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Introdução: O Síndrome de Beckwith-Wiedemann (SBW) é um síndrome com uma prevalência de 0,7: 10 000 nascimentos, caracterizado por um distúrbio do crescimento e com uma variedade de achados clínicos. Mais frequentemente, as crianças com SBW apresentam, gigantismo, com macrossomia pré ou pós-natal, macroglossia, visceromegalia, defeitos da parede abdominal e dismorfia facial. O criptorquidismo e a diminuição da fecundidade estão entre os sinais descritos nestes doentes. O testículo não descido é o distúrbio de diferenciação sexual mais comum nos rapazes, sendo bilateral em apenas 10% dos casos. Não existem casos descritos de testículos impalpáveis bilaterais no SBW. Caso Clínico: E. X., é, atualmente, um adolescente de 17 anos, com diagnóstico pós-natal de SBW, que foi observado pela Cirurgia Pediátrica no primeiro ano de vida por testículos impalpáveis ao nascimento. E.X. foi submetido a exploração inguinal bilateral aos 16 meses. Intra-operatoriamente, à direita, exploração inguinal negativa; à esquerda, exploração inguinal com testículo localizado ao nível do anel inguinal interno, sendo realizada a fixação do testículo na região inguinal com ajuda de rede de silastic. Por rejeição da rede, submetido a orquidectomia esquerda. Aos 8 anos, foi submetido a exploração laparoscópica sendo, nessa altura, observado à direita, testículo em posição muito alta, junto o polo inferior do rim. Aos 16 anos, feito abaixamento do testículo direito para a bolsa escrotal homolateral, pela técnica de Stephens-Fowlers via laparoscópica, com colocação de prótese testicular esquerda no mesmo procedimento. Actualmente segui- do na consulta externa. Comentários: Os testículos não descidos podem ser classificados em palpáveis (na raiz do canal inguinal) e não palpáveis (intra-abdominais, canaliculares ou atróficos). A maioria destes são palpáveis, sendo impalpáveis em apenas 20% dos casos. Destes 40% são atróficos, 40% são canaliculares, e apenas 20% são intra-abdominais. Diferentes técnicas são usadas para o tratamento dos testículos intra-abdominais. O tratamento das crianças com testículos intra-abdominais é ainda um assunto controverso, apesar de vários anos de pesquisa e experiência neste tema. Ainda não existem resultados a longo termo deste procedimento, e quais os seus efeitos na vascularização testicular e fertilidade., NASCER E CRESCER - BIRTH AND GROWTH MEDICAL JOURNAL, Vol 24 (2015): Supplement II: XVII Reunião Anual de Pediatria do CMIN

ResumoA incontinência fecal é uma complicação frequente das crianças com malformação anorretal e espinha bífida, sendo fator perturbador da qualidade de vida e da integração social destas. As atitudes terapêuticas médicas (laxantes, enemas, criação de hábitos defecatórios e alimentares) são frequentemente ineficazes. Das opções terapêuticas cirúrgicas disponíveis, nenhuma foi estabelecida como a forma de abordagem ideal. Relata-se o caso clínico de um adolescente de 17 anos, com incontinência fecal e espinha bfida proposto para realização de cecostomia endoscópica percutânea (CEP). A CEP evidencia-se como uma boa opção para abordagem da incontinência fecal. Apesar de não ser o tratamento ideal para esta condição, alia à segurança e facilidade de execução da técnica o sucesso no controlo da continência e elevado grau de satisfação do doente e familiares.AbstractFecal incontinence is common in children with ano-rectal malformations and spina bifida being a disturbing cause of life quality and social integration. Medical treatments (laxatives, enemas, daily habits and diet attitudes) are frequently unsuccessful. Many surgical therapies have been proposed to treat this condition but all of them are far away from being the best treatment option. We report a case of a 17 years-old adolescent with spina bifida and fecal incontinence that has been submitted to percutaneous endoscopic cecostomy. Even though it is not the ideal treatment for this condition, it revealed to be a good option: it's easy and safe to perform; provides a successful management of fecal continence and it's associated with high satisfaction from the adolescent and its family.

Recently, surgical intervention has gained increasing support in adolescents with extreme obesity. This study summarizes the analysis into the effect of laparoscopic adjustable gastric bands (LAGB) on cardiovascular risk factors in 14 extremely obese Portuguese adolescent patients. Data collected both pre- and postoperatively included age, gender, body mass index (BMI), percentage of excess weight loss, cardiovascular risk factors, and cardiovascular outcomes. Ten girls and four boys aged from 13.5 to 17.5 years underwent LABG. The mean preoperative weight and BMI were 127.4 kg and 46.1 kg/m(2), respectively. The average percentage of weight loss calculated was 32 % at 1 year, 38.8 % at 2 years, and 48.1 % at 3 years of follow-up. Simultaneously, blood pressure and insulin resistance index returned to normal, and there was an increase in high-density lipoprotein levels, 3 years after the LABG was fitted. LABG fitting is a safe and effective treatment strategy for the improvement of cardiovascular status following weight loss.

