Transverse colon volvulus in a patient with sickle cell disease

Abstract

Although colonic volvulus is a relatively rare cause of large bowel obstruction, accounting for up to 5% of all cases of intestinal obstruction, transverse colon volvulus is extremely uncommon compared with volvulus of the sigmoid colon or caecum and is responsible for only 3% of all reported cases. We report an unusual case of spontaneous volvulus of the transverse colon in a young man with sickle cell disease who underwent resection with primary anastamosis. Having a high index of suspicion and early operative intervention allowed for this patient to have an uneventful postoperative course.

Background

Volvulus of the transverse colon constitutes a rare surgical emergency. It was first described in 1932 by the Finnish surgeon Kallio.¹ Its clinical presentation and radiological findings are that of large bowel obstruction. Prompt surgical intervention is key to reducing the morbidity and mortality associated with transverse colon volvulus. To date, <100 cases of transverse colon volvulus have been reported in the literature.² To the best of our knowledge, this is the first reported case in a patient with sickle cell disease.

Case presentation

An 18-year-old Bahraini male, known to have sickle cell disease, was admitted for sudden onset vaso-occlusive pain crisis. After seeking a surgical consultation for abdominal pain, the patient was reviewed in the ward. He reported a 2-day history of severe abdominal pain and constipation with vomiting. His medical history was significant for sickle cell painful crises treated with opioid analgesics. There was no surgical history. Physical examination showed a distended abdomen; there was generalised tenderness associated with voluntary guarding, but no rigidity or rebound tenderness. The abdomen was tympanic to percussion with absent bowel sounds. Digital rectal examination demonstrated an empty rectum.

Investigations

Laboratory investigations revealed anaemia (haemoglobin 7.9 g/dL), leukocytosis (white cell count of 16.6×10⁹/L) with neutrophilia (66.9%) and liver function tests showed picture of sickle cell hepatopathy. The abdominal X-ray suggested bowel obstruction, with multiple air–fluid levels and distended small bowel and ‘U-shaped’ large bowel loop in the left hemidiaphragm (figure 1). An urgent CT scan reported a dilated, stool-filled transverse colon as well as dilated proximal small bowel, raising possibility of large bowel obstruction (figure 2).

Figure 1.

Abdominal X-ray showing air–fluid levels, dilated small bowel loops and ‘U-shaped’ loop in the left hemidiaphragm.

Figure 2.

CT scan showing dilated small bowel and transverse colon filled with faecal matter.

Treatment

The patient was transfused two units of packed red blood cells and was taken to the operating room for exploratory laparotomy. Intraoperative findings were dilated small bowel and redundant transverse colon that was rotated 360° clockwise on its mesentery, with no evidence of bowel ischaemia (figure 3). The volvulus was delivered into the incision and detorsed. Segmental resection of the transverse colon with primary anastomosis was performed.

Figure 3.

Gross specimen showing dilated small bowel and transverse colon rotated 360^o on its mesentery.

Outcome and follow-up

The postoperative course was uneventful, and the patient was discharged from the hospital after 6 days. Histologically, there was no evidence of any ischaemic changes with viable resection margins.

Discussion

Volvulus is a rare cause of colonic obstruction, accounting for 3%–5% of all cases of intestinal obstruction.³ Transverse colon volvulus is extremely uncommon, representing ~3% of all cases of colonic volvulus.⁴ Risk factors for the development of transverse colon volvulus may be divided into anatomical, mechanical and physiological.^{3 5} The two anatomical factors are redundancy and non-fixation,² as in our case. Mechanical causes include previous volvulus, distal colonic obstruction, adhesions, malposition of the colon during previous surgery, mobility of the right colon, inflammatory strictures and carcinoma.³ Physiological factors include large bowel distension secondary to distal impediment to defecation in chronic constipation.^{2 5} Other causes have been reported, such as Chilaiditi’s syndrome,⁶ Clostridium difficile associated pseudomembranous colitis⁷ and impaired intestinal motility in pregnancy.⁸ In our case, constipation from use of chronic opioids for controlling symptoms of sickle cell disease might have precipitated the condition. This complicates the diagnosis given his ongoing abdominal pain. Additionally, his young age and gender are also complicating factors as these patients are often female with a mean age of 51 years.⁴

It can present either as acute fulminating or subacute progressive transverse colon volvulus.^{3 6} The former is characterised by sudden onset of severe abdominal pain associated with rebound tenderness, nausea, vomiting, limited abdominal distension and marked leukocytosis.² Bowel sounds are initially hyperactive but later may become absent.⁶ Our patient presented with the acute fulminating type, despite absence of typical peritoneal signs on physical examination, which were possibly masked by his long-term use of opioids as well as inpatient administration of opioid analgesics at the time of examination. In the latter, there is massive abdominal distension in the setting of mild abdominal pain without rebound tenderness, nausea or vomiting, and the leucocyte count is either normal or only mildly elevated.³ The progressive onset of symptoms in the subacute progressive type can delay the diagnosis and result in failure to provide timely intervention, causing disease progression to the acute fulminating type with bowel infarction, peritonitis and death.^{2 3}

Diagnosis of transverse colon volvulus is usually not made preoperatively, as radiological findings are not characteristic, unlike in sigmoid colon volvulus, and is made intraoperatively.⁹ In our case, dilated bowel loops with air–fluid levels and the presence of U-shaped loop in the left upper quadrant on abdominal X-ray raised the possibility of diagnosis, but this is not a consistent radiological finding.⁵ There are no typical radiological features for transverse colon volvulus on CT scan.¹⁰ The classic ‘bird’s beak’ deformity in the transverse colon seen on contrast enema is diagnostic, but in the acute setting, surgery should not be delayed to perform contrast study.⁶

A colonoscopic decompression is the initial treatment of sigmoid colon volvulus, it is not recommended for transverse colon volvulus, due to high probability of failure and necrosis.^{9 11} Resection with primary anastomosis, as in our case, or with stoma formation is the treatment of choice in cases of transverse colon volvulus to prevent recurrence.^{3 6} Compared with resection, simple detorsion of the bowel or untwisting with colopexy is associated with higher rate of recurrence or even death.^{2 4} At operation, there were no signs of bowel ischaemia, attributable to the lack of ischaemia at early stages, especially if immediate surgical intervention is undertaken.^{2 3}

Learning points.

• Volvulus of the transverse colon is an extremely rare entity and should be recognised as a surgical emergency.

• Diagnosis requires a high index of suspicion in a patient who presents with abdominal pain and features of bowel obstruction. It is difficult to identify radiologically, so preoperative diagnosis becomes even more difficult to make with certainty.

• Delay in diagnosis can result in bowel strangulation with perforation, peritonitis and sepsis with increased morbidity and mortality.

• Prompt recognition with emergency surgical intervention, through resection with or without primary anastomosis, is necessary to minimise these complications and achieve a successful outcome.

• An additional learning point from this case would be that in a sickle cell disease patient presenting with abdominal pain, one should also consider surgical causes of acute abdomen other than sickle cell crisis as a cause of pain.