Prevalence and surgical management of pubic hypertrophy in hypospadias patients: results from a high-volume surgeon

ABSTRACT Introduction: Pubic hypertrophy, defined as an abnormal and abundant round mass of fatty tissue located over the pubic symphysis, is frequently underestimated in patients with hypospadias. We examined the prevalence of this condition, as well as the outcomes associated with its surgical treatment. Material and methods: Within 266 hypospadias patients treated at our clinic, we assessed the prevalence of pubic hypertrophy, and we schematically described the surgical steps of pubic lipectomy. Multivariable logistic regression (MLR) tested for predictors of pubic hypertrophy. Finally, separate MLRs tested for predictors of fistula and any complications after pubic lipectomy. Results: Of 266 hypospadias patients, 100 (37.6%) presented pubic hypertrophy and underwent pubic lipectomy. Patients with pubic hypertrophy more frequently had proximal hypospadias (44 vs. 7.8%), disorders of sex development (DSD) (10 vs. 0.6%), cryptorchidism (12 vs. 2.4%), and moderate (30°-60°) or severe (>60°) penile curvature (33 vs. 4.2%). In MLR, the location of urethral meatus (proximal, Odds ratio [OR]: 10.1, p<0.001) was the only significant predictor of pubic hypertrophy. Finally, pubic lipectomy was not associated with increased risk of fistula (OR: 1.12, p=0.7) or any complications (OR: 1.37, 95% CI: 0.64-2.88, p=0.4) after multivariable adjustment. Conclusions: One out of three hypospadias patients, referred to our center, presented pubic hypertrophy and received pubic lipectomy. This rate was higher in patients with proximal hypospadias suggesting a correlation between pubic hypertrophy and severity of hypospadias. Noteworthy, pubic lipectomy was not associated with increased risk of fistula or any complications.

Proteus syndrome: Clinical profile of six patients and review of literature.

OBJECTIVE: Proteus syndrome (PS) is characterized by patchy or segmental overgrowth and hyperplasia of multiple tissues and organs, along with susceptibility to development of tumors. Very few cases are reported in literature from developing countries. Due to certain overlapping features with other overgrowth syndromes, diagnosis is usually delayed. Our aim was to describe clinical profile of this rare condition in six patients. MATERIALS AND METHODS: Retrospective case sheet review of patients followed in a Pediatric Genetic and Metabolic clinic at a tertiary care institute of North India with a diagnosis of hemihypertrophy/overgrowth syndrome. RESULTS: Six cases presented with asymmetric overgrowth and peculiar features suggestive of PS were included in this study. Age at presentation was 2 months to 10 years; two were males and four were females. Hemihypertrophy was noticed in only one case at birth, and focal overgrowths in rest of other patients were seen later during childhood. CONCLUSION: Due to certain overlapping features with other overgrowth syndromes, diagnosis of PS is usually delayed. Pediatricians are the first persons who come across such patients and they should be aware about this rare condition.

Associação entre hipertrofia adenotonsilar, tonsilites e crises álgicas na anemia falciforme / Association between adenotonsillar hypertrophy, tonsillitis and painful crises in sickle cell disease

OBJETIVOS: Determinar a prevalência da hipertrofia adenotonsilar obstrutiva em crianças e adolescentes portadores de anemia falciforme; investigar possível associação entre presença de mais de cinco episódios de tonsilite nos últimos 12 meses e episódios de crise álgica no mesmo período; e comparar a hemoglobina anual média entre os que apresentam e os que não apresentam hipertrofia adenotonsilar obstrutiva. MÉTODOS: Trata-se de estudo prospectivo, observacional do tipo corte transversal, com 85 crianças e adolescentes com anemia falciforme. Todos responderam questionário e avaliação otorrinolaringológica, incluindo endoscopia nasossinusal. Para o diagnóstico da hipertrofia adenotonsilar obstrutiva foram adotados os critérios de Brodsky. RESULTADOS: A prevalência da hipertrofia adenotonsilar obstrutiva foi de 55,3 por cento. A hipertrofia adenotonsilar obstrutiva associou-se à história de dificuldade para alimentar-se (76,7 versus 23,5 por cento; p = 0,003), presença de mais de cinco episódios de tonsilites nos últimos 12 meses (70,6 versus 29,4 por cento; p = 0,021), roncar alto (73,0 versus 27,0 por cento; p = 0,004) e apneia do sono assistida (71,8 versus 28,2 por cento; p = 0,005). Portadores de hipertrofia adenotonsilar obstrutiva apresentaram maior número de infecções das vias aéreas superiores (62,5 versus 37,5; p = 0,010). Também foi observada associação entre presença de mais de cinco episódios de tonsilite nos últimos 12 meses e episódios de crise álgica no mesmo período (mediana = 12 versus 2; p = 0,017). Não houve diferença significante da hemoglobina anual média entre portadores de hipertrofia adenotonsilar obstrutiva versus hipertrofia adenotonsilar não-obstrutiva (7,6 versus 8,2 g/dL; p = 0,199). CONCLUSÃO: A prevalência da hipertrofia adenotonsilar obstrutiva foi de 55,3 por cento em crianças e adolescentes com anemia falciforme. A presença de mais de cinco episódios de tonsilite nos últimos 12 meses associaram-se com...