El hemangioendotelioma hepático infantil (HHI) es una enfermedad grave y con elevada mortalidad. A pesar de ello, estas lesiones vasculares pueden evolucionar espontáneamente para su resolución completa entre 12 y 18 meses. La decisión de intentar un tratamiento específico y la elección entre las varias opciones terapéuticas continúa siendo polémica, especialmente en los casos asintomáticos de afectación multifocal o bilobular.Describimos un caso de HHI, multifocal y bilobular, asintomático, no sometido a ningún tipo de terapia y con regresión de las lesiones antes de los 2 años de edad. : Infantile hepatic hemangioendothelioma is a severe disease with a high mortality rate. Nevertheless these vascular lesions may experience spontaneous regression within 12 to 18 months. The decision of trying a specific treatment and the choice among the several therapeutic options remains controversial, particularly in those asymptomatic cases of multifocal, bilobular involvement.We describe a case of multifocal, bilobular and asymptomatic infantile hepatic hemangioendothelioma, untreated, and with spontaneous regression before 2 years of age.

In Portugal, there is very few experience in pediatric retroperitoneoscopy. The authors present the first Portuguese series of retroperitoneal laparoscopic nephrectomies (RLN) in children younger than nine years-old, as well as a literature review about the theme.Retrospective analysis of clinical charts of all children submitted to RLN between January 2009 and December 2009 in a Pediatric Surgery Department. The literature review was made searching related articles in Medline.Eight RLN were preformed. The medium age of the patients was 4,5 years-old (minimum = 11 months, maximum = 8,6 years). The indications for surgery were: four multicystic kidneys, three reflux nephropathy, and one obstructive nephropathy. Four RLN were preformed on the left side. The medium operative time was 99 minutes (minimum = 50 minutes, maximum = 180 minutes). There was a shortening of operative time as the surgical team got more experienced. There were no conversions to open surgery. Medium hospital stay was 1,5 days (minimum = 1 day, maximum = 2 days). There were no intra-operative and no post-operative complications.RLN is feasible in children younger than nine years-old and should be considered standard treatment in pediatric population.

We present the extremely rare case of a male newborn with Beckwith-Wiedemann Syndrome (BWS) presenting as delayed abdominal wall closure and neonatal intussusception. Fetal ultrasound had shown omphalocele that resolved spontaneously. When feeding was attempted, he had various episodes of vomiting. An x-ray showed signs of high bowel obstruction. Jejunal intussusception was found on laparotomy. Enterectomy and primary jejuno-jejunal anastomosis was performed. During post-operative period subtle physical findings became prominent: plain hemangioma, posterior helical indentations, and macroglossia. Cardiac ultrasonography showed a patent foramen oval with small left-to-right shunt. Ultrasonography showed renal hyperplasia. Genetic study showed hypomethylation of DMR2 region of 11p15 chromosome.

Background: Treatment of varicocoele is aimed at eliminating the retrograde reflux of venous blood through the internal spermatic veins. The purpose of this investigation was to compare laparoscopic varicocoelectomy (LV) with open high ligation technique in the adolescent population. Materials and Methods: We retrospectively evaluated 33 adolescents who underwent varicocoelectomy at our paediatric hospital, between May 2004 and September 2008. Patients were divided into two groups depending on the technique: those who had an LV and those submitted to an open varicocoelectomy (OV). We analysed side, age of surgery, follow-up period and the incidence of recurrence/persistence, hydrocoele formation and wound complication. Results: There were 24 patients in the LV group and 9 in the OV group. All varicocoeles were in the left side. Mean age was 12 years in both groups. Mean follow-up time was 32 months for the LV group and 38 months for the OV group (P = 0.49). There was no significant difference in the incidence of hydrocoele in both the groups (25% versus 22%, P = 0.626). There was no recurrence/persistence on the LV group, while in the OV group there were three cases (P = 0.015). Conclusion: LV seems more efficient than open high ligation technique in the treatment of adolescents' varicocoeles. Larger series are necessary to draw more reliable conclusions.

Objective. To describe a simplified technique already used in our institution for several years in the open heminephrectomy for duplication anomalies, now performed through a retroperitoneal laparoscopic approach.Methods. The technique begins with upper pole parenchyma incision since the demarcation between the affected upper moiety and the healthy lower pole is easily established. The dissection proceeds until the urothelium of the collecting system is entered, which will guide further excision, minimizing damage of the surrounding structures. The vascular supply is then identified since the upper pole is attached to the remaining renal parenchyma only by these structures that can be safely divided. Dissection and division of the ectopic ureter is carried next.Results. The operative time was 188 minutes. The blood loss was not significant, and there were no other complications during the procedure. The patient was discharged home 48 hours after the procedure, without any early or late postoperative complications.Conclusion. We believe this simplified technique allows a safer excision of nonfunctioning upper pole renal tissue by avoiding the initial dissection of the renal hilum, which associated with the known advantages of a laparoscopic approach makes us consider it the procedure of choice for upper pole nephrectomy in children.

Les auteurs presentent deux cas de duplication kystique du rectum qui se presentaient comme des prolapsus. Les deux filles de race caucasienne, âgees de 8 mois et de 12 ans, sont arrivees en urgence pour un prolapsus rectal. Apres que le diagnostic ait pu etre fait, l'excision chirurgicale par voie trans-anale a ete pratiquee. Les suites post-operatoires ont ete rapides et sans complication. L'examen histologique a confirme une duplication kystique du rectum.