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Hirschsprung’s disease: Why 22 year Old Man who Passed 12.7kg of Faeces Was Constipated from Birth

A 22 year old Chinese man was finally relieved of faeces that had accumulated over 20 years. The man had been constipated throughout his life according to the report. His constipation was never fully relieved by the use of laxatives or other medications till surgeons intervened by opening up the abdomen to remove the poop and extremely large colon. The 3-hour operation was successful. According to the doctors, the man was ‘’nine months pregnant’’ and looked like he ‘’could explode at any time.’’ Thankfully, he is expected to recover from this painful, distressing and embarrassing condition.

Why did he become severely constipated?

He had a condition called Hirschsprung’s disease (HIRSH-sproongz). This is present at the time the child is born. Within 24 to 48 hours of birth, a child is expected to pass his or her first poop. That is why parents become worried if the child does not pass any stool in the first few days. However, in Hirschsprung’s disease, nerve cells that instruct the bowels to move faeces down from the large intestines to the rectum are absent. In rare cases, this may include the stomach.

So, the flow or systematic passage of poop is disrupted at the point where there are no nerves to generate involuntary movement. This results in accumulation over time. This may harden, completely blocking the passage of stools.

Unfortunately, for the young man, the accumulation continued for years leading to the ‘birth’ of faeces weighing 12.7kg.  The expanded colon called ‘mega colon’ is one of the complications of Hirschsprung’s disease. Some of the other complications include:

  • Perforation
  • Bleeding
  • Ulcers
  • Infection of the intestines (enterocolitis) which is life threatening

In view of these complications, there may be a need to surgically remove the segment of the bowel with missing nerves and reduce the length of the colon too.

When Should a Baby pass the first Poop?

The baby is expected to pass the first stool known as ‘meconium’ within 24 to 48 hours after birth. Meconium consists of what the baby ingested while in the womb. It includes mucus and amniotic fluid. The arrival of meconium is a good sign of a functioning digestive system.

A parent should become worried if a full term baby fails to pass meconium in 48 hours. There may be delay in premature babies 1 out of 3 fails to pass stool within 48 hours. However, almost all premature babies should have passed stool by the ninth day.

It is important to note that the delay in passage of  meconium may be missed at birth and in some cases may not be the presenting symptoms. Furthermore, 10% of babies may pass meconium normally and still have Hirschsprung’s disease.

According to experts, suspect intestinal blockage if the delay in passing stool is accompanied by the following:

  • Abdominal swelling
  • Refusal to feed
  • Vomiting

Hirschsprung’s disease accounts for 1 out every 4 cases of large intestinal obstruction in the new-born. It is four times likely to occur in boys than girls. Do not hesitate to seek medical help if you notice any of these signs of intestinal obstruction. In Nigeria and other developing countries, it has been reported that cases of Hirschsprung’s disease reported late, usually with complete intestinal blockage. For instance, in an 8 year review of cases in University of llorin Teaching Hospital, Nigeria, most babies presented at about 2 years of age. Late presentation is often linked with mortality.

The doctor may need to do x-rays, barium enema and tissue biopsy to confirm the diagnosis because there are other causes of intestinal obstruction in children. Surgery is the definitive treatment. Therefore, centres that have paediatric surgeons are best suited for the management of this condition.

Medically reviewed by Dr Adedayo A.A. MBBS, FWACS

Read more: She’s Huge! Mother exclaims after giving birth to Baby weighing 6.1kg

 

 

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