José Carlos Soares de Fraga

Urethral Lengthening with Anterior Bladder Wall Flap for Urinary Incontinence: A New Approach

Urethral irregularity (impeding catheterization) and failure to achieve continence are common complications in surgery for urinary incontinence. We describe a surgical technique using an anterior bladder wall flap that is sutured to the posterior wall in an onlay fashion creating a flap valve mechanism. Experimental work in dogs demonstrated a significant increase in the leak point pressure in the surgical group when compared to controls (p = 0.019). Voiding cystourethrography and bladder inspection demonstrated an anterior flap valve with no fistula formation in all animals. Histological examination showed a viable anterior bladder wall flap in all cases. This technique was then applied to 6 patients with neurogenic bladder and low urethral resistance that failed to resolve with medical treatment. Urinary continence was achieved in 4 patients. In 1 patient a vesicourethral fistula developed 3 months postoperatively, since the mother failed to catheterize for 12 hours. This technique is a useful alternative in the treatment of urinary incontinence.

Diagnosis, misdiagnosis, and associated diseases of achalasia in children and adolescents: a twelve-year single center experience.

PurposeAlthough achalasia is a rare disorder in children, its symptom may mimic common childhood diseases. This study aimed to assess the diagnosis and management of achalasia in children and adolescents in a Brazilian single center during a 12-year period.MethodsPatients with achalasia were identified from a database built during the period of January 2000–January 2012 from a Pediatric Gastroenterology reference center. Information regarding demographic data, clinical symptoms, diagnosis, treatment, and long-term follow-up were described.ResultsThirteen patients were studied; median age was 7 (1–14)xa0years. Most frequent symptoms were vomiting (84.6xa0%) and dysphagia (69.2xa0%). Weight loss occurred in 46.0xa0% of patients and chronic cough in 46.1xa0%. Associated disorders were Down’s syndrome, Allgrove syndrome, and congenital central hypoventilation syndrome. Achalasia was misdiagnosed with anorexia nervosa. Six patients were previously treated as having gastroesophageal reflux disease and asthma. Five patients had pneumatic balloon dilation as initial therapy whereas five had esophageal myotomy. Finally, 11 patients had surgical therapy with a favorable follow-up.ConclusionAchalasia symptoms may mimic common diseases in children, and therefore, may delay the diagnosis. This study emphasizes the importance of the clinical symptoms for the diagnosis of achalasia, mainly in those cases with associated disorders.

The association between laryngeal cleft and tracheoesophageal fistula: Myth or reality?

Laryngeal cleft (LC) associated with tracheoesophageal fistula (TEF) with or without esophageal atresia (EA) has rarely been described. The purpose of this study is to review our experience, clinical features, management, delay in diagnosis, and complications in children with these anomalies.

Terapia de reposição enzimática para as mucopolissacaridoses I, II e VI: recomendações de um grupo de especialistas brasileiros

