Functional results after carpal tunnel release in mucopolysaccharidosis.

BACKGROUND: Mucopolysaccharidosis consists of a group of diseases caused by the deficiency of lysosomal enzymes, which may lead to the compression of the median nerve in the carpal tunnel due to the accumulation of glycosaminoglycan, resulting in the hand disability. The study purpose is to present functional results of carpal tunnel release in mucopolysaccharidosis patients. Patients were selected from an enzyme replacement group in the Department of Pediatric Neurology. The legal guardians of the patients were informed about the likely functional change of the hands induced by compression of the median nerve. Clinical evaluation was performed in those patients who received their legal guardians' consent to participate and was included inspection, assessment of functional level, wrinkle test and the digital pinch function to manipulate small and large objects. Ultrasound and electromyography were performed to confirm the clinical median nerve compression. Bilateral extended opening technique was performed to access the carpal tunnel and analyze the anatomic findings of the median nerve and the flexed tendons of the fingers. After the surgical release of the carpal tunnel, the clinical evaluation was repeated. Subjective observations of the legal guardians were also considered. RESULTS: Seven patients underwent bilateral surgical opening of the carpal tunnel; six boys, mean age of 9.5 (5 to 13), five of them presenting Type II mucopolysaccharidosis, 1 Type I and 1 Type VI. The average follow-up was 12 months (10-13 months). The functional results observed included the improvement in the handling of small and large objects in all children who underwent decompression of the median nerve. The comparison between the pre-operative and post-operative functional levels revealed that 2 patients evolved from Level II to IV, 3 from Level III to IV, 1 from Level IV to V and 1 patient remained in Level III. Tenosynovitis around the flexor tendons and severe compression of the median nerve in the fourteen carpal tunnels were observed during the surgical procedure. In 6 wrists, partial tenosynovitis was performed. CONCLUSIONS: Despite the improvement in the overall function of the children' hands, we cannot conclude that only surgery was responsible for the benefit. Better designed studies are required.

Fraturas instáveis do fêmur em crianças tratadas com hastes intramedulares elásticas de titânio / Unstable femoral fractures treated with titanium elastic intramedullary nails, in children

OBJETIVO: Avaliar as indicações, epidemiologia, lesões associadas, complicações e prognóstico das fraturas diafisárias instáveis do fêmur na criança tratadas com hastes intramedulares elásticas de titânio. MÉTODO: Estudo retrospectivo composto por 24 pacientes com fraturas diafisárias instáveis do fêmur com idade entre cinco e 12 anos, submetidos a tratamento cirúrgico com hastes intramedulares elásticas de titânio no Hospital Universitário Cajuru - Curitiba, PR. Pacientes atendidos no período de abril de 2002 a março de 2008, com seguimento mínimo de 36 meses, submetidos à avaliação de dados epidemiológicos, desvios angulares, encurtamento e consolidação óssea. RESULTADOS: Foram reavaliados os prontuários de 113 casos operados de abril de 2002 a março de 2008, destes, incluídos no estudo 24 casos com fraturas de fêmur diafisárias instáveis tratadas com hastes intramedulares elásticas de titânio com inserção retrógrada. Apresentando duas fraturas bilaterais, duas fraturas expostas, sete pacientes do sexo feminino e 17 masculinos, com idade média de 8,3 anos. Apresentaram, ao final do estudo: encurtamento, desvios em varo ou valgo, retrocurvato ou antecurvato final igual a zero, não havendo ainda presença de retardo de consolidação ou pseudartrose. CONCLUSÕES: As hastes intramedulares elásticas de titânio são de fácil colocação e remoção. Consideramos a utilização das hastes intramedulares elásticas de titânio uma boa opção para a fixação de fraturas instáveis do fêmur em crianças.

