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Peripartum Management of a Parturient with Gorham Disease
Abstract Number: T1D-1
Abstract Type: Case Report/Case Series
Gorham disease (GD) is a rare form of sporadic idiopathic osteolysis. Characterized by destruction of bone by proliferation of neovascular tissue; it is also known as 'vanishing bone disease'. (1) Patients with GD present with pathological fractures or pain and weakness. It can also involve soft tissues, leading to hemangiomas and lymphangiomatosis. Consumptive coagulopathy, development of severe preeclampsia, chylothorax and involvement of the mandible and cervical spine resulting in difficult airway management have been reported (1,2). We describe the peripartum management of a parturient with GD presenting for elective cesarean delivery (CD).
A 38-year-old G2P0 with an IVF-conceived singleton pregnancy and gestational diabetes was seen in the high-risk anesthetic clinic. GD was diagnosed in her 20s confined to her right arm. She required major reconstructive surgery with bone-grafting of the humerus and artificial wrist prosthesis with resultant loss of function, lymphedema, repeated bouts of cellulitis and neuropathic pain. There was no spine or airway involvement. Previous infertility treatment was complicated by cellulitis in her affected arm requiring hospitalization and IV antibiotics. The patient was concerned of transient bacteremia increasing the risk of cellulitis during labor and thus requested elective CD. At 38+5 weeks, the arm swelling and pain worsened, CD was expedited and performed under spinal anesthesia. Surgery proceeded uneventfully; neonatal Apgars were 9,9. Antibiotic prophylaxis with IV cefazolin was continued for an additional 24 h. On postpartum day 1, the patient suffered burning incisional pain that was successfully managed with lidocaine infiltration and pregabalin. There were no signs of infection post-delivery and discharge was on day 3.
This case illustrates the peripartum management of a parturient with GD complicated by lymphedema, loss of function and neuropathic pain in the affected limb. Most cases of parturients with GD in the literature have undergone CD due to complications; vaginal delivery may be possible, but our patient elected for CD to minimize infective complications. GD may be exacerbated during pregnancy due to neovascularization of active disease. Careful antenatal anesthesia evaluation to confirm a stable disease process with close postpartum monitoring for pain and rare complications are vital in the safe management of parturients with GD.
1. Bargagli E. et al. AJP Rep 2016
2. Gambling et al. IJOA 2011