The first signs of Marie's Romberg's is documented in a picture of her at about age 2. It shows up as a slight drooping of the left lower eyelid, plus a shininess of the skin on the left cheek. Later pictures, taken at about two year intervals, show progressive shrinkage of the cheek area, and more drooping of the eyelid. Most pictures taken after about age ten were taken with the left side turned away from the camera for cosmetic reasons, but some deterioration still shows up.
Marie's parents never tried to make her feel ashamed about it, there just was nothing that could be done about it. One doctor thought it might be some kind of burn, possibly radiation, and treated it with ultraviolet light and lotion, but this did not work.
Marie finally started investigating what was wrong in an attempt to appease her mother-in-law. She would see Marie about once a year and kept saying it looked like it was getting worse. The picture search was to prove her wrong, but worked the other way. Marie and her husband, Rex, and Marie's parents took these pictures with them to the University of Iowa Hospitals and Clinics in Iowa City, Iowa, and met wit Dr. John Frodel, chief of the Otolaryngology Dept. This was in August of 1989. It was then that she was finally diagnosed, although Dr. Frodel said he did not remember the exact name at the time. He had read about this deformity, but had never seen a case. All he knew of it was one paragraph in the medical books describing the symptoms.
Over the next two years, Marie's records traveled the country with University of Iowa's doctors to various medical conferences in search of more information on the treatment of this deformity. At this time, her left cheek was almost void of soft tissue, the left temple was concave about 1/4", the left cheek bone was about 1/3 of it's normal mass, and her upper and lower jaws did not mesh and were pulled to the left about 3/4" and tilted about 15 degrees. (We found out later that, with careful examination of the early pictures, the jaw misalignment was present from the start.)
Her treatment started in May, 1990 with the application of orthodontic braces to bring her teeth into proper alignment. This was necessary in order to be able to level and center her jaw later. This took about a year. In May, 1991, Dr. Frodel performed the first free flap operation. He took vascularized muscle tissue from her abdomen and inserted it into her cheek, connecting the blood vessels to existing ones in her neck. This seemed to work well for a while, but, as muscle will do when not exercised, it shrank much more than anticipated. It ended up a hard lump no bigger than a pea.
In the fall of 1991, other surgeries were performed to graft skull bone to her cheek, move her jaws, build up her lower eyelid to a more normal profile, and other minor procedures to keep the effects of scar tissue down. A word of caution: the skull bone graft revealed an unknown effect of the disease. While normally built of two layers, the skull bone on her left side was fused into one layer. When the bone segment was removed, both layers came out, exposing the duramatter around the brain. This had to be patched with healthy bone from the right side of her skull.
In August of 1996, the second free flap operation was performed. This time they took fatty tissue and its blood vessels from her left forearm. They said this was much more durable and effective tissue to use, but warned us that it would leave a large, ugly scar. We did not realize how bad it would be until after the surgery. It is quite bad and hard to hide. We know now that other areas can be used just as well and the scars are out of sight. The benefit is that this flap seems to be holding up well and shows good promise of a long lasting benefit. They over filled the cheek, to allow for some shrinkage, and would not trim it back until they were sure it was stable, over a year after insertion.
During that next year, Marie noticed that her upper jaw seemed to move. It got sore and acted like it was just hanging there. Upon examination, the doctors found out that the upper jaw had never fused after the operation. Also, two of the stainless steel plates used to hold it in place had broken. The joint would have to be refused and new plates would have to be inserted to hold it while it healed. This operation and the final trimming of the flap were done on Oct. 30, 1997. At the same time, an artificial material called Gortex, similar to Teflon, was inserted in her left temple to fill it out.
At this writing, Marie is doing well. She is on a no chew diet for two months, then very soft foods for another month. She is doing everything she can to be sure the jaw sets properly this time. So far all is going well. We are hoping for the best. The facial shape is much better, the constant pulling, which causes headaches, is much relieved. While the skin discoloration cannot be corrected, when she wants to, she can cover that with make up, which does a very good job of making her look very good.
We hope for the best from now on.
Feel free to leave a message for Marie at rombergs@hotmail.com