OBJECTIVES: To determine the prevalence of obstructive adenotonsillar hypertrophy in children and adolescents with sickle cell anemia; to investigate possible association between the presence of more than five episodes of tonsillitis in the last 12 months and episodes of painful crises in the same period; and to compare the mean annual hemoglobin level in children and adolescents with and without obstructive adenotonsillar hypertrophy. METHODS: Prospective, observational, cross-sectional study involving 85 children and adolescents with sickle cell anemia. All patients answered a questionnaire and underwent a standard otolaryngology examination, including endoscopic endonasal approach. The diagnosis of obstructive adenotonsillar hypertrophy was made according to the Brodsky scale. RESULTS: The prevalence of obstructive adenotonsillar hypertrophy was 55.3 percent. Obstructive adenotonsillar hypertrophy was associated with history of difficulty in eating (76.7 vs. 23.5 percent, p = 0.003), presence of more than five episodes of tonsillitis in the last 12 months (70.6 vs. 29.4 percent, p = 0.021), loud snoring (73.0 vs. 27.0 percent, p = 0.004), and sleep apnea (71.8 vs. 28.2 percent, p = 0.005). Patients with obstructive adenotonsillar hypertrophy had more episodes of recurrent upper airway tract infection (62.5 vs. 37.5; p = 0.010). The presence of more than five episodes of tonsillitis in the last 12 months was associated with episodes of painful crises (median = 12 vs. 2, p = 0.017). There was no significant difference between mean annual hemoglobin levels of patients with obstructive adenotonsilar hypertrophy vs. nonobstructive adenotonsillar hypertrophy: 7.6 vs. 8.2 g/dL, p = 0.199. CONCLUSION: The prevalence of obstructive adenotonsillar hypertrophy was 55.3 percent in children and adolescents with sickle cell anemia; the presence of more than five episodes of tonsillitis in the last 12 months was associated with episodes of painful...

Estenosis hipertrófica congenita del piloro: experiencia en el Hospital cayetano Heredia abril 1969 febrero 1991 / Congenital hipertrofic piloric stenosis: experience at the Cayetano Heredia Hosptal from april 1969 to february 1991

Este trabajo se realizó en el Servicios de Cirugía Pediatrica, Hospital Cayetano Heredia, Lima. Se revisaron Historias Clínicas de abril 1969 a febrero 1991 con Diagnóstico de Estenosis Hipertrófica de Píloro (EHCP), 76 cumplieron los criterios de selección (paciente pediátrico y confirmación diagnóstica post-operatoria). El objetivo fue realizar un estudio sobre EHCP donde se resaltan los hallazgos clínicos de esta patología en un Hospital General; se muestra el porcentaje en lo referente a la palpación de la Oliva Pilorica, estableciendo asi el diagnóstico definitivo; la evaluación clínica, el tratamiento quirúrgico, la morbi-mortalidad. La edad promedio al ingreso fue de 39.11 días; relación varón/mujer: 4.4/1 (p menor 0.01); siendo primogénitos 43.42 por ciento. El 100 por ciento de los pacientes iniciaron con vómitos post prandiales, a la edad promedio de 23.73 días. Estreñimiento en 52.63 por ciento, avidez por alimento en 40.79 por ciento. La oliva pilórica se palpó en el 93.42 por ciento de todos los casos, estableciendose así el diagnóstico clínico definitivo. La palpación fue negativa en 5 pacientes siendo su diagnóstico definitivo radiológico. La anomalía congénita asociada más frecuente fue la hernia inguinal (6.58 por ciento). En todos se realizó piloromiotomía de Fredet-Ramstedt. 22.36 por ciento de los pacientes tuvieron por lo menos una complicación; siendo la mas frecuente la infección de la hernia operatoria (5.26 por ciento), hipotermia (5.26 por ciento) y la perforación iatrogénica de la mucosa pilórica (3.95 por ciento). En el 48.68 por ciento no existió vómitos post-prandiales, y la mortalidad fue 1.32 por ciento (1 de 76 pacientes)

Hipertrofia parcial congenita: descripcion de cinco casos con compromiso de MMII / Congenital partial hypertrophy: description of five cases with compromise of lower limbs

Se describen los hallazgos clinicos de cinco pacientes con hipertrofia parcial congenita, tipo segmentario. Se realiza una revision de la literatura y se establecen comparaciones con los resultados obtenidos en el presente estudio