Gastrointestinal surgery in adult patients with cystic fibrosis

Abstract

Objective Gastrointestinal conditions requiring surgical intervention are becoming increasingly frequent in adults with cystic fibrosis (CF) as life expectancy increases. In addition, patients with CF are at risk of specific gastrointestinal complications associated with their disease. This includes distal intestinal obstruction syndrome (DIOS), which may affect up to 15% of patients, and can present diagnostic and therapeutic challenges. The aim of this study was to determine the nature and frequency of general surgical procedures undertaken in a large cohort of adult CF patients so as to guide future care. Design The medical records of all surviving adult CF patients followed at a large tertiary referral centre in the UK were scrutinised and details retrieved on those who had undergone abdominal surgery after the age of 16 years. Results A total of 377 patients with CF were identified from the prospectively held database. Thirty-three patients had undergone 43 abdominal operations. The median age at surgery was 22.7 years (range 16-58 years). The three most commonly performed operations were: surgery for DIOS (n=9); cholecystectomy (n=8) and fundoplication (n=6). A past history of surgically treated meconium ileus at birth was a significant risk factor for requiring surgery for DIOS as an adult. Conclusions The treatment of DIOS-related complications is one of the main reasons for abdominal surgery in the adult CF population. The general surgical community needs to be increasingly aware of the existence of diseaserelated gastrointestinal conditions in adult CF patients so that treatment can be optimised.

Abdominal Surgical Procedures in Adult Patients With Cystic Fibrosis: What Are the Risks?

“…A 2012 UK study reported on 377 patients in an effort to characterize outcomes for adult CF patients undergoing GI surgery. 12 Sharma et al identified DIOS, biliary disease, and gastroesophageal reflux as the most common indications for surgery. Of patients operated on for DIOS ( n = 11), there was no mortality, although 3 had prolonged hospitalization due to respiratory complications.…”

Microgallbladder: Self-Remitting Acute Cholecystitis-Like Condition Unique to Patients with Cystic Fibrosis

“…In noncirrhotic CFLD, the predominant clinical manifestations are at least one of the following: (1) elevation of AST, ALT, and GGT more than twice the upper limit of normal, (2) hepatic steatosis (liver parenchyma hyperechogenicity and poor penetration on US, decreased attenuation on CT, or signal dropout on chemical shift MRI sequences), (3) hepatic fibrosis (histologic diagnosis), or (4) cholangiopathy (beading and strictures of the biliary system on US, MRI, CT, or ERCP) [7, 8, 17, 21–23]. In advanced cases, CFLD can progress to focal biliary cirrhosis and multilobular diffuse cirrhosis (small-size liver, nodular hepatic contour, and coarse heterogeneous parenchyma) with or without portal hypertension (splenomegaly, abdominal varices, and ascites) [17, 18, 20, 24].…”

Distal intestinal obstruction syndrome: an important differential diagnosis for abdominal pain in patients with cystic fibrosis

“…Intussusception occurs in 1–2% of patients with CF and appears to occur more frequently in those with DIOS, with published rates of up to 25%. It is usually ileocolic and can subside spontaneously …”

“…Recurrence rates for DIOS range from 33% to 81% . The rates of DIOS and recurrence are increasing, likely related to increasing the lifespan of adults with CF …”