 Please advise me on the management of this 56
year old male, suffering from Pycnodysostosis. He is mainly wheelchair bound, but
transfers with the aid of crutches.
|
 |
Approximately 15 years ago he presented with a draining sinus at the MP
joint of his big toe, which was treated with long-term antibiotics. This sinus started
draining again about two months ago, but stopped after a few weeks on antibiotics.
|
 |
|
Current problem: Draining sinus on pulp of thumb. This was painful just
over a year ago. The skin overlying the sharpened, palpable tip of the phalanx (see
attached X-ray) was sore, especially when using his crutch with that hand. It subsided
slowly whist only being observed. At that time I was worried about the tip of the phalanx
(which was palpable proximal to the bulk of the pulp, as the terminal tuft has resorbed).
Two weeks ago he presented, with (yes, you guessed it)a sinus over the tip of this
phalanx, where the skin seemed to have broken down, draining only a little (still awaiting
culture results). The patient is very worried about surgery (I am thinking of a
debridement with rounding off of the tip of the phalanx), as it will make him bed-bound
for the duration of the recovery (which I suspect might be lengthy) as he will not be able
to use his crutch |
 |
Follow up This patient was previously reported in the literature (Mills
KL, Johnston AW. Pycnodysostosis. J Med Genet. 1988 Aug;25(8):550-3.)
Patient is of normal intelligence (he e-mailed me some of the images), transfers from
wheelchair to bed, using crutches.
Thumb developed draining sinus over tip of terminal phalanx (sharp tip of phalanx eroded
through skin due to increased pressure when taking weight on crutch). Healed with
self-applied topical antibiotics (!)
Previous draining sinus over MP joint R big toe. It healed with only antibiotics. This has
been quiescent for 10 years
|
| Does anybody have experience with similar problems in
Pycnodysostotic patients? If so, please let me have your thoughts.
Marthinus Roos
Elgin, Scotland
|
|
Orthopaedic Rare Conditions Database (ORCID)
We subscribe to the HONcode principles.
Verify here