Colocolonic intussusception in an adult cystic fibrosis patient

Abstract

Purpose:

To raise awareness of colocolonic intussusception as a gastrointestinal complication of CF mimicking distal intestinal obstruction syndrome (DIOS) and discuss risk of recurrence.

Case summary:

A 33-year-old Caucasian male with cystic fibrosis presented with an acute abdomen diagnosed via imaging as colocolonic intussusception. He was managed with fluid replacement therapy and polyethylene glycol. He was re-admitted due to recurrence likely secondary to recurrent constipation and development of a fecalith. Surgery was contraindicated due to absence of tissue ischemia or necrosis.

Discussion:

Several possible etiological factors have been described, especially some that tend to occur within the context of CF disease, such as DIOS and PERT, and symptoms of colocolonic intussusception are similar to those of other causes of an acute abdomen but distinguishable by advanced imaging modalities. Due to risk of recurrence, an etiology of intussusception should be sought.

Conclusion:

Colo-colonic intussusception is a rare cause of an acute abdomen in the adult Cystic Fibrosis (CF) patient and may be associated with underlying constipation or presence of a fecalith.

1. Introduction

Gastrointestinal manifestations of cystic fibrosis result from the defective cystic fibrosis transmembrane conductance regulator (CFTR). Principally, manifestations arise due to lack of CFTR-dependent bicarbonate secretion through the epithelium and regulation of fluids in the intestinal lumen [1]. Of several possible complications arising from the intestinal defect, colocolonic intussusception is rarer but clinically relevant as a possible cause of an acute abdomen in patients with cystic fibrosis. We present a case report documenting the clinical course of a patient who presented with recurrent colocolonic intussusception secondary to an adherent fecalith.

2. Case report

A 33-year-old white male with past medical history of CF (F508del/2184insA) and pancreatic insufficiency presented to the hospital with a two-day history of dull non-radiating abdominal pain in the right upper quadrant, without history of abdominal surgery, gallstones, or prior DIOS. He reported 2 episodes of non-bloody, non-bilious emesis and denied diarrhea, constipation, fever, chills, night sweats, or weight loss.

Physical examination was significant for right upper quadrant abdominal tenderness with slightly reduced but active bowel sounds in all 4 quadrants. Laboratory evaluation was notable for an elevated WBC count of 13,600/mm³ with 89% neutrophils and elevated CRP level (29.9 mg/L). Total bilirubin was high at 1.6 mg/dL, while admission FEV₁ was 73% predicted (baseline 75% predicted).

An abdominal USS showed an approximate 5 cm of colocolonic intussusception at the hepatic flexure extending into the transverse colon, the mid-transverse portion of which is relatively narrowed with respect to the right colon and hepatic flexure. Computed tomography of the abdomen/pelvis with contrast corroborated ultrasound findings, revealing concomitant colocolonic intussusception at the hepatic flexure and a large amount of inspissated stool within the terminal ileum and ascending colon, with bowel thickening. The classic “target” or “doughnut sign” was seen on transverse/sagittal imaging, as well as the “pseudokidney” on longitudinal/coronal section (Fig. 1). There was no proximal small bowel obstruction.

Fig. 1.

Classic imaging findings of Colocolonic Intussusception. The “Doughnut Sign” on sagittal section (A) and “Pseudokidney” on coronal section (B). Ascending Colon, AC extended about 5 cm into the Transverse Colon, TC at the Hepatic Flexure, HF. Ascending Colon and the Terminal Ileum, TI can be seen filled with stool.

Conservative management with polyethylene glycol (PEG), fluid replacement therapy using normal saline intravenous infusion at 75 mL per hour and bowel rest was employed. A clear liquid diet regimen was commenced and advanced to full liquids, after plain abdominal x-ray investigation at four days of management revealed that the majority of stool in the left colon had evacuated. The patient’s pain resolved with increasing bowel movements following bowel irrigation. He was discharged after eight inpatient days on a regular diet and daily polyethylene glycol. The importance of increased adherence with pancreatic enzyme replacement therapy (PERT) and the need to maintain frequent bowel movements were emphasized. At one week following discharge, he had returned to normal bowel habits with full resolution of symptoms.

At seven weeks following discharge, the patient had recurrence, presenting to the CF clinic with symptoms of abdominal pain and intractable nausea after ingestion of possibly contaminated food. The patient admitted continued sub-optimal adherence with PERT and PEG. Symptoms also affected patient’s ability to comply with airway clearance regimen, with consequent drop in FEV₁ from about 75% predicted to 64% at admission. Since his case did not warrant surgery due to lack of tissue ischemia or necrosis, he was placed on a full liquid diet with aggressive overnight bowel regimen and managed conservatively for two weeks on PEG, simethicone, and ondansetron as needed. In addition to the osmotic agents, the patient also was treated with docusate, though this is not a standard agent for CF colonic complications. Prior to discharge, he had a colonoscopy demonstrating an adherent fecalith at the hepatic flexure which was not removable endoscopically despite colonoscopic decompression and resolution of pain symptoms. General surgery assessed the patient thereafter and elected for conservative management with plan for hemicolectomy if he has recurrence of the intussusception after continued bowel regimen and resumption of pancreatic enzymes.

3. Discussion

Intussusceptions occur rarely in adults, and a multicenter 6-patient case series of adult CF intussusceptions published by Nash et al., revealed no case of the colocolonic variety [2], while a systematic review showed 4 out of 791 documented cases of adult intussusception occurred in adult CF patients, representing a 0.5% prevalence. The ileocolic type of intussusception accounts for 80–95% of pediatric intussusceptions and can resolve spontaneously, whereas the colocolonic variety remains rare [3]. Thus, the propensity for spontaneous resolution cannot be estimated.

Pathological lead points resulting from inspissated secretions, epithelial damage, enlarged lymphoid follicles, distension of the appendix and distal intestinal obstruction syndrome (DIOS) [3,4] have been identified as predisposing factors for intussusception due to severe constipation. Modern PERT formulations have not been associated with constipation. We report a unique case of colocolonic intussusception induced by inspissated stool in an adult CF patient.

Colocolonic intussusception can easily be diagnosed with sonography or computed tomography of the abdomen/pelvis. These imaging modalities will help to distinguish this rare complication from the more common CF gastrointestinal complications which include: DIOS, fibrosing colonopathy, appendicitis, and intestinal malignancy [5]. In this case, while USS saw the colocolonic intussusception, a CT and endoscopic evaluation were ultimately needed to confirm the diagnosis, rule out proximal small bowel obstruction, and establish the underlying cause of the pathologic lead point.

In general, adult intussusceptions routinely require a surgical approach to reduction [1], however when associated with CF, surgical intervention is often not necessary and recurrence may be associated with underlying constipation as shown in this case study.

Here we present a case of a colocolonic intussusception, a rare abdominal complication of adult CF that was recurrent and caused by a leading edge and highly adherent fecalith. Unfortunately, removal of the fecalith was impossible with endoscopic techniques alone. Further studies on management approaches would enable more understanding of the treatment needs and approach to management in the adult CF population. Adult CF physicians should consider colocolonic intussusception as a rare cause of abdominal pain in CF patients when considering alternative diagnoses to DIOS, while being mindful of a potential for recurrence. Involvement of a multidisciplinary team including gastroenterologists and gastrointestinal surgeons is necessary to determine the best plan of care.