Fibrous dysplasia: an overview of disease process, indications for surgical management, and a case report.

INTRODUCTION: First described by Von Recklinghausen in 1891, fibrous dysplasia is a developmental defect of osseous tissue such that bone is produced with an abnormally thin cortex and marrow is replaced with fibrous tissue that demonstrates characteristic ground-glass appearance on x-ray examination. The underlying defect in fibrous dysplasia is a mutation of the GNAS1 gene, which leads to constitutive activation of gene products that preclude the maturation of osteoprogenitor cells and lead to development of abnormal bone matrix, trabeculae, and collagen, produced by undifferentiated mesenchymal cells. There exists a mainly self-limiting form of fibrous dysplasia classified as monostotic, which is characterized by dysplastic bone in a single location that remains relatively stable throughout life and a polyostotic form, which can exhibit aggressive growth placing adjacent structures at risk for compressive sequelae. METHODS: We present the surgical management of an unusual case of monostotic fibrous dysplasia, which exhibited aggressive growth with mass effect, and late presentation, both uncharacteristic features for the monostotic form. The authors also performed a comprehensive review of the literature and discuss the disease process, management options, and indications for surgical treatment. RESULTS: An overview of the disease process and management options is presented. The authors also present details of reconstruction in an unusual form of symptomatic monostotic fibrous dysplasia. CONCLUSION: Conservative management is usually the mainstay of therapy in asymptomatic cases of fibrous dysplasia. In patients fulfilling criteria for surgical management, craniofacial reconstruction offers a viable option in the surgeon's armamentarium, providing good functional and cosmetic outcomes.

Revisiting the free nipple graft: an opportunity for nipple sparing mastectomy in women with breast ptosis.

OBJECTIVE: Nipple areolar complex (NAC) sparing mastectomy improves the cosmetic outcome of patients with breast cancer. However, women with significant breast ptosis are not candidates for this technique due toexcessive skin flap length and ensuing risk of NAC ischemia.1 (-) 3 We report a novel technique using free nipple graft during skin sparing mastectomy for patients with significant ptosis while concurrently maintaining oncologic integrity. DESIGN: Case series. SETTING: Community and tertiary care hospital practices. PATIENTS: Women with breast cancer desiring NAC preservation who are otherwise candidates for nipple sparing mastectomy, but with significant breast ptosis that precludes NAC viability. All women underwent immediate, autologous breast reconstruction. INTERVENTIONS: Bilateral and unilateral free nipple grafts were harvested, placed on ice during skin sparing mastectomy and free flap reconstruction, grafted at the conclusion of the case and secured with a bolster. OUTCOME MEASURES: Full or partial NAC preservation, ischemia time, local wound complications at NAC grafting site, pathologic outcomes. RESULTS: A total of three patients underwent free nipple grafting at the time of skin sparing mastectomy and free or pedicled flap for breast cancer between March and September 2012. Of five total nipple grafts, one had partial NAC loss but did not require operative debridement. Pathologic review of areolar tissue removed during intraoperative defatting of free nipple graft demonstrated residual duct epithelium. CONCLUSIONS: Women with significant breast ptosis that would preclude them from NAC sparing mastectomy can successfully preserve their NAC using a free nipple graft. Duct epithelium present in defatted tissue during preparation of the free nipple graft suggests that oncologic integrity can also be maintained.

Reconstruction of massive localised lymphoedema of the scrotum with a novel fasciocutaneous flap: A rare case presentation and a review of the literature.

Massive localised lymphoedema (MLL) is a benign lymphoproliferative soft-tissue overgrowth in the morbidly obese patient. The diagnosis may be challenging, and is a form of secondary lymphoedema, often described as idiopathic scrotal elephantiasis. The lesion presents as a large mass in the morbidly obese, and patients seek treatment late in the disease course due to limitation of daily living or excoriation and wound breakdown. Resection, followed by reconstruction, is indicated in these cases. We present a unique case of a morbidly obese 52-year-old male with massive enlargement of the scrotum present for several years duration, despite massive weight loss (88.85 kg) from gastric bypass surgery and no other identifiable cause of lymphoedema. Scrotal lymphoedematous tissue was resected and scrotal reconstruction with a novel posterior fasciocutaneous flap from the scrotum was performed in addition to penile reconstruction with a skin graft and local fasciocutaneous flaps as well as a panniculectomy. Histologically, the tissue was characterised by marked oedema with dermal fibrosis and patchy mild perivascular chronic inflammation. Postoperative follow-up revealed wound integrity and patient satisfaction with the outcome. MLL is an important disease process with distinct clinical and histopathologic characteristics that often requires complex reconstruction. Although there are several opposing classification schema, we propose the incorporation of idiopathic scrotal elephantiasis into the diagnostic category of MLL.

The use of intra-abdominal tissue expansion for the management of giant omphaloceles: review of literature and a case report.

Giant omphaloceles present a reconstructive challenge in planning, management, and eventual closure of the abdominal wall defect. The goal of reconstruction is to recreate a functional abdominal wall domain and return the extra-anatomically placed viscera into the peritoneal cavity in a safe manner. Traditionally, placement of tissue expanders has been in the subcutaneous and intramuscular planes. Recently, however, there have been reports of intra-abdominal placement of expanders. We present a detailed review of the literature regarding the use of tissue expanders in the management of giant omphaloceles with specific emphasis on the intra-abdominal technique of placement. We also present a case report with the longest follow-up till date in which the patient underwent staged reduction using the intra-abdominal approach. Initial reports of this modality are promising both as a primary strategy and in patients in whom conventional techniques have failed. Results from our review of literature and case report suggest that this technique appears to be durable and effective with successful functional and cosmetic outcomes.