and adaptation capacity (p<0.017). Thrombosis and hepatopathy, as well as the program era, had an impact on survival, need for transplantation and adaptation (p<0.05).
Conclusions: Thanks to new medical and surgical therapeutic strategies, as well as to the creation of specialized units, pediatric IF presents better survival than in previous decades. However, children with FI continue to arrive at the Unit with potentially preventable complications that worsen their prognosis in terms of survival and need for transplantation.
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