Plastic surgery, because of its scope of treatment possibilities from head to toe, undoubtably sees the greatest number of strange and weird disease conditions that require surgical care. In my Indianapolis plastic surgery practice over the years, I have come across a fair number of them. When recently asked by a patient as to what is the weirdest thing I have seen as a ‘plastic surgery doctor’, I stated that I could not cite just one. Instead, I thought I would commit the list to print.
This ‘old child’ disease is caused by a very rare genetic defect that causes one to age at a highly accelerated rate. It is much like the reverse of the Benjamin Button movie. Most of these patients will be dead by their early teen years and will look ancient. These children quickly become bald, thin, and develop early osteoporosis and heart disease. Their heads appear enlarged, which is more exaggerated by the need to wear glasses, compared to their small frail bodies. They are prone to teeth and jaw problems. A local dentist removed a tooth and unexpectedly fractured the lower jaw. Fixing of the fracture was needed with plates and screws.
Children born with this rare disease have cranial nerve defects, particularly that of the facial nerve. They have complete facial paralysis which does not permit them to have any facial expressions. No matter what they are feeling, their face remains frozen and expression free. They can not blink or close their eyes at night. Some facial expression can be restored through vascularized gracilis muscle flaps placed between the cheek and upper lip, plugging the muscle into the fifth cranial nerve for innervations. Eventually the patient learns to smile by clenching their teeth.
Patients with this very descriptive condition have their face covered in hair. The patient that I have treated had 2/3s of his face covered with a giant congential hairy nevus and he did look exactly like a wolfman. There was no transition between his scalp and his face with one confluent layer of thick dark hair. Through tissue expanders and full-thickness skin grafts, I was able to restore much of his face to non-hair bearing skin…albeit with the tradeoff of scars and a patchwork of quilted skin grafts.
In this very well known, albeit fairly rare, psychiatric disorder, the person creates actual medical problems in themselves to gain treatment and the attention and comfort from medical personnel. In many cases, these patients are highly knowledgeable about medicine and are able to produce symptoms that result in multiple unnecessary operations. I can speak to this problem in great detail as I have had the misfortune of treating two of them. The first one, with recurring oro-antral fistulas and non-healing face and jaw wounds underwent over thirty operations by me before I can to the realization that every surgical technique that I knew could not possibly fail in an otherwise healthy person with normal well-vascularized tissues. I was much wiser the second time around.
Poland’s syndrome or sequence is a rare birth defect of the trunk seen for breast reconstruction which is not hard to miss. Characterized by an underdevelopment or absence of the chest muscle on one side of the body, webbing across the armpit, a shorter arm and sometimes webbing of the fingers, the breast on that side is also small and may even be completely absent. As unusual as this developmental defect is, it is not that rare in the plastic surgery world. Breast reconstruction, usually using an implant, helps lessen the dramatic differences between the two sides. The upper arm may be slightly restricted when raising it high due to the web across the armpit. In the three cases that I have treated, web release was only needed in one.
Dr. Barry Eppley