Monostotic fibrodysplasia
I didn’t often refer patients to Addis. You will probably have heard of Catherine Hamlin and the ‘Hospital by the river’. They had a vast experience with post-delivery fistulae and paid for the patients’ stay and charged nothing for their treatment, so I referred those cases. I had worked in Addis and knew hospital attitudes, capabilities and standards so I didn’t refer much else. In addition usually if I tried to refer them, patients wouldn’t go. It was too expensive and way outside their experience.
On one occasion however I saw a lady with a huge intra-oral mass, so that on her left side of the mouth there was a mere crevice to place liquid food into. I tried to refer her.
She refused to go. It was obvious that if nothing was done she’d soon die. We had poor X-ray facilities. I was fortunate to have with me, if only for a few weeks, a visiting Australian anaesthetic trainee. I believed it was a case of maxillary monostotic fibrous dysplasia. We set up for a tracheotomy in case it became necessary, but planned first to do an awake blind intubation under local anaesthetic. After appropriate anti-anxiety medication, local anaesthetic sprayed up her nose and through the slit in her mouth and then injecting LA into the trachea, we were able to intubate her blindly; we listened for her breathing coming up the tube before gently pushing the endotracheal tube into the trachea. She was then put to sleep. I was able to keep her orbital margin intact and her vision was not impaired. I was able to preserve enough skin from her palate to primarily close the defect after the tumour wa removed. Because of absence the of her maxilla, she had a very sunken hemi-face on the right. Maybe you would consider plastic surgery to get a more cosmetic result, but she was able to eat normally and was very happy with the result. In the immediate postoperative photo you can see how her tongue had been displaced. Initially even gravity didn’t make it fall into place. Time did!
Most patients didn’t come back for many reviews. On her first return visit she complained not that she only had 2 teeth but that they were exactly opposite each other (first left upper and lower molars as I remember) and that, while her mouth had been held wide open by the tumour, the teeth had grown and she still couldn’t completely close her mouth. I didn’t want to remove them so I told her to keep clenching her teeth together hard. She did that, and drove them back so that she could close her mouth.
When I had suggested it I thought that I might have to extract the teeth. As you can see in the next picture her hemi-face became very heavily pigmented, but she had put on weight and was very happy!
Barry Hicks
Medical Memoirs 