OBJECTIVE: To evaluate the indications, epidemiology, associated lesions, complications and prognosis among children with unstable femoral diaphysis fractures who were treated with titanium elastic intramedullary nails. METHOD: This was a retrospective analysis on 24 patients aged 5-12 years with unstable femoral diaphysis fractures who underwent surgical treatment with elastic titanium intramedullary nails at the Cajuru University Hospital, Curitiba-PR between April 2002 and March 2008, with a minimum follow-up of 36 months. The epidemiological data, angular deviations, leg shortening and bone consolidation were evaluated. RESULTS: The medical files of 113 cases operated between April 2002 and March 2008 were reassessed. From these, 24 cases of unstable femoral diaphysis fractures treated with elastic titanium intramedullary nails with retrograde insertion were included in the study. There were two bilateral fractures and two exposed fractures. Seven patients were female and 17 were male, and the mean age was 8.3 years. The following were presented at the end of the study: shortening, varus or valgus displacement, final retrocurvatum or antecurvatum of zero, and absence of delayed consolidation or pseudarthrosis. CONCLUSIONS: The elastic titanium intramedullary nails were easily placed and removed. We believe that using elastic titanium intramedullary nails is a good option for fixation of unstable femoral fractures in children.

UNSTABLE FEMORAL FRACTURES TREATED WITH TITANIUM ELASTIC INTRAMEDULLARY NAILS, IN CHILDREN.

OBJECTIVE: To evaluate the indications, epidemiology, associated lesions, complications and prognosis among children with unstable femoral diaphysis fractures who were treated with titanium elastic intramedullary nails. METHOD: This was a retrospective analysis on 24 patients aged 5-12 years with unstable femoral diaphysis fractures who underwent surgical treatment with elastic titanium intramedullary nails at the Cajuru University Hospital, Curitiba-PR, between April 2002 and March 2008, with a minimum follow-up of 36 months. The epidemiological data, angular deviations, leg shortening and bone consolidation were evaluated. RESULTS: The medical files of 113 cases operated between April 2002 and March 2008 were reassessed. From these, 24 cases of unstable femoral diaphysis fractures treated with elastic titanium intramedullary nails with retrograde insertion were included in the study. There were two bilateral fractures and two exposed fractures. Seven patients were female and 17 were male, and the mean age was 8.3 years. The following were presented at the end of the study: shortening, varus or valgus displacement, final retrocurvatum or antecurvatum of zero, and absence of delayed consolidation or pseudarthrosis. CONCLUSIONS: The elastic titanium intramedullary nails were easily placed and removed. We believe that using elastic titanium intramedullary nails is a good option for fixation of unstable femoral fractures in children.

Linfangite granulomatosa de genitália infantil: relato de caso e revisão da literatura / Granulomatous lymphangitis of children's genitalia: report of a case and review of the literature

Linfangite granulomatosa de genitália não-infecciosa é causa rara de linfedema genital autolimitado e idiopático em crianças(4, 6, 9). A maioria dos casos de linfedema com granuloma não-infeccioso de genitália em pacientes jovens ocorre em associação à doença de Crohn subseqüente ou concomitante(1, 3, 4, 9). O caso relatado é de um menino de 14 anos que apresentava história de linfedema genital principalmente em região dorsal do corpo do pênis, poupando prepúcio, parte distal do pênis e saco escrotal. Estudos laboratoriais não revelavam doenças sistêmicas. O exame anatomopatológico da peça revelou tratar-se de linfangite granulomatosa não-infecciosa.

Non-infectious granulomatous lymphangitis of genitalia is a rare cause of self-limited and idiopathic genital lymphoedema in children(4, 6, 9). Most cases of lymphoedema with non-infectious genital granulomas in young patients occur subsequent to or concomitant with Crohn's disease(1, 3, 4, 9). The case described is of a 14-year-old boy with history of genital lymphoedema mainly on the dorsal region of the body of the penis, sparing the prepuce, the distal part of the penis and the scrotum. Laboratory studies did not reveal systemic diseases. The histopathological analysis showed it was non-infectious granulomatous lymphangitis.