Hirschsprung's disease, named after Harold Hirschsprung who first described the disorder in 1888, is a congenital blockage of the large intestine caused by missing nerves in a portion of the bowel. Without these nerves, the muscles cannot push material through, and the end result is a big belly. A really big belly. A really, really big belly.

The signs of Hirschsprung's Disease occur very early in newborns and infants, as they are observed to have a failure to pass meconium after birth.

(INTERJECTION: Oh my Gawd, you guys - did you know about meconium? It's so gross. It's the first poop a baby passes and it "consists of composed of materials ingested during the time the infant spends in the uterus: intestinal epithelial cells, lanugo, mucus, amniotic fluid, bile, and water. Meconium, unlike later feces, is viscous and sticky like tar, its color usually being a very dark olive green; it is almost odorless. When diluted in amniotic fluid, it may appear in various shades of green, brown, or yellow. It should be completely passed by the end of the first few days after birth, with the stools progressing toward yellow (digested milk)." Look, here's a picture of it. Ugh... that's so disgusting! I can't even!)

Okay... Deep breaths... sniffing salts... I'm okay...

Back to Hirschsprung's Disease - so, yeah, the baby has trouble pooping and when he does (Hirschsprung's is five times more frequent in boys), the stools are explosive or watery. Sometimes in more mild cases, the symptoms may not be noticed until the child is older, constipation gets worse, and the child develops a swollen belly. The disease also causes malnutrition and the child's growth is slowed.

The treatment is to surgically remove the defective portion of the colon. Most commonly, the rectum and abnormal part of the colon are removed. The healthy part of the colon is then pulled down and attached to the anus. The surgery is often done in two stages: a colonostomy in the first stage, and the reattachment of the healthy colon in a later stage. Generally, in first world countries, this issue is caught and treated early and little lasting damage is done to the baby.

Hirschsprung's Belly in an older child.

X-ray showing the grossly distended colon.

The distended colon during surgery.

Consider, however, the fate of those unfortunate enough to be cursed with both Hirschsprung's Disease and the misfortune of living in a third world country? The website for Praanadah Specialty Hospital in India displays in graphic detail several extreme cases of Hirschsprung's in older children. The suffering these poor kids must have experienced, I can't even imagine.

Six-year-old Indian boy.

The boy's megacolon. Can you imagine? I go a couple days without pooping and I'm in pain. Yikes...

Surgery on the six-year-old.

Look at the after picture. What a relief he must feel!

This kid from a very poor family is a REALLY awful case. I'm not sure his age, but it says that he spent 13 years with this horrifying prolapsed colostomy.

I'm pretty sure that's not supposed to be on the OUTSIDE!

The dreadful adhesions around his colon. The doctors were able to correct it all in one go though!

Closing up the abdomen.

And again - the relief that this kid must have felt. Unimaginable.

There are more pictures and stories at the Praanadeh Specialty Hospital website.

Consider, however, the even WORSE fate of those who suffered from this disorder many years ago, before surgical treatments were available. The most famous of these is the sideshow freak known as "The Human Balloon". His colon grew to be 9 feet in length and contain 47 lbs of impacted excrement. He died at the age of 29 and his colon ended up in the magnificent Philadelphia Mütter Museum. He must have suffered tremendously throughout his life... but then again, how many of us can say that our colon will end up memorialized as a stuffed toy?


And after.

Death Threats...