Proximal small bowel obstruction in a patient with cystic fibrosis: a case report

Abstract

Background: As advancements are made in the management of cystic fibrosis (CF), survival of the CF patient into adulthood has increased, leading to the discovery of previously unknown CF complications. Though gastrointestinal complications of CF, such as distal intestinal obstruction syndrome, are common, this case demonstrates a variant presentation of small bowel obstruction in this population.

Case presentation: We describe a 42-year-old male with CF who presented with 2 days of worsening upper abdominal pain, emesis, and loss of bowel function. The patient had no history of any prior abdominal surgeries; however, imaging was concerning for high-grade mechanical small bowel obstruction possibly related to internal hernia. Given leukocytosis and diffusely tender abdomen found on further workup, the decision was made to proceed with diagnostic laparoscopy after a brief period of intravenous fluid resuscitation. Intraoperatively, the transition point was found in the mid-jejunum and was noted to be due to kinking of the bowel causing vascular congestion in the proximal portion. Surgical manipulation of the bowel was required for return of normal perfusion and patency.

Conclusion: Though the exact mechanism cannot be definitively delineated, we speculate that the increased viscosity and prolonged intestinal transit time, characteristic of CF, resulted in inspissated fecal content in the proximal small bowel, which then acted as a lead point for obstruction. Thus, though small bowel obstruction in patients with CF is often attributed to distal intestinal obstruction syndrome, a broader differential must be considered. Early surgical intervention may be necessary to prevent bowel ischemia and subsequent small bowel resection in a patient presenting with concerning clinical and image findings, as was seen in this patient.

Keywords: Abdominal pain; Cystic fibrosis; Distal intestinal obstruction syndrome; Small bowel obstruction; Surgical management.

Fig. 1

CT abdomen/pelvis with contrast. The CT scan of this CF patient demonstrated dilated loops of bowel proximally (a), with associated mesenteric venous congestion (b). Additionally, a transition point (c) was noted in the left hemiabdomen, distal to which the small bowel was decompressed (d). Just proximal to the transition point, gas bubbles and particulate matter were noted, suggestive of fecal contents in the small bowel

Fig. 2

Dilated loops of bowel. Intraoperatively, hyperemia and dilation of the proximal small bowel was noted from the distal duodenum to the mid-jejunum. Hypervascularity of the bowel wall is suggestive of vascular congestion secondary to increased luminal pressures exceeding venous pressures. There was no evidence of ischemia, necrosis, or perforation

Fig. 3

Transition point with decompressed loops of bowel distally. At the level of the mid-jejunum, kinking of the small bowel was noted, without involvement of the mesentery or evidence of adhesions. Immediately distal to this point, the bowel was entirely decompressed, without evidence of poor perfusion

Fig. 4

The small bowel feces sign. On further inspection, the patient’s CT scan was determined to have a characteristic small bowel feces sign in the mid-jejunum, just proximal to the suspected transition point. Defined in 1995 as the presence of gas and particulate material in a dilated segment of the small bowel, the small bowel feces sign indicates fecal content in the small bowel and is due to intraluminal stagnation of enteric material [11].