Torsion of Granulosa Cell Tumor of the Ovary in a Preschool Patient: A Rare Cause of Acute Abdomen.

BACKGROUND Granulosa cell tumor of the ovary is very rare in childhood; its most common clinical manifestation is isosexual precocious puberty. Clinical presentation as acute abdomen due to pain and ovarian torsion is rare, but a granulosa cell tumor must be suspected in a patient with this acute presentation and signs of early puberty. Adult-type granulosa cell tumor is an even rarer occurrence in children. CASE REPORT We report a case of torsion of adult-type granulosa cell tumor of the ovary in a 5-year-old patient with acute abdominal pain and ovarian torsion and highlight the importance of histological diagnosis of this tumor for the therapeutic plan and progression of these patients. CONCLUSIONS Precocious puberty, pain, abdominal distension, and an ultrasonography with suspicion of ovarian torsion are warning signs that may indicate the presence of a gonadal stromal tumor in pediatric patients seen at an emergency unit. These patients require long-term follow-up by a pediatrician and gynecologist because of the potential for late recurrence.

Torsion of a Communicating Hydrocele Presented as Acute Scrotum: A Case Report and Literature Review.

Torsion of a communicating hydrocele is extremely rare, and the cause is unclear. We report the case of a 3-year-old boy referred to us with acute scrotum. Operative findings revealed torsion of a communicating hydrocele with a 360-degree rotation of the distal end. We performed surgical excision of the necrotic cystic mass and high ligation of the peritoneal communication. A high index of suspicion is required for the correct diagnosis and treatment of this condition, which should be included among the causes of acute scrotum in childhood.

Choledochal cyst in childhood: review of 30 cases.

OBJECTIVE: To analyze and discuss the clinical data, diagnosis and treatment of a number of patients with cystic dilatation of the common bile duct of a Brazilian pediatric hospital. METHODS: We analyzed 30 patients treated at the Martagão Gesteira Institute of Pediatrics and Child Care of the Federal University of Rio de Janeiro for 23 years ,with statistical analysis of epidemiological data, clinical manifestations, diagnosis, treatment and postoperative outcome. RESULTS: We observed a marked female predominance (73.4% of cases), the diagnosis being made in the first decade of life in 90% of patients. The most prevalent clinical manifestation was jaundice (70% of cases) and the classic triad of choledochal cyst was not observed. Abdominal ultrasound was the first imaging examination performed, with a sensitivity of 56.6%, with diagnostic definition in 17 children. Two patients (6.6%) had prenatal diagnosis. All patients underwent surgical treatment, cyst resection with Roux-en-Y hepaticojejunostomy being performed in 80% of cases. The incidence of postoperative complications was 13.3% and the mortality rate was 6.6%, ie two patients were diagnosed with Caroli's disease. CONCLUSION: The non-observance of the classic triad of choledochal cyst suggests that its incidence is lower than that reported in the medical literature. The surgical treatment of choledochal cysts, with resection and bilioenteric anastomosis, is safe even for small children.

Choledochal cyst in childhood: review of 30 cases / Cisto de colédoco na infância: revisão de 30 casos

Objective: To analyze and discuss the clinical data, diagnosis and treatment of a number of patients with cystic dilatation of the common bile duct of a Brazilian pediatric hospital. Methods: We analyzed 30 patients treated at the Martagão Gesteira Institute of Pediatrics and Child Care of the Federal University of Rio de Janeiro for 23 years ,with statistical analysis of epidemiological data, clinical manifestations, diagnosis, treatment and postoperative outcome. Results: We observed a marked female predominance (73.4% of cases), the diagnosis being made in the first decade of life in 90% of patients. The most prevalent clinical manifestation was jaundice (70% of cases) and the classic triad of choledochal cyst was not observed. Abdominal ultrasound was the first imaging examination performed, with a sensitivity of 56.6%, with diagnostic definition in 17 children. Two patients (6.6%) had prenatal diagnosis. All patients underwent surgical treatment, cyst resection with Roux-en-Y hepaticojejunostomy being performed in 80% of cases. The incidence of postoperative complications was 13.3% and the mortality rate was 6.6%, ie two patients were diagnosed with Caroli's disease. Conclusion: The non-observance of the classic triad of choledochal cyst suggests that its incidence is lower than that reported in the medical literature. The surgical treatment of choledochal cysts, with resection and bilioenteric anastomosis, is safe even for small children. .

