January 13, 2006

OEIS complex occurs in approximately 1 in 250,000 live births; it stands for Omphalocele, Exstrophy of the cloaca, Imperforate anus, and Spinal defect.

Ellie's omphalocele was present at birth and was corrected during her first surgery. Her umbilical cord contained her intestines and part of her liver. The doctors tied off and cut her cord above her herniated organs and used the cord like a tube of toothpaste to squeeze her liver and intestines back into her abdomen. They then tied off the cord and new skin grew over the defect. Ellie is still left with a large hernia in her abdomen that will need to be corrected in the future. (The hernia was partially corrected on April 20, 2009.)

Eleanor's cloacal exstrophy includes bladder exstrophy (bladder on the outside of the body and inside out), as well as kidney, reproductive and pelvic abnormalities. Not all cases of cloacal exstrophy include abnormalities in all of these areas; some are less extreme (just bladder exstrophy and pelvic abnormalities). Her bladder exstrophy was corrected during her fourth surgery. A vaginoplasty was also performed during her fourth surgery. (She was born with no external genitalia of any kind; we found out she was a girl during her first surgery, which took place on her third day of life.) Ellie's right kidney is about 1/3 longer than her left. It looks like they took a normal kidney and stretched it. It's function is not compromised by its odd shape, thank goodness! Her pelvis, instead of surrounding her abdominal organs, was splayed open, causing her legs to lay out to the sides. She looked like a little frog when she was lying on her back. During her second surgery, pelvic osteotomies were performed to correct her pelvis. She may need to have them done again, as her bone was soft during the first correction. Ellie will also need to have surgery done in the future to make her urinary tract continent. As it is now, she has no urinary control whatsoever.

When Eleanor was born, she had bowel coming up between the two halves of her bladder; this was how she eliminated solid waste. During her first surgery, the bowel was taken from between her bladder halves and a colostomy was created on her left side. Then the two halves of her bladder were sewn together and left on the outside of her body. She will probably have the colostomy for the rest of her life.

Eleanor's spinal defect is called a lipomyelomeningocele. It is a form of spina bifida that is covered over completely by skin. The defect is made up of fat cells which tether the spinal cord. If the fat is not removed and the cord not untethered, permanent loss of movement in the legs and possibly other extremities is almost certain. Ellie's neurosurgery to correct the defect was her tenth surgery. She is able to move her arms and legs pretty much like any other neurologically healthy child. She will still continue to visit the Spina Bifida Clinic at Riley on a regular basis.

So what does the future hold for our daughter? We are hopeful that by the time she is five, all of her big surgeries will be over. What a special little girl she is; we wouldn't trade her for the healthiest child in the world.