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1.A Case of pulmonary lymphangioleiomyomatosis developed in tuberous sclerosis with renal angiomyolipoma.

Myung Im KIM ; Hong Mo KANG ; Ho Jong LEE ; Yong Ho KOH ; Yong Seon CHO ; Jee Hong YOO

Tuberculosis and Respiratory Diseases 2000;48(5):788-793

Tuberous sclerosis is a rare hereditary anomaly of variable penetrance characterized by mental retardation, epilepsy, and adenoma sebaceum. Pulmonary involvement is uncommon, but, when involve by tuberous sclerosis, it shows characteristic reticulonodular infiltration and cystic changes. Lymphangioleiomyomatosis is a rare disease of unknown cause that affects women of reproductive age. It is characterized by progressive proliferation of smooth muscle in the lung. We experienced one case of pulmonary lymphangioleiomyomatosis associated with tuberous sclerosis in a 26-year-old female patient. This case is reported with a brief of the literature.
Adult ; Angiomyolipoma* ; Epilepsy ; Female ; Humans ; Intellectual Disability ; Lung ; Lymphangioleiomyomatosis* ; Muscle, Smooth ; Penetrance ; Rare Diseases ; Tuberous Sclerosis*

Grice extra-articular subtalar arthrodesis has been performed as either a temporizing or a definite procedure in young children to correct the dynamic hindfoot valgus deformity without affecting subsequent growth of the foot. Fifty eight extra-articular subtalar arthrodesis in thirty patients, performed at Seoul National University Children's Hospital from setpember 1985 to June 1989, were retrospectively reviewed to evaluate the clinical and radiographic results. There were 52 valgus feet in 26 patients and 6 varus feet in 4 patients secondary to cerebral palsy, meningomyelocele, congenital snomalies, and other neuromuscular diseases. In many instances, additional tendon surgeries were also required to correct deformities or achieve the muscle balance. The review consisted of personal interview, physical examination, and radiological assessment. On physical examination, preoperative hindfoot valgus deformty(mean: 14.3°clinically) was well corrected, postoperative hindfoot valgus ranging from neutral to valgus 5°clinically in 35 of 52 cases(67.3%). Correction of the lateral talocalcaneal angle on standing lateral radiographic view averaged 8.5 degrees(from 57.3 degrees preoperatively to 37.6 degrees postoperatively). In valgus feet, there were 3 cases with mild pain around the ankle joint. There were 5 cases of graft resorption and 2 cases of nonunion. Graft failure was more frequent when the proximal end of the graft was directed anterior to the weight bearing axis. With original Grice subtalar arthrodesis, satisfactory results were obtained in 34 of 52(65.4%) hindfoot valgus feet. In 6 varus feet, there were 1 undercorrection and 2 recurrence of varus deformity. We re-emphasize the importance of strict operative technique to obtain satisfactory results. We also believe that Grice procedure may be used for the correction of subtalar instability in selected cases of varus foot before muscle baancing procedures.
Ankle Joint ; Arthrodesis ; Cerebral Palsy ; Child ; Congenital Abnormalities ; Foot ; Humans ; Meningomyelocele ; Neuromuscular Diseases ; Physical Examination ; Recurrence ; Retrospective Studies ; Seoul ; Tendons ; Transplants ; Weight-Bearing