Pathophysiology
Histologically, Gorham’s disease is characterized by inflammatory osteolysis of bony segments replaced by localized proliferative lymphatic channels [PMID:25880917].
Epidemiology
It usually occurs in children and young adult patients, most commonly in the 2nd and 3rd decades, though cases span from 1.5 to 72 years, with no race or gender predilection [PMID:25880917].
Management
We present a case managed by a single-stage craniectomy and skull reconstruction using a synthetic bone flap [PMID:25880917].
In a study by Berli et al. [PMID:26163837], 7 patients underwent immediate reconstruction with customized craniofacial implants following calvarial resection, including 4 with malignant pathology, with no identified implant-related complications during follow-up.
In a study by Blake et al. [PMID:2224346], titanium was successfully utilized for cranioplasty and malar defect reconstruction, yielding very satisfactory results without complications.
Complications
Berli et al. [PMID:26163837] reported no implant-related complications in their cohort of 7 patients with follow-up ranging from 1 to 16 months post-implantation.
Blake GB et al. [PMID:2224346] reported no complications following the use of titanium prostheses in reconstructive procedures for skull and facial defects.
Prognosis & Follow-up
The study by Berli et al. [PMID:26163837] noted that customized craniofacial implants required intraoperative modifications averaging 13.8% of the predicted defect size, with adjustments ranging from 0.2% to 40.8%.
The study by Blake GB, MacFarlane MR, and Hinton JW [PMID:2224346] indicated very satisfactory results from titanium implant use, suggesting positive long-term prognoses for these patients.
References
1 Ohla V, Bayoumi AB, Hefty M, Anderson M, Kasper EM. Complex single step skull reconstruction in Gorham's disease - a technical report and review of the literature. BMC surgery 2015. link 2 Berli JU, Thomaier L, Zhong S, Huang J, Quinones A, Lim M et al.. Immediate Single-Stage Cranioplasty Following Calvarial Resection for Benign and Malignant Skull Neoplasms Using Customized Craniofacial Implants. The Journal of craniofacial surgery 2015. link 3 Blake GB, MacFarlane MR, Hinton JW. Titanium in reconstructive surgery of the skull and face. British journal of plastic surgery 1990. link90115-g)