Isolated mediastinal lymphangioma herniating through the intercostal space

Lymphangiomas are congenital malformations of the lymphatic system, and 90% have manifested by the end of the second year of life. While 75% of these are located in the cervical region, only 2% to 3% are associated with an intrathoracic extension. An isolated mediastinal lymphangioma without a cervical component is an uncommon occurrence. Presented here is an isolated mediastinal lymphangioma that herniated through the intercostal space to present as a cystic mass in the parasternal region, which has not been reported so far.     

Reversal of pancytopenia following kidney transplantation in a patient of primary hyperoxaluria with bone marrow involvement

Combined liver and kidney transplantation is the ideal treatment for patients with end-stage renal failure secondary to primary hyperoxaluria and systemic oxalosis, with a functioning liver providing replacement of the deficient enzyme and a functioning kidney providing the route of excretion for the oxalate crystals. Pancytopenia from bone marrow infiltration of oxalate crystals is a rare complication of primary hyperoxaluria, and its reversal following transplant has not been described. We report the first case of pancytopenia from marrow infiltration by oxalate crystals reversing following a successful kidney transplant alone. Although kidney alone transplants do not provide the best chance of survival or quality of life as compared to a combined kidney and liver transplant, a well functioning kidney transplant is able to take care of the systemic oxalate load and ameliorate, at least for a period of time, the systemic complications of oxalosis.     

Efficacy of multiwavelength light therapy in the treatment of pressure ulcers in subjects with disorders of the spinal cord: A randomized double-blind controlled trial

OBJECTIVE: To study the efficacy of multiwavelength light therapy in the treatment of pressure ulcers in subjects with spinal cord disorders. DESIGN: Randomized controlled trial. SETTING: Neurologic rehabilitation ward of a referral center in India. PARTICIPANTS: Thirty-five subjects with spinal cord injury, with 64 pressure ulcers (stage 2, n=55; stage 3, n=8; stage 4, n=1), were randomized into treatment and control groups. One subject refused consent. Mean duration of ulcers in the treatment group was 34.2+/-45.5 days and in the control group, 57.1+/-43.5 days. INTERVENTIONS: Treatment group received 14 sessions of multiwavelength light therapy, with 46 probes of different wavelengths from a gallium-aluminum-arsenide laser source, 3 times a week. Energy used was 4.5 J/cm(2). Ulcers in the control group received sham treatment. MAIN OUTCOME MEASURES: Healing of the ulcer, defined as the complete closure of the wound with healthy scar tissue, time taken for the ulcer to heal, and stage of the ulcer and Pressure Sore Status Tool score 14 days after last treatment. RESULTS: There was no significant difference in healing between the treatment and control groups. Eighteen ulcers in treatment group and 14 in control group healed completely ( P =.802). Mean time taken by the ulcers to heal was 2.45+/-2.06 weeks in the treatment group and 1.78+/-2.13 weeks in the control group ( P =.330). Time taken for stage 3 and 4 ulcers to reach stage 2 was 2.25+/-0.5 weeks in treatment group and 4.33+/-1.53 weeks in control group ( P =.047). CONCLUSIONS: Multiwavelength light therapy from a gallium-aluminum-arsenide laser source did not influence overall healing pressure ulcers. Limited evidence suggested that it improved healing of stage 3 and 4 pressure ulcers.     

Benign metastasizing leiomyomatosis: case report and review

The authors report an interesting case of a minimally symptomatic 23-year-old African American woman who was found to have extensive diffuse reticulonodular opacities of the lungs on a routine chest radiograph. She had a hysterectomy 5 years previously for multiple leiomyomas of the uterus. She had no history of any prior exposure to dusts or toxins. Collagen vasculitides and bacterial, mycobacterial, and fungal infectious causes were excluded through standard testing, and a bronchoscopic lung biopsy was nonspecific. An open lung biopsy revealed multiple nodules of proliferating smooth muscle cells intermixed with irregular areas of epithelial-lined spaces. Histologically, the muscle cells appear benign with a very low mitotic rate, and the pathologic findings were consistent with benign metastasizing leiomyomatosis (BML). Staining for estrogen and progesterone receptors, actin, and c-kit were performed. This case and the review of the medical literature support the concept that BML originates from an antecedent leiomyoma of the uterus in virtually all cases with rare exceptions. It appears that tumor metastasizes to lungs or other extrauterine tissues via hematogenous spread. However, the origin of the tumor remains controversial. BML is a rare entity, with only a handful of reports in the medical literature. The authors report an interesting case of BML in a 23-year-old patient who, to their knowledge, is the youngest such patient described and who, at 13 years, has the longest period of clinical follow-up. In this article, the authors review the pathogenesis, cytogenetics, histologic markers, and management options of this rare entity.     

Pulmonary function tests in children with adenotonsillar hypertrophy

OBJECTIVE: Adenotonsillar hypertrophy, a common malady in children may lead on to cardiopulmonary dysfunction in untreated cases. The present study was designed to see alteration in pulmonary function tests i.e. spirometric parameters and blood oxygen saturation consequent to adenotonsillar hypertrophy and subsequent changes after adenotonsillectomy. METHODS: Spirometry and pulse oximetry were carried out in 40 children with adenotonsillar hypertrophy of both sexes between the age of 7 and 14 years and 40 age and sex matched healthy children. The test was repeated one and half months after surgery in study group and data was statistically analyzed using students' t-test. RESULTS: The flow volume plot was abnormal in all the patients along with hypoxia, reduced FIF50% and, increased FEF50%/FIF50%, FEV1/PEFR, FEV1/FEV0.5 ratios. There was statistically significant improvement in above parameters following surgery. CONCLUSIONS: Spirometry can prove an useful diagnostic tool in adenotonsillar hypertrophy for deciding early intervention to prevent cardio pulmonary complications.     

Histologic and Manometric Studies on the Esophagus Following Endoscopic Sclerotherapy

Objective The aim of this work was to study the histologic and manometric changes in the distal esophagus beyond 2 years following endoscopic sclerotherapy (EST) and/or surgical intervention, and to try to understand the etiological factors associated with these changes. Patients and interventions Forty patients, with an average age of 61.5 years, were studied for 2–12 years following sclerotherapy and/or surgical intervention. The causes of liver disease were alcoholic cirrhosis (78.6%), primary biliary cirrhosis (14.3%), and chronic aggressive hepatitis (7.1%). A predominant number of cases (65%) had a mesocaval interposition shunt due to the failure of EST, 32.5% EST alone, and 2.5% esophageal devascularization. All patients had esophageal manometry following mucosal biopsies taken in duplicate endoscopically from three levels of the distal esophagus. Results In the EST and shunt groups, 88.5% had manometric abnormalities, esophagitis, and chronic inflammatory changes. In the EST group, all but two patients had manometric abnormalities and chronic inflammatory changes. Analysis of the patient groups on the basis of the number of EST sessions and the amount of sclerosant injected showed that both histologic changes and dysmotility were more profound in those treated over five times with EST. The differences were significant. Conclusion It appears that EST causes persistent manometric abnormalities and chronic inflammatory changes in the distal esophagus, the severity of which seems to vary directly with the frequency of sclerotherapy and not amount of sclerosant injected.