MICI: approccio chirurgico UOC di Chirurgia Pediatrica Università degli Studi di Napoli Federico II Dir. Prof. Alessandro Settimi MICI: approccio chirurgico Ciro Esposito Alessandro Settimi Napoli 2003

DIAGNOSIS A minority (25%) of children with CD present with the classic triad of abdominal pain, diarrhoea and weight loss The development of standardised diagnostic criteria (the Porto criteria) by the IBD working group of the ESPGHAN has done much to ensure uniformity in diagnosis and management

DIAGNOSIS The gold standard in diagnosis is combined upper and lower gastrointestinal endoscopy, together with small bowel radiology UC CD

DIAGNOSIS Increased experience with MR enterography, the lack of exposure to radiation, and the utility of MRI in the evaluation of perianal CD all favour this study over contrast meal Capsule endoscopy is showing promise as an adjunct in cases of diagnostic difficulty, but carries the risk of obstruction if the capsule becomes impacted at an occult area of narrowing

SURGICAL MANAGEMENT OF ULCERATIVE COLITIS The objective in both elective and emergency surgical interventions for UC is the removal of the colon The most common indication for colectomy is: Failure of medical therapy whether due to frequent disease activation with short periods of remission Unacceptability of the side-effects of medical therapy Inadequate disease control may also be reflected by: Reduced growth velocity Delayed puberty, inadequate nutrition, poor bone mineralisation Loss of time from school Colonic cancer per se does not feature in the indications for colectomy in childhood since the quoted risk is 2% at 10 years, 8% at 20 years and 18% at 30 years Eaden JA et al. Gut 2001

SURGICAL MANAGEMENT OF ULCERATIVE COLITIS Colectomy rates at 5 years from diagnosis range between 14% and 24% in children Gower-Rousseau C et al. Am J Gastroenterol 2009 The Ileal Pouch Anal Anastomosis (IPAA) is now the gold standard for UC surgery The most conservative approach for elective surgery is a 3-stage procedure: Total colectomy with end ileostomy Construction of IPAA with completion of proctectomy and temporary diverting loop ileostomy Ileostomy closure In a 2-stage approach, the first stage is: Total proctocolectomy (TPC) with IPAA and temporary diverting loop ileostomy 2. Ileostomy closure

SURGICAL MANAGEMENT OF UC Single-stage IPAA may be performed in only very carefully selected cases: patients who are not on immunomodulator or biologic therapy on minimal to no steroids are not obese and otherwise with favorable body habitus are without other nutritional derangements or anemia Clearly single-stage surgery is feasible but is associated with a higher risk of major complications such as anastomotic dehiscence, sepsis and late pouch failure (Brown et al, Z Gastroenterol , 2012 ) Advances such as the double-stapled IPAA have been incorporated into paediatric practice with considerable reduction in operating time when compared with traditional hand-sewn IPAA (Annals of Surgery, 2006 )

+ ILEOSTOMY

IPAA offers a definitive cure for the colonic manifestations of UC without need for permanent ileostomy in the majority, with pouch survival of > 90% at 20 years (Baillie et al. 2015) However, IPAA is associated with a high surgical complication rate, mainly related to the pouch: Alexander F et al. (J Pediatr Surg, 2009 ) reported that one fifth of patients will have at least one complication in the first month after surgery: Pouchitis (48%) Chronic refractory pouchitis (7%) Pouch failure (9%) In another pediatric series (Koivusalo et al, J Pediatr Surg 2013) the complication rate was as high as 21/37 (57%): Pouchitis (62%) Intestinal obstruction (48%) Recurrent pouchitis (35%) Pelvic abscess/sepsis (10,8%) Pouch prolapse (2,7%) Wound complications (16,2%) There is very little experience in the paediatric surgical literature of revision pouch surgery which is a further strong argument for close links with high case load adult specialist units Smith NP, J Pediatr Surg 2007

OUTCOME OF SURGERY FOR UC QoL after surgery was improved or maintained and generally stable over time, consistent with short-term results in children and long-term results in adults Polites et al. J Pediatr Surg 2015

SURGICAL MANAGEMENT OF ULCERATIVE COLITIS The option of Ileorectal Anastomosis (IRA) for UC is rarely considered in childhood Potential advantages include: Reduced stool frequency with improved faecal continence Improved fertility in females Reduced likelihood of impaired sexual functioning due to nerve injury during the pelvic dissection This has to be balanced with the: Need for regular endoscopic surveillance Potential for failure of medical control of the residual disease in the rectum The ultimate failure rate of IRA for UC is as high as 57% but this does not argue against the procedure in females if time is gained for pregnancy before later restorative proctectomy Waljee K, Am J Gastrenterol 2011

ACUTE SEVERE COLITIS Early introduction of salvage medical therapies World J Gastroenterol 2012 Early introduction of salvage medical therapies (cyclosporine, tacrolimus and infliximab) has reduced the emergency colectomy rate in ASC from 30%-70% to the current 10%- 20% with concomitant reduction in mortality Upper limits of normal colonic width in children with ASC should take age into consideration: 4 cm < 11 years 6 cm in older children Absolute indicators for surgery in the setting of unresponsive ASC include perforation and significant haemorrhage The surgical procedure of choice is colectomy and end ileostomy preserving the rectal stump

A- Hand-assisted Laparoscopic Surgery (HALS) Laparoscopic approaches have therefore increased in prevalence in the population of patients with IBD: A- Hand-assisted Laparoscopic Surgery (HALS) B- Laparoscopic-assisted Surgery C- Laparoscopic «incisionless» surgery

