A 16-year-old Caucasian boy was admitted as an emergency case with a history of abdominal distension, nausea, vomiting and constipation over the previous 3 days. From his medical history he had had constipation since he was a toddler, with faecal overflow incontinence. He also had bronchial asthma.
He was referred to paediatricians when he was 6 years old and subsequently to a colorectal surgeon. An examination under anaesthetic with sigmoidoscopy, manual evacuation of faeces and a rectal mucosal biopsy was performed at that time. The histology showed a mild chronic inflammatory infiltrate.
He was one of three siblings, the others being 23 and 19 years of age and in good health. Regular medications included movicol (polyethylene glycol 3350, sodium bicarbonate, sodium chloride and potassium chloride), senna and sodium docusate.
On clinical examination, he was cachectic, emaciated and dehydrated. His pulse rate (PR) was 129 beats/min. Blood pressure was 153/107 mm Hg. His respiratory rate was 22 breaths/min. His oxygen saturation (SaO2) was 95% on room air. He was drowsy at the time of presentation.
The abdomen was grossly distended with palpable faeces. Digital rectal examination revealed hard stools in the rectum with overflow of liquid faeces. The patient was resuscitated with intravenous fluids and catheterised. Phosphate enemas were given with no benefit. A decision was taken to perform a manual evacuation of faeces under a general anaesthetic. At 2 h after his admission, he became cardiovascularly compromised with SaO2 of 74%, PR of 158 beats/min and blood pressure of 160/105 mm Hg. Arterial blood gases showed a pH of 6.9, base excess of 13.1 mEq/litre, a lactate level of 8.0 μmol/litre, pO¼sub>2 11.51 kPa and pCO2 12.41 kPa.
He was taken urgently to the operating theatre for manual evacuation of faeces, to decompress the abdomen and relieve the pressure on his airways and mediastinum. After awake intubation in the sitting position, 3.8 kg of semisolid faeces were evacuated.
This resulted in some improvement of his airway pressures and improved oxygenation. He was ventilated overnight in the Intensive Therapy Unit (ITU). His vital signs improved.
The following morning he was feverish and a CT scan of the abdomen revealed free air in the peritoneal cavity. The patient was therefore prepared for a laparotomy. At surgery, there was huge megacolon and megarectum with impending caecal and transverse colonic perforations. There was turbid fluid in the peritoneal cavity. He underwent a subtotal colectomy and end ileostomy with a mucous fistula.