Nathan Douthwaite · Prevention of Future Deaths report

On 23rd December 2010 I commenced an investigation into the death of Nathan Douthwaite aged 17 years. The investigation concluded at the end of the inquest on 25 February 2014.. The conclusion of the inquest was natural causes, the cause of death was
1a. Abdominal compartment syndrome, perforated caecum
1b. Hirschsprung’s disease.

Nathan was born in November 1993 at full term and passed meconium on the first day of life and continued to open his bowels regularly over several days in hospital after birth. In 1995 when Nathan was 21 months old there is mention in his medical records of him suffering from constipation (with blood in the stool). In 1999 Nathan was admitted to hospital for his first clear out of faeces. In 2000 was 6 ½ years old he began treatment with a consultant paediatrician who had particular interest in paediatric constipation and who was involved in his care until his death in December 2010. During this 10 year period Nathan was frequently seen at hospital suffering from severe bouts of constipation and by 2010 had been admitted to hospital on 13 occasions for clear out of faeces. His treating consultant in 2007 on his 10th admission for faeces described his abdomen as “grossly distended” and with “the most enormous faecal mass the size and shape of a rugby ball extending from pelvis to diaphragm with gross distention of the colon”. The clear out took the “best part of 3 weeks, with medication”. In 2007 Nathan was referred to a regional paediatric surgeon in Newcastle with a view to consider an antegrade enema continence procedure (ACE). This specialist considered a diagnosis of Hirschsprung’s Disease exceptionally unlikely. The specialist nurse did not believe that Nathan was likely to comply with post ACE procedures and he was not seen again by the surgical team. In 2010 it was noted that he lost 11.8 kilos (over 17% of his body weight. Nathan’s treating consultant had made a diagnosis of functional constipation. She had not thought that he was suffering from Hirschsprung’s disease.
Nathan was admitted to hospital at 04.20 hours on the 21st of December 2010 as an emergency and died at 07.15 hours. At autopsy there was presence of a massive megacolon with compression of the abdominal and thoracic organs consistent with abdominal compartment syndrome, together with a perforation of the caecum which was considered to be a very late event. The cause of death given was
1a. Abdominal compartment syndrome, perforated caecum
1b. Hirschsprung’s disease.
NICE guidelines with regard to Hirschsprung’s disease were introduced in 2010. No rectal biopsy was performed on Nathan as it was felt at the time (prior to the NICE guidelines being published) that he did not meet the criteria for Hirschsprung’s disease.
Even after the publication of NICE guidelines Nathan’s condition would not have met the criteria for a rectal biopsy. An independent consultant paediatric surgeon gave evidence that there was a possibility that there were more cases of undiagnosed Hirschsprung’s disease in older children than it was generally believed, that there was a nationwide survey being undertaken and that the NICE guidelines with regard to indications for rectal biopsy and investigations that should be performed before undergoing an ACE procedure should be reviewed, when dealing with children with severe constipation.

It is likely that if Nathan had undergone a rectal biopsy, Hirschsprung’s disease would have been diagnosed with the opportunity then being available for the appropriate treatment and thus I consider
(1) That NICE undertake a review of its guidelines in this regard
(2) That Count Durham and Darlington NHS Trust does review its own practices and procedures in avoidance of a NICE review and
(3) The Department of Health be aware of the circumstances of this case so that it can consider whether guidance should be issued in this regard pending the NICE review.