Successful en bloc resection with acceptable cosmesis of a right recurrent giant scalp-orbital AVM causing severe disfigurement: a case report

Scalp arteriovenous malformation (AVM) accounts for only 8.1% of all AVM cases. These lesions are made up of an abnormal fistulous tangle of tortuous dysmorphic network of vessels “vascular nidus” directly connecting between the feeding arteries and draining veins, without capillary connection, located within the incision subcutaneous layer. With time, these congenital lesions may evolve and enlarge and clinically manifest with variable features. The only effective method of preventing evolution of these malformations is to exclude the lesion completely from the circulation. Involvement of the orbit and face may cause severe facial disfigurement, proptosis, visual obstruction and even facial palsy. These lesions may be complicated by ulceration, infection and profuse bleeding. We present a rare case of 40-year-old female with a recurrent congenital scalp-orbital AVM causing proptosis, visual obstruction on the right eye with severe facial disfigurement. The lesion was successfully excised with acceptable cosmesis and removal of the visual obstruction. A brief literature review, imaging findings and the surgical techniques have been presented.

Malignant peripheral nerve sheath tumor of the nose with coincidental HIV infection: Management challenges in limited resource setting

Malignant peripheral nerve sheath tumor (MPNST) is an uncommon soft-tissue sarcoma, with origin from the peripheral nerves or somatic tissues associated with nerve sheath. This tumor rarely affects the nose. The clinicopathological presentations vary from one case to another and depend on cells of origin. Hence, the diagnosis is often difficult. We report a 36-year-old woman who presented in our facility with progressive nasal swelling, facial deformity, and mucopurulent rhinorrhea of 3 years duration. Examination revealed a significant facial disfigurement. She was also found to be seropositive. Computerized tomography scan of paranasal sinuses showed an expansile, subtle enhancing, predominantly low-density mass, with area of air density within it, completely occupying the nasal cavity. No calcific focus, lytic or sclerotic surrounding bones changes seen. Her tumor was resected under general anesthesia with a wide margin, and regional vital structures were preserved. The histological/immunohistochemistry revealed MPNST. She had adjuvant radiotherapy and chemotherapy 2 weeks after surgery. However, a nodule was seen on the floor of the nose few days after completion of radiotherapy, which was biopsied, and histological report was positive for malignancy. The persistence of the malignancy and HIV hampered her facial reconstruction. Also, limited finance hampered procurement of the nasal prosthesis. She was followed up for 1 year before she was lost.

Policies and Price Tags: The Public’s Perception of Face Transplantation and Its Funding

Study Design: Survey study. Objective: Facial vascularized composite allotransplantation (FVCA) can cost over 1 million dollars per procedure and is usually not covered by insurance, yet this financial burden and public opinion surrounding this procedure are not well understood. This study is the first to evaluate the layperson’s opinions on the allocation of financial responsibility for FVCA and its inclusion in organ donation registries. Methods: Eight hundred and fifteen laypersons were surveyed through MTurk to assess their agreement with 11 statements about FVCA perceptions, funding, and inclusion on organ donation registries. Responses were analyzed with the Wilcoxon Signed-Rank test, the Kruskal-Wallis test, and the Dunn’s test. Results: The majority of respondents were supportive of FVCA in 10 out of 11 statements ( P < 0.0001). They would be willing to undergo FVCA if they suffered from facial disfigurement; believe FVCA is as important as other organ transplants; believe faces should be included on the organ donation registry; support insurance companies providing coverage for FVCA regardless of trauma etiology; support tax dollars funding the procedure; and believe FVCA improves physical appearance and quality of life. Although respondents generally supported their tax dollars funding the procedure, fewer supported this for self-inflicted trauma ( P > 0.01). Conclusions: This study highlights a disconnect between public preference for insurance coverage of FVCA and current lack of coverage in practice. Respondents’ acceptance of including faces in organ donation registries may help alleviate the issue of locating a donor, and increasing financial coverage may broaden this procedure’s accessibility to a wider range of individuals.