Advanced Unilateral Fibrous Dysplasia of the Scapula: A Rare Clinical Entity and Surgical Challenge.

INTRODUCTION: Fibrous dysplasia (FD) is an uncommon benign tumor of bone. Although FD can affect flat bones, it is rare for the scapula to be involved. In addition, little is known about the management of FD when it involves the scapula. We present possibly the first comprehensive case report of the management of advanced unilateral FD of the scapular region. CASE REPORT: A 47-year-old male presented to us with pain and swelling over the left shoulder. The swelling was 11 cm × 15 cm × 8 cm and was hard and tender with rough texture. Radiograph showed large homogenous lesion with irregular but well-defined margins and a ground glass appearance. Magnetic resonance imaging scans showed well-defined borders with the expansion of the bone, with intact overlying cortices and endosteal scalloping. Biopsy confirmed the lesion to be FD. An innovative application of an existing surgical technique to minimize the impact of the residual deformity and dead space left after curettage of the scapula was done. The patient had good clinical and functional outcome at 6-month follow-up. CONCLUSION: Surgical exercise in FD is purely on symptomatic basis. In our case, the swelling was causing most discomfort, and we curettaged and compressed the bony swelling which resulted in excellent outcome in this patient.

Surgical Treatment of Dupuytren's Contracture; Results and Complications of Surgery: Our Experience.

Dupuytren's disease is one of the fibro-proliferative conditions affecting the palmar and digital fascia. This disease has been known to surgeons and treated by them for at least 200 years. Dupuytren's disease is very common in Northern Europe and also in countries inhabited by immigrants from Northern Europe. Dupuytren's contracture is stated to be uncommon in Indian subcontinent and very little has been written about this disease in India. The authors have however come across 30 patients with Dupuytren's contracture and they are the subject of this paper, with special emphasis on its surgical correction and complications of surgical treatment. Patients were categorized into stages I, II and III as per the severity of disease. Regional fasciectomy was performed in 90% of the patients and extensive fasciectomy in 10% of patients. Most of wounds were closed by primary closure with z-plasty. In rest of the patients free skin grafts were used to close the wound, when primary closure was not possible. The patients were followed up regularly for 5 years and the results of surgical treatment were categorized into excellent, good, fair and poor. The results were excellent in 23 patients (76.66%), good in six patients (20%) and fair in one patient (3.33%). Post operative edema and some stiffness was seen in two patients. Wound infection was seen in one patient and haematoma in one patient. The correct surgical technique and meticulous post operative care is needed to achieve higher rates of correction and to limit the complications and recurrence.

Incidence and pattern of bear maul injuries in Kashmir.

BACKGROUND: Injuries due to mauling by bears are rarely reported in the literature. The high incidence of such injuries in Kashmir, India, which is a valley surrounded by dense forests and is a habitat of Asiatic Black bears, urged us to undertake such a study in our department. MATERIALS AND METHODS: The study was conducted both retrospectively (January 1990-July 2005) and prospectively (August 2005-December 2007). RESULTS: A total of 417 cases were recorded and all the injuries were caused by black bears alone. Majority of the patients were young to middle-aged (96.8% of cases) and predominantly males (80.33%). The incidence was highest during July to November (76.82%), and most of the attacks (97%) occurred during daytime. Soft-tissue injury occurred in all 417 cases, bones were involved in 131 (31.41%) while the visceral organs were injured in four (12.94%) patients. The face (80.57%) was most common part of the body injured, followed by the head (54.67%), and all the patients had soft-tissue injury (100%). In spite of devastating injuries caused by bear maulings, the mortality rate was only 2.39%. CONCLUSION: Injuries due to mauling by black bear occur mainly between July and November. The young and middle-aged men have a higher tendency to be wounded. The face and head were the most commonly affected sites, while visceral injuries were rare. Mortality was low, and reconstruction of many of the injuries was challenging, often necessitating a staged procedure. Those living in villages close to black bear habitats may benefit from education related to the risk and severity of the attacks in the hope of reducing the number of injuries seen.

Mutational spectrum of conserved regions of TP53 and PTEN genes in Kangri cancer (of the skin) in the Kashmiri population.

Kangri cancer is a unique, thermally induced squamous cell carcinoma (SCC) of the skin that develops due to persistent use of a Kangri (a brazier) by the Kashmiri people to combat the cold temperature during winter. Unlike classical UV-induced SCC of the skin, Kangri cancer appears on the legs and abdomen. Its common features are erythematous patches, recurrence and metastasis. In the absence of any molecular etiology, we made a preliminary attempt to estimate the nature and frequency of mutations in the TP53 and PTEN genes in Kangri cancer patients from Kashmir. PCR-SSCP analysis followed by direct sequencing revealed that TP53 mutations account for 40% (12/30) of sporadic Kangri cancer patients and that PTEN mutations account for only 6.6% (2/30). There were 16 mutations in TP53 exons 5 and 7, found in 12 patients. They consisted of 11 substitutions (7 transitions, 3 transversions and 1 double-base) and 5 insertions. The 11 substitutions represent 8 distinct missense mutations, 3 of which were silent mutations. The mutations detected in the PTEN gene consisted of one insertion and one C>T transition. This high percentage of TP53 mutations (especially A>G) showed a statistically significant association with age and positive lymph node status. Our results indicate that TP53 is a predominant target of chronic hyperthermia in the development of Kangri cancer in the moderate risk Kashmiri population. The differences in the TP53 mutation spectrum of UV-induced SCC of the skin and Kangri cancer are probably due to the nature of the respective environmental carcinogens. The study also suggests that TP53 may function as a potential molecular marker and prognostic tool, at least in a subset of sporadic Kangri tumors.

Primary cutaneous mucormycosis in an immunocompetent host.

Mucormycosis in immunocompetent hosts is rare, and is often related to trauma. We report a case of primary cutaneous mucormycosis in a 26 year old immunocompetent female due to Mucor species. A combination of Amphotericin B and surgical debridement completely eradicated the infection.