ABSTRACT
McKittrick-Wheelock syndrome is caused by chronic water and electrolyte hypersecretion from an intestinal tumor, usually a villous adenoma, located in the rectum or sigmoid. Patients often have dehydration, hypovolemic shock and kidney failure associated with hypokalemia, hyponatremia, hypochloremia and metabolic acidosis. We report a 62-year-old male, suffering chronic diarrhea for eight years who was admitted after a syncope. He had severe hypokalemia, hyponatremia, metabolic acidosis, hypovolemia and acute renal failure. After his metabolic disorders were corrected, a colonoscopy showed a large rectosigmoid tumor with the characteristics of a villous adenoma. During the follow up after the complete tumor resection, the patient has remained asymptomatic.
Subject(s)
Humans , Male , Middle Aged , Uterine Diseases/diagnosis , Abnormalities, Multiple/diagnosis , Polydactyly/diagnosis , Coronary Disease/diagnosis , Hydrocolpos/diagnosis , Heart Defects, Congenital/diagnosis , Uterine Diseases/surgery , Abnormalities, Multiple/surgery , Polydactyly/surgery , Diagnosis, Differential , Hydrocolpos/surgery , Heart Defects, Congenital/surgeryABSTRACT
INTRODUCCIÓN Y OBJETIVOS: El hidro y/o hematocolpos es una condición patológica rara, de incidencia 1/16.000 niñas, que consiste en la dilatación de vagina y/o útero por material líquido o hemático. Sus diferentes formas de presentación y especialmente al grupo etario que afecta, puede ser un diagnostico no siempre fácil como también un manejo inadecuado puede determinar diferentes complicaciones de diversa gravedad. El objetivo de este trabajo es analizar los casos tratados en el servicio de urología pediátrica de acuerdo al grupo etario que correspondió su debut clínico. Material y métodos: Estudio retrospectivo de pacientes con diagnóstico de hidro o hematocolpos entre los años 2007 y 2011. Se analizan datos demográficos, patologías asociadas, tratamiento y complicaciones. Resultados: Se analizaron 8 niñas, todas con ambigüedad genital al nacer, 7 con hiperplasia suprarrenal congénita. En 6 pacientes se realiza examen endoscópico bajo anestesia general que confirma la presencia de un seno urogenital (SUG) a una edad promedio de 36 meses (r 3m-12a). La edad promedio de presentación del hidrocolpos fue de 6 años (r 1m - 14 a) con un peak entre los 0-2 años y a los 11-14 años, diagnóstico confirmado mediante ecografía. Otras patologías asociadas fueron: cardiopatía congénita (1) e ITU recurrente (5). Una paciente presentó una sobreinfección del hidrocolpos con shock séptico asociado. En dos pacientes (11 y 14 años) se ha realizado el descenso en bloque, 3 pacientes se drena el hidrocolpos por vía endoscópica (cistoscopía). Conclusiones: El hidrocolpos es una patología rara, pero que se debe tener presente especialmente en diagnostico prenatal y/o recién nacidos que se estudian por masa pelviana y/o abdominal, en pacientes con SUG; ya que su detección precoz y tratamiento pueden evitar consecuencias graves como shock séptico de origen uroginecológico.
INTRODUCTION AND OBJECTIVES: hydro and / or hematocolpos are a rare pathological condition, incidence 1/16.000 girls, which involves dilation of vagina and / or uterus with hematic fluid or material. Due to its different forms of presentation and especially the affected age group, diagnosis may not always be easy as improper Management can determine different complications of varying severity. The aim of this paper is to analyze the cases treated in our pediatric urology service according to age group of clinical debut. Material and methods: Retrospective study of patients diagnosed with hydro or hematocolpos between 2007 and 2011. Demographics, co morbidities, treatment and complications were analyzed. Results: 8 girls, all with ambiguous genitalia at birth, 7 with congenital adrenal hyperplasia were analyzed. In 6 patients endoscopic examination performed under general anesthesia confirmed the presence of a urogenital sinus (SUG) at an average age of 36 months (r 3m-12a). The average age of hydrocolpos presentation was 6 years (r 1m - 14) with a peak between 0-2 years and 11-14 years, confirmed by ultrasound diagnosis. Other associated diseases were: congenital heart disease (1) and recurrent UTI (5). One patient had a superinfection of hydrocolpos associated with septic shock. In two patients (11 and 14y.o.) an en bloc descent was performed, in 3 patients the hydrocolpos was drained endoscopically (cystoscopy). Conclusiones: hydrocolpos is a rare condition, but it must be especially considered in prenatal diagnosis and / or infants studied by pelvic and / or abdominal mass, in patients with SUG, as early detection and treatment can prevent serious consequences as septic shock of urogynecological origin.
Subject(s)
Humans , Female , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Hydrocolpos/surgery , Hydrocolpos/epidemiology , Age Distribution , Age of Onset , Retrospective Studies , Hydrocolpos/complicationsABSTRACT
Os autores relatam um caso de hímen imperfurado congênito determinando volumoso hidrocolpo em recém-nascido. Esta anomalia é uma condição rara, pois a maioria dos casos de hímen imperfurado é diagnosticada na fase pré-púbere com o atraso da menarca. O objetivo deste relato é demonstrar os achados ultrassonográficos do hidrocolpo no recém-nato e realizar uma breve revisão de literatura sobre o assunto. É de fundamental importância nestes pacientes o rastreamento de outras malformações congênitas que podem estar associadas ao quadro.
The authors report a case of newborn hydrocolpos secondary to congenital imperforate hymen. This anomaly is a rare condition, since most cases of imperforate hymen are diagnosed in the prepubertal period with the delay of menarche. The objective of this report is to demonstrate ultrasound findings of newborn hydrocolpos and make a review of literature about it. It is vital to search other congenital malformations that may be associated.