ROLE OF MINIMALLY INVASIVE SURGERY ADVANTAGES - Small incisions with less pain and improved cosmesis - More rapid return to full functional status like work and school - Adhesion prevention to reduce the risk of adhesive small bowel obstruction CONTRAINDICATIONS TO MIS TECHNIQUES IN PATIENTS WITH IBD

CD

Site involvement in paediatric CD Terminal ileum and colon (40-60%) Colonic alone (10-40%) Proximal small bowel (8-15%) Entire digestive tract (5-15%)

INDICATIONS FOR ELECTIVE SURGERY Intestinal subocclusion Internal fistulas Abdominal abscess or mass Growth failure Unsuccessful medical treatment

INDICATIONS FOR EMERGENCY SURGERY Free perforation Massive bleeding Toxic megacolon Bowel obstruction

SURGICAL OPTIONS Intestinal resection and primary anastomosis Strictureplasty Intestinal diversion

SURGICAL MANAGEMENT OF CD The life time risk of surgery for CD is approximately 80% (Caprilli et al, Gut 2009) Indications for surgical management of children with CD include: Failure of medical therapy Associated extra-intestinal manifestations (especially eye and joint pathology) Complications of the disease (fistula, obstruction, perforation, abscess formation, and bleeding) Timely surgical intervention has been demonstrated to improve height velocities in patients refractory to medical therapy (Singh Ranger G, Pediatr Surg Int 2006) Requirement for surgical resection ranges from 20%-29% at 3 years, and 34%-50% at 5 years from diagnosis in paediatric practice World J Gastroenterol 2014

STRICTUREPLASTY Repeated resectional surgery in CD is clearly associated with a risk of short bowel syndrome Di Abriola GF et al demonstrated that strictureplasty is a good and effective surgical option for sparing bowel length in CD patients with extensive intestinal strictures (J Pediatr Surg 2011)

RESECTIONAL SURGERY FOR CD In accordance with one of the five golden rules of Alexander-Williams and Haynes: «resect only symptomatic macroscopic disease» Children with ileocolic disease (with colonic involvement proximal to the mid-transverse colon) are readily managed by right hemicolectomy and primary anastomosis with low associated morbidity The phenotype associated with left sided colitis in childhood has been shown to relapse early following segmental resection In this case a subtotal colectomy with end ileostomy is favoured in children

MIS APPROACH IN CD OPEN MIS The small bowel in patients with full-thickness fibrostenotic/inflammatory disease can typically be run during laparoscopy without difficulty, often in high-definition on large monitors (Holder-Murray et al . , Inflamm Bowel Dis 2015) Primary uncomplicated ileocolic or small bowel CD should not undergo open surgery unless deemed to have an absolute contraindication to MIS MIS has also to be proven safe for recurrent or fistulizing ileocolic disease

SPECIAL SITES - PERIANAL CROHN’S DISEASE (PACD) Effective management of PACD follow 3 principles: Prompt drainage of any septic focus Conservative surgery Appropriate medical therapy Most surgeons favor the use of non-cutting setons to control septic complications from fistulas Setons are well-tolerated and after drainage of pus represent the principal adjunctive surgical contribution to medical management The final application of MIS to patients with IBD is the palliation of symptoms due to PACD More complex fistulating disease involving the vagina or urinary tract is rarely seen in paediatric practice For these patients, diversion of fecal stream may allow more optimal conditions for healing

PRE-OPERATIVE OPTIMISATION Risk factors associated with post-operative sepsis include: Poor nutritional status (albumin < 30 g/L) Presence of abscess or fistula Preoperative steroids Recurrent clinical exacerbations of CD To date there is little strong evidence supporting delaying surgery to allow for a period of pre-operative hyperalimentation by either enteral or parenteral routes However medical management of sepsis and percutaneous drainage of intra-abdominal abscesses may reduce post-operative septic complications Clin Colon Rectal Surg 2007

NEW DEVELOPMENTS IN SURGERY IN CHILDREN WITH IBD Single –incision Laparoscopic Surgery (SILS) The SILS approach uses only 1 incision through which an operating platform allows several working instruments in addition to the laparoscopic camera It offers improved cosmesis by eliminating several 5- to 12-mm incisions in the abdominal wall as in multiport laparoscopy The SILS approach has already been used successfully for colectomy in UC (Fichera et al, 2011) Additional long-term studies are needed to clarify the role of this approach in the paediatric population

TRANSITIONAL CARE The separation of adult and paediatric IBD practice may disadvantage children, delaying adaption of innovative treatments and timely transition Consensus Guidelines , issued jointly by the European Crohn’s and Colitis Organization and the ESPGHAN, state that every adolescent should be included in a transitional care programme which is adapted to fit local paediatric and adult health care models In addition, within the paediatric setting, adolescents should be encouraged to take increasing responsibility for their treatment and visit the clinic at least once without their parents

Int J Colorectal Dis. 2015 Adequate management of IBD requires a multidisciplinary team including gastroenterologist, pharmacist, pediatric surgeon, psychiatrics and nutrition therapist; all should be involved for good decision making in that disease spectrum Key factors for successful outcome are careful patient selection after evaluating the balance between the risk of the disease and the expected surgical complications

CONCLUSION -CD CD is a medical disease. The role of surgery: DOES NOT cure or prevent recurrences BUT IT CAN BE a life-saving procedure AND IT CAN HAVE a dramatic effect on improving the patient’s quality of life

Conclusions UC Surgery is curative: Total colectomy Type: J pouch + ileostomia High incidence of redo surgery Side effects IBD Team Transitional care Long term follow-up