Gorham's disease of femur.

Gorham's disease is a rare condition of bones characterized by spontaneous massive and progressive osteolysis. Less than 200 cases have been reported so far. Femur is an uncommon site of Gorham disease. We report a young female, presenting as pathological fracture of the femur, wherein rapid osteolysis of femur occurred. The clinical, pathological, and radiological picture suggested a diagnosis of Gorham's disease. The patient was subjected to radiotherapy (40 Gys) and put on bisphosphonates. At a follow-up of 2 years, the disease process had stopped and partial recalcification of the bone had occurred.

Walnut tree falls as a cause of musculoskeletal injury--a study from a tertiary care center in Kashmir.

BACKGROUND: Fall from height is one of the important causes of musculoskeletal injuries. Fall from walnut trees constitutes an important entity that leads to a significant mortality and morbidity amongst those engaged in fruit collection. The present study aimed to determine the prevalence of different musculoskeletal injuries in these victims and to highlight the importance of recognizing this incident as an occupational injury. METHODS: A retrospective study of all patients admitted to the Orthopedic Department of the Sher-i-Kashmir Institute of Medical Sciences (SKIMS) Medical College from January 2003 to December 2007 was conducted. The medical records were studied for the different types of bony injuries, associated injuries and delay in the referral of patients. RESULTS: 94% of falls occurred from August to October. Of the 115 patients, 63 (54.7%) had associated non-orthopedic injuries. Head injury was the most common associated injury, presenting in 34 patients (29.5%). Thoracolumbar (16.5%), calcaneum (10.5%) and distal radius (8.7%) fractures constituted the common fractures encountered in the victims. CONCLUSION: Walnut tree injuries mostly involve young males who form the productive group of the population. Being a seasonal injury, it puts a considerable load on the health resources of the region over a small period of time.

Big toe metastases as first clinical sign of occult bronchogenic carcinoma.

Metastasis to bone is a common manifestation of malignant tumours. However, metastasis to the bones of the foot is exceedingly rare. It usually arise from genitourinary tract and colon, with the tarsal bones accounting for half of all metastases. We report a rare case of pain and destruction of the distal phalanx of great toe, initially suspected as osteomyelitis, which on biopsy proved to be metastases from a clinically silent bronchogenic carcinoma.

Osteoblastoma of body of the talus--Report of a rare case with atypical radiological features.

Osteoblastoma is a benign bone tumour found commonly in the spine and long tubular bones. Involvement of the talus is uncommon, and when present, is found in the neck of the talus. Osteoblastoma of the body of talus is a very rare entity. We report a young male, presenting as chronic ankle pain, with a radiolucent lesion with a thick periosteal shell in the body of the talus. Analysis of clinical, radiological and histological findings confirmed the diagnosis of osteoblastoma. The case is reported for the rarity of the site and atypical radiological features that osteoblastoma can present with.

Ellis-van Creveld syndrome with facial hemiatrophy.

Ellis-van Creveld (EVC) syndrome is a rare autosomal recessive congenital disorder characterized by chondrodysplasia and polydactyly, ectodermal dysplasia and congenital defects of the heart. We present here a case of a 16-year-old short-limbed dwarf with skeletal deformities and bilateral postaxial polydactyly, dysplastic nails and teeth, also having left-sided facial hemiatrophy. The diagnosis of EVC syndrome was made on the basis of clinical and radiological features. To the best of our knowledge, this is the first report of EVC syndrome with facial hemiatrophy in the medical literature from India.

Mitchell's osteotomy in the management of hallux valgus: An Indian perspective.

BACKGROUND: Hallux valgus is a common condition that affects the forefoot. A large number of procedures are described for managing this condition. Mitchell's osteotomy and its modifications are being widely used for treating hallux valgus. However, most of the studies describe the results from the developed world. We present results of the classic Mitchell's osteotomy in hallux valgus in Indian subcontinent. MATERIALS AND METHODS: Forty eight adult patients (including 12 bilateral ones) in the age range of 18-60 years with hallux valgus were managed with the classic Mitchell's osteotomy. Pain over the bunion was the reason for surgery in 53 of 60 feet and cosmesis in the remaining 7 feet. Patients with hallux valgus angle more than 20 degrees and not responding to a trial of conservative treatment were included. Patients having metatarsophalangeal (MTP) joint osteoarthritis (Grade II and higher), hallux rigidus, rheumatoid arthritis, and with subluxation of MTP joint were excluded from the study. Further, patients with first metatarsal more than 3 mm shorter than second metatarsal were also excluded. RESULTS: The average follow-up period is 3 yrs (range 18months - 6yrs). About 55 feet (83%) were painless after surgery. Forty-two (70%) patients were happy with the cosmetic results of the surgery. Metatarsalgia was the reason for dissatisfaction with the procedure in five patients. The average correction of hallux valgus and the intermetatarsal angles achieved was 19.7 degrees and 6.9 degrees , respectively. Using the Broughton and Winson scoring system, 37 (61.7%) feet had excellent results, 18 (30%) had good, and five (8.3%) feet had a poor results. CONCLUSION: The classic Mitchell's procedure is a simple procedure and gives good cosmetic and radiological results.

Familial reactive perforating collagenosis.

BACKGROUND: Reactive perforating collagenosis (RPC) is one of the rare forms of transepidermal elimination in which genetically altered collagen is extruded from the epidermis. This disease usually starts in early childhood as asymptomatic umbilicated papules on extremities, and the lesions become more conspicuous with age. AIMS: The objective of our study was to determine the clinico-pathological features of RPC and the response to various treatment modalities. METHODS: Ten patients of RPC, belonging to five different families, were studied clinically. Various laboratory investigations were carried out and diagnosis was made by histopathology of the lesions. Patients were given various topical and oral treatments. RESULTS: RPC is familial in most cases without any definite inheritance pattern. It begins in childhood and the lesions are usually recurrent and become profuse and large with age. Systemic diseases have no role in the onset of lesions. CONCLUSION: Oral and topical retinoids in combination with emollients is the best treatment option.

Unilateral cleft hand with cleft foot.

Congenital anomalies of the hand form an important class of congenital malformations. They have a huge functional importance because of the part played by the hand in the daily activities of a person. The deformities also have significant cosmetic significance and may also be associated with other anomalies. Amongst the congenital anomalies, central deficiency or cleft hand is relatively rare. The association of cleft foot with cleft hand is an even more rare occurance. We present a case report of a 6 year old child, born of a non-consanginous marriage, having congenital central deficiency of ipsilateral hand and foot.