A rare Australian case of paediatric caecal volvulus.

Abstract

Caecal volvulus is an uncommon cause of intestinal obstruction in adults and is extremely rare in children.1 The true paediatric incidence remains undefined due to its rarity and limited literature reports. It has been described in children with chronic constipation, neurological and dysmotility disorders.2, 3 Identification of this serious surgical condition can be challenging, often requiring a high index of clinical suspicion to enable timely diagnosis and initiation of appropriate treatment to avoid associated morbidity and mortality. Moreover, clinical symptoms and radiology findings can be non-specific and at times the diagnosis is made intra-operatively.4 By presenting a paediatric caecal volvulus case study, we aim to raise awareness of this rare entity and describe pitfalls to avoid. A two-year-old, non-verbal boy presented to hospital febrile with a 36 h history of increased respiratory effort, abdominal distension, absolute constipation and non-bilious vomiting. The patient had a complex medical background including global developmental delay secondary to a chromosome 6 deletion abnormality and chronic neonatal lung disease for which he was on home oxygen. One week prior to this presentation he had been treated with oral antibiotics for a respiratory infection. Due to his developmental delay, he was difficult to assess. Clinically he appeared unwell, with audible grunting and subcostal recession. He was pyrexial to 38.2°C, tachycardic 170 beats/min and tachypnoeic 32 breaths/min with an oxygen requirement elevated from his baseline. His abdomen was distended with palpable loops, globally tender but not peritonitic with absent bowel sounds. Initially thought to have a viral respiratory infection however failing to respond to non-invasive ventilation, further investigations were organized. Laboratory results revealed leukocytosis (13.8 × 103/μL), neutrophila (8.0 × 103/μL), stable haemoglobin 100 g/L and C-reactive protein elevated to 107 mg/dL. He had normal liver and renal function with mild hypokalaemia. Chest radiograph was unremarkable but note was made of a dilated bowel loop in the inferior aspect of the film. A subsequent abdominal X-ray revealed a dilated large bowel loop occupying almost the entire abdominal cavity concerning for volvulus (Fig. 1). An urgent paediatric surgical referral review ensued and the patient was booked for immediate exploratory laparotomy. Intra-operative findings revealed a significantly distended, non-fixed, gangrenous caecal volvulus to 520° resulting in a closed loop with imminent perforation. The small bowel and remainder of large bowel was viable (Fig. 2). An extended right hemicolectomy with ileostomy and mucus fistula formation was performed given the extent of bowel damage as well as taking into consideration his co-morbidities and sepsis. The child recovered well post-operatively and was discharged day eight when fully enterally fed. Six months later he successfully underwent an elective stoma reversal. A review of the limited literature reveals that paediatric caecal volvulus has been associated with neurological conditions and neurodevelopmental delay. Whereas sigmoid volvulus is thought in part to be attributable to an overloaded heavy sigmoid loop, congenital incomplete dorsal mesenteric fixation of the caecum or ascending colon predisposes to caecal volvulus.2, 3 Although abdominal X-ray is commonly used as first-line imaging for obstructive symptoms in paediatrics, it can be difficult to differentiate the cause from possible differentials including volvulus (caecal or sigmoid) and congenital bands. Most common X-ray findings of caecal volvulus include focal colonic loop dilatation and absence of rectal gas.4 Other imaging modalities described in studies include contrast enema, computed topography and ultrasound.2-4 Caecal volvulus is a surgical emergency and although additional imaging modalities may be diagnostic and therapeutic, they may also delay operative treatment. A distal enema was considered in this case, but the decision was made to forego this and proceeded to emergency laparotomy based on our clinical findings of a septic child with an acute surgical abdomen and X-ray findings concerning for obstruction. It is not surprising then, in instances such as our experience, the diagnosis is made intra-operatively. There are varied management options for the treatment of paediatric caecal volvulus1 with documented interventions including colonoscopic decompression, caecopexy and bowel resection with either primary anastomosis or ileostomy.5, 6 Resective surgery carries the lowest risk of recurrence, offers definitive management and is the procedure of choice where there is concern for gangrenous or compromised bowel.2, 5, 7 Although rare, caecal volvulus is a cause of paediatric bowel obstruction and can be a challenging diagnosis to make. This case demonstrates the need for clinicians to consider abdominal pathology including caecal volvulus in children with intestinal obstruction or unexplained respiratory compromise. Informed consent was sought from patient's next of kin and all personal details are de-identified. Open access publishing facilitated by Bond University, as part of the Wiley - Bond University agreement via the Council of Australian University Librarians. Bharti Arora: Conceptualization; writing – original draft; writing – review and editing. Melissa Short: Supervision; writing – review and editing. Jennifer AhToy: Supervision.