Objetivo: analisar e discutir os dados clínicos, o diagnóstico e tratamento de uma série de pacientes portadores de dilatações císticas do colédoco de um hospital pediátrico brasileiro. Métodos: foram analisados 30 pacientes tratados no Instituto de Pediatria e Puericultura Martagão Gesteira da Universidade Federal do Rio de Janeiro durante 23 anos, com análise estatística de dados epidemiológicos, manifestações clínicas, diagnóstico, tratamento e evolução pós-operatória. Resultados: foi observada marcada predominância do sexo feminino (73,4% dos casos), sendo o diagnóstico feito na primeira década de vida em 90% dos pacientes. A manifestação clínica mais prevalente foi a icterícia (70% dos casos) e a tríade clássica do cisto de colédoco não foi observada. A ultrassonografia abdominal foi o primeiro exame de imagem realizado, demonstrando sensibilidade de 56,6%, com definição diagnóstica em 17 crianças. Dois pacientes (6,6%) tiveram diagnóstico pré-natal. Todos os pacientes foram submetidos a tratamento cirúrgico, sendo a ressecção do cisto com hepaticojejunostomia com Y de Roux realizada em 80% dos casos. A incidência de complicações pós-operatórias foi 13,3% e a taxa de mortalidade foi 6,6%, ou seja, dois pacientes com diagnóstico de doença de Caroli. Conclusão: a não observação da tríade clássica do cisto de colédoco nos pacientes avaliados sugere que a sua incidência seja menor que a relatada na literatura médica mundial. O tratamento cirúrgico dos cistos de colédoco, com sua ressecção e anastomose bíleodigestiva é seguro, mesmo em crianças pequenas. .

Morphologic study of embryonic development of rat duodenum through a computerized three-dimensional reconstruction: critical analysis of solid core theory.

To date, intrinsic obstructions of the duodenum have been explained by the "solid core" theory, described by Tandler in 1902 (Morphol Jahrb 29:187-216, 1902). This study aimed to evaluate the epithelial occlusion of rat duodenum during embryonic development, through optical microscopy and computerized three-dimensional reconstruction. The Wistar rat embryos used in this study had 13, 14, 15, and 16 days of gestation. This corresponds to human embryos with 33, 40, 44, and 52 days of development, which is between the fifth and eighth week. The study included 12 embryos studied by optical microscopy, and four by three-dimensional reconstruction (those with 13, 14, 15, and 16 days). Through optical microscopy, an intense epithelial proliferation was observed in the gestation embryo of 13 days, with no occlusion of the opening of the duodenum. In the embryos with 14, 15, and 16 days of gestation, an increase in diameter of the duodenum was observed along with intestinal development. Through three-dimensional reconstruction, it was observed that the opening of the digestive tube of rat embryos with 13-16 days of gestation is never obstructed by epithelial proliferation, although it may follow a sinuous path. This study concludes that the "solid core" phase described by Tandler does not occur in the embryonic development of rat duodenum.

Hepatobiliary effects of cholic and lithocholic acids: experimental study in hamsters.

Etiopathogenesis of biliary atresia remains unknown. Among several theories, one proposes that the disorder may be caused by the toxic effect of monohydroxy bile acids on fetal and neonatal hepatobiliary system. In this paper we evaluated toxic effects produced by ingestion of cholic acid, a trihydroxy bile acid, and lithocholic acid, a monohydroxy bile acid in the hepatobiliary system of a hamster during gestational and perinatal periods. A diet composed by 0.5% cholic acid and 0.25% lithocholic acid was administrated to pregnant hamsters. Liver and bile ducts of the adult and newborn animals were analyzed to point out the changes induced by these acids after birth. Because hamsters and humans have a similar bile metabolism, these animals were eligible for the study. The ingestion of 0.5% lithocholic acid, during hamster's gestation, caused maternal intense ductal/ductular proliferation, inflammatory signs, hepatic cells degeneration and regeneration, hyperplasia of extra hepatic ducts epithelium, and abortion. Both 0.5% cholic acid and 0.25% lithocholic acid ingested by pregnant hamsters, caused ductal/ductular proliferation and hepatobiliary inflammatory damage in a different degree of intensity in adult animals and mild intensity in the young; and also the number of the young was reduced in the litter. We found that the ingestion of these bile acids by hamsters, during gestational period caused different degrees of toxicity on maternal and neonatal hepatobiliary systems. The histopathologic findings observed in biliary atresia patients could not be found in newborn hamsters. New experimental models are needed in the attempt to establish a correlation of these acids with neonatal cholestatic diseases.