Appendix I - Percorso del paziente:

http://malattierare.toscana.it/static/media/uploads/sindromeintestinocorto_appendix_i.pdf

Appendix II - Libretto informativo creato dalla Chirurgia Pediatrica AOU Meyer per i genitori di pazienti affetti da SBS:

http://malattierare.toscana.it/static/media/uploads/sindromeintestinocorto_appendix_ii.pdf

http://www.unfiloperlavita.it/          

http://www.shortbowelsurvivor.co.uk/

https://shortbowelfoundation.org/

https://oley.org/

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=104008

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Expert=2301

https://www.uptodate.com/contents/pathophysiology-of-short-bowel-syndrome

1. Coletta R, Khalil BA, Morabito A. Short bowel syndrome in children: surgical and medical perspectives. Semin Pediatr Surg 2014;23(5):291-7.
2. Ba'ath ME, Almond S, King B, Bianchi A, Khalil BA, Morabito A. Short bowel syndrome: a practical pathway leading to successful enteral autonomy. World J Surg 2012;36(5):1044-8.
3. Khalil BA, Ba'ath ME, Aziz A, Forsythe L, Gozzini S, Murphy F, Carlson G, Bianchi A, Morabito A. Intestinal rehabilitation and bowel reconstructive surgery: improved outcomes in children with short bowel syndrome. J Pediatr Gastroenterol Nutr 2012;54(4):505-9.
4. Wood SJ, Khalil B, Fusaro F, Folaranmi SE, Sparks SA, Morabito A. Early structured surgical management plan for neonates with short bowel syndrome may improve outcomes. World J Surg 2013;37(7):1714-7.
5. Bianchi A. From the cradle to enteral autonomy: the role of autologous gastrointestinal reconstruction. Gastroenterology 2006;130(2 Suppl 1):S138-46.
6. Edge H, Hurrell R, Bianchi A, Carlson G, Zaidi T, Gozzini S, Khalil BA, Morabito A. Caregiver evaluation and satisfaction with autologous bowel reconstruction in children with short bowel syndrome. J Pediatr Gastroenterol Nutr 2012;54(4):510-5.
7. Jakobsen MS, Jorgensen MH, Husby S, Andersen L, Jeppesen PB. Low-fat, high-carbohydrate parenteral nutrition (PN) may potentially reverse liver disease in long-term PN-dependent infants. Dig Dis Sci 2015;60(1):252-9.
8. Rege AS, Sudan DL. Autologous gastrointestinal reconstruction: review of the optimal nontransplant surgical options for adults and children with short bowel syndrome. Nutr Clin Pract 2013;28(1):65-74.
9. Romano C, van Wynckel M, Hulst J, Broekaert I, Bronsky J, Dall'Oglio L, Mis NF, Hojsak I, Orel R, Papadopoulou A and others. European Society for Paediatric Gastroenterology, Hepatology and Nutrition Guidelines for the Evaluation and Treatment of Gastrointestinal and Nutritional Complications in Children With Neurological Impairment. J Pediatr Gastroenterol Nutr 2017;65(2):242-264.
10. Soden JS. Clinical assessment of the child with intestinal failure. Semin Pediatr Surg 2010;19(1):10-9.

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Short bowel syndrome is a multisystemic disorder that results from the loss of a significant amount of small bowel. Massive intestinal resection in neonates and infant are due to necrotizing enterocolitis (NEC), intestinal atresia, gastroschisis, and extensive aganglionosis in Hirschsprung.

The goal of treatment in these patients is to achieve complete enteral autonomy while minimizing complications.

Data from a large tertiary centre in Canada shown that the overall incidence of SBS was 22.1 per 1000 neonatal intensive care unit (NICU) admissions and 24.5per 100,000 live births. According to Orphanet the prevalence of SBS is Prevalence: 1-9 / 100 000 live births.

Chances of successful achievement of enteral autonomy (EA) are enhanced by the early (at diagnosis) institution of specialized unit which briefly consists of hepato-sparing parenteral nutrition (PN), careful central venous access management, reconstructive intestinal surgery where indicated, and addressing social and developmental needs.

Hepato sparing TPN involves initially a low-fat regime (maximum of 1.5 g/kg/day of intralipid or 2 g/kg/day of SMOF). During the second 6 months of life, a maximum of 2.5 g/kg/day can be used provided that the liver function tests are normal. This is in addition to aggressive enteral feeding. The services of dieticians in this aspect are extremely important.

Several blood tests to check electrolytes, vitamin B12, ferritin, cupper, magnesium, lactates, bone densitometry are needed to tailor TPN requirement and vitamin supplementation. Furthermore, SBS patients will request though their life several radiological to check liver, bowel and vascular access such as chest x-ray, abdominal x-ray, liver ultrasound, neck ultrasound.

Reconstructive surgery to length and tailor the remnant bowel can involve one or more procedures.

This high specialized surgery is usually preceded by a period of 20–24 weeks of bowel expansion in cases of un-dilated bowel. This is accomplished using a tube stoma on the proximal bowel end. This tube is clamped intermittently (immediately before feeds) to produce gradual proximal bowel distension. This intermittent obstruction of bowel helps to achieve expansion while avoiding mucosal damage and bacterial overgrowth. The effluent of the proximal tube stoma is then recycled into a similar tube stoma in the distal bowel. This is done slowly, and we have found that it helps with absorption of fluids and electrolytes.

Longitudinal Intestinal Lengthening (LILT), serial transverse enteroplasty (STEP) and spiral intestinal lengthening (SILT) are the most common reconstructive surgical approach to improve bowel length. The most important factor in deciding the type of surgery is the remaining bowel length, the quality of the mesentery and the degree of bowel distension present.

It is good practice to monitor the patient time in intensive care during the post-operative time, checking bloods every 24-48hrs, monitoring output and maintaining intake with 24 hours of TPN. After canalization of intestinal content, the patient can re-start the feeding and the reduction of TPN hours will be progressive depending on the improve of enteral feeding quote.

SBS patients will benefit from outpatient check every sixth month in a multidisciplinary outpatient clinic where the family can be assessed by intestinal surgeon, gastroenterologist, dietitian, play therapist and if needed psychologist to meet the patient and family needs.

The common pharmacological therapies to avoid the SBS consequences include antimotility agents, anti-secretory agents, and parenteral infusion therapy. Antimotility medications are used to slow peristalsis and improve absorption of fluid, electrolytes, and nutrients. Loperamide and diphenoxylate-atropine are typically the first-line choices for antimotility agents.

Small bowel bacterial overgrowth can occur in conjunction with SBS and is related to alterations of intestinal anatomy, motility, and gastric acid suppression. Intestinal bacterial overgrowth is treated with antibiotic therapy. However, when treating symptomatic intestinal bacterial overgrowth with antibiotics, drug rotation and inclusion of antibiotic-free intervals may decrease the potential for the development of resistant strains. If symptoms persist despite antibiotic and probiotic therapy, reducing dosages of motility and acid-suppressing drugs or switching to antidiarrheal agents may be helpful.

Transplant should only be used if the patient fails to achieve enteral adaptation after reconstructive surgery has been exhausted, develops irreversible liver failure, or is about to lose all central venous access.

A sample of our pathway for SBS patients is showed in Appendix 1.

Prof. Antonino Morabito direttore ADO Chirurgia Pediatrica Meyer - Specialista in Riabilitazione e Ricostruzione Intestinale; Dr. Riccardo Coletta Chirurgo Pediatrico Meyer – Specialista in Riabilitazione e Ricostruzione Intestinale.

Tuesday 18 September 2018