Mucopolysaccharidoses (MPS) are rare genetic diseases caused by deficiency of specific lysosomal enzymes that affect catabolism of glycosaminoglycans (GAG). Accumulation of GAG in various organs and tissues in MPS patients results in a series of signs and symptoms, producing a multisystemic condition affecting bones and joints, the respiratory and cardiovascular systems and many other organs and tissues, including in some cases, cognitive performance. So far, eleven enzyme defects that cause seven different types of MPS have been identified. Before introduction of therapies to restore deficient enzyme activity, treatment of MPS focused primarily on prevention and care of complications, still a very important aspect in the management of these patients. In the 80s treatment of MPS with bone marrow transplantation/hematopoietic stem cells transplantation (BMT/HSCT) was proposed and in the 90s, enzyme replacement therapy (ERT),began to be developed and was approved for clinical use in MPS I, II and VI in the first decade of the 21st century. The authors of this paper are convinced that a better future for patients affected by mucopolysaccharidoses depends upon identifying, understanding and appropriately managing the multisystemic manifestations of these diseases. This includes the provision of support measures (which should be part of regular multidisciplinary care of these patients) and of specific therapies. Although inhibition of synthesis of GAG and the recovery of enzyme activity with small molecules also may play a role in the management of MPS, the breakthrough is the currently available intravenous ERT. ERT radically changed the setting for treatment of mucopolysaccharidosis I, II and VI in the last decade., Benefits can even be extended soon to MPS IV A (ERT for this condition is already in clinical development), with prediction for treatment of MPS III A and the cognitive deficit in MPS II by administration of the enzyme directly into the central nervous system (CNS). A large number of Brazilian services, from all regions of the country, already have experience with ERT for MPS I, II and VI. This experience was gained not only by treating patients but also with the participation of some groups in clinical trials involving ERT for these conditions. Summing up the three types of MPS, more than 250 patients have already been treated with ERT in Brazil. The experience of professionals coupled to the data available in international literature, allowed us to elaborate this document, produced with the goal of bringing together and harmonize the information available for the treatment of these severe and progressive diseases, which, fortunately, are now treatable, a situation which bring new perspectives for Brazilian patients, affected by these conditions.As mucopolissacaridoses (MPS) sao doencas geneticas raras causadas pela deficiencia de enzimas lisossomicas especificas que afetam o catabolismo de glicosaminoglicanos (GAG). O acumulo de GAG em varios orgaos e tecidos nos pacientes afetados pelas MPS resulta em uma serie de sinais e sintomas, integrantes de um quadro clinico multissistemico que compromete ossos e articulacoes, vias respiratorias, sistema cardiovascular e muitos outros orgaos e tecidos, incluindo, em alguns casos, as funcoes cognitivas. Ja foram identificados 11 defeitos enzimaticos que causam sete tipos diferentes de MPS. Antes do advento de terapias dirigidas para a restauracao da atividade da enzima deficiente, o tratamento das MPS tinha como principal foco a prevencao e o cuidado das complicacoes, aspecto ainda bastante importante no manejo desses pacientes. Na decada de 80 foi proposto o tratamento das MPS com transplante de medula ossea/transplante de celulas tronco hematopoieticas (TMO/TCTH) e na decada de 90 comecou o desenvolvimento da Terapia de Reposicao Enzimatica (TRE), que se tornou uma realidade aprovada para uso clinico nas MPS I, II e VI na primeira decada do seculo 21. Os autores deste trabalho tem a conviccao de que um melhor futuro para os pacientes afetados pelas MPS depende da identificacao, compreensao e manejo adequado das manifestacoes multissistemicas dessas doencas, incluindo medidas de suporte (que devem fazer parte da assistencia multidisciplinar regular destes pacientes) e terapias especificas. Embora a inibicao da sintese de GAG e o resgate da atividade enzimatica com moleculas pequenas tambem possam vir a ter um papel no manejo das MPS, o grande avanco disponivel no momento e a TRE intravenosa. A TRE permitiu modificar radicalmente o panorama do tratamento das mucopolissacaridoses I, II e VI na ultima decada, sendo que ainda pode estender seus beneficios em breve para a MPS IV A (cuja TRE ja esta em desenvolvimento clinico), com perspectivas para o tratamento da MPS III A e do deficit cognitivo na MPS II atraves de administracao da enzima diretamente no sistema nervoso central (SNC). Um grande numero de centros brasileiros, incluindo servicos de todas as regioes do pais, ja tem experiencia com TRE para MPS I, II e VI. Essa experiencia foi adquirida nao so com o tratamento de pacientes como tambem com a participacao de alguns grupos em ensaios clinicos envolvendo TRE para essas condicoes. Somados os tres tipos de MPS, mais de 250 pacientes ja foram tratados com TRE em nosso pais. A experiencia dos profissionais brasileiros, somada aos dados disponiveis na literatura internacional, permitiu elaborar este documento, produzido com o objetivo de reunir e harmonizar as informacoes disponiveis sobre o tratamento destas doencas graves e progressivas, mas que, felizmente, sao hoje trataveis, uma realidade que traz novas perspectivas para os pacientes brasileiros afetados por essas condicoes.

Bronchoscopic removal of foreign body from airway through tracheotomy or tracheostomy

OBJECTIVEnMost foreign bodies in the airway are removed by respiratory endoscopy. Rarely, the removal of the foreign body has to be performed through endoscopic control by tracheotomy or tracheostomy. This article reports three cases of foreign body removal in children performed by tracheal opening.nnnDESCRIPTIONnRetrospective review of records with report of three cases of children who aspirated foreign bodies into the airway. In the first case, there was rupture of the tracheostomy tube, with aspiration of its distal portion. Endoscopic removal was performed by tracheostomy. The second child aspirated a pen cap. It could not be removed by endoscopy because it would not pass through the subglottic region. Cervical tracheotomy was performed and the foreign body was removed with endoscopic control. In the last case, the foreign body was in the left main bronchus. It was removed by bronchoscopy through tracheostomy opening. All children presented good outcome after the endoscopic procedure. The trachea of the patient submitted to tracheotomy was sutured after the foreign body removal. Tracheostomy was not necessary. In the children with previous tracheostomy, the tube was put back after the foreign body removal.nnnCOMMENTSnMost foreign bodies in the airway of children can be removed by endoscopy. When the foreign body is too large to pass through the subglottic region, or so sharp that it can injure the airway, the use of tracheotomy or tracheostomy is indicated.

Abordagem cirúrgica da efusão pleural parapneumônica e suas complicações

Objective: surgical treatment of parapneumonic pleural effusion in children is controversial. The type of intervention is based mainly on personal experience and on the small number of reported cases. This article aims at presenting a literature review and the authors experience in the surgical management of parapneumonic pleural effusion in children. Sources: data were searched in the Medline and Lilacs databases. Summary of the findings: complicated parapneumonic effusion should be surgically drained if thoracentesis reveals the presence of pus, positive Gram stain or culture, or pH less than 7.0 or glucose less than 40 mg/dl. The surgical drainage depends on the stage of parapneumonic pleural effusion: at the acute stage closed thoracostomy drainage is enough; at the fibrinopurulent stage thoracoscopy is indicated; at organizational stage thoracotomy can be performed in children with stable anesthetic conditions, and open thoracostomy drainage should be used in patients in a poor state of health. Echography is very important to evaluate the staging of parapneumonic effusion. Conclusions: surgical treatment of complicated parapneumonic effusion should be done as early as possible, and the kind of procedure depends on the stage of pleural effusion. In children with complicated parapneumonic effusion echography is very important to evaluate the staging of parapneumonic effusion.

Toracoscopia em crianças com derrame pleural parapneumônico complicado na fase fibrinopurulenta: estudo multi-institucional

OBJECTIVE:To determine the efficacy of thoracoscopy in the management of children with complicated parapneumonic pleural effusion at the fibrinopurulent stage. METHODS: Retrospective study of 99 children submitted to thoracoscopy for the treatment of complicated parapneumonic pleural effusion at the fibrinopurulent stage between November of 1995 and July of 2005. The mean age was 2.6 years (range, 0.4-12 years), and 60% were males. Thoracoscopy was performed at three different hospitals following the same treatment algorithm. RESULTS: Thoracoscopy was effective for 87 children (88%). In 12 (12%), a second surgical procedure was required: another thoracoscopy (n = 6) or thoracotomy/thoracostomy (n = 6). Mean duration of chest tube drainage following successful thoracoscopy was 3 days vs. 10 days in patients submitted to a second procedure (p < 0.001). In all of the children, the pleural infection resolved after treatment. Thoracoscopy-related complications included air leak (30%), chest tube bleeding (12%), subcutaneous emphysema associated with trocar insertion (2%) and surgical wound infection (2%). None of the children required additional surgical procedures due to the complications. CONCLUSIONS: The effectiveness of thoracoscopy in children with parapneumonic pleural effusion at the fibrinopurulent stage was 88%. The procedure was safe, with a low rate of severe complications. Thoracoscopy should be the first-choice treatment for children with parapneumonic pleural effusion at the fibrinopurulent stage.

Impact of antibiotic therapy on laboratory analysis of parapneumonic pleural fluid in children.

OBJECTIVEnThe therapeutic management of parapneumonic pleural effusions (PPE) is controversial in children. Decision-making often relies on parameters such as gross appearance of pleural fluid and on bacteriologic and biochemical analyses. Our goal was to describe the laboratory profile of PPE in children and to assess the influence of previous administration of antibacterial agents on culture and biochemical results.nnnPATIENTS AND METHODSnThis was a prospective study including children (age, 1 month to 16 years) with a diagnosis of PPE. Two groups were evaluated: children with or without antibiotic treatment up to 48 hours before analysis of pleural fluid. Results were analyzed using the χ(2) or Mann-Whitney test (α = .05). Odds ratio and 95% confidence intervals (95% CIs) were calculated, with control of previous antibiotic therapy using multivariate logistic regression analysis, to determine the risk of empyema associated with specific biochemical parameters.nnnRESULTSnOne hundred ten children were selected. Fifty percent had received antibiotics at least 48 hours before pleural fluid analysis. Differences were observed between the groups in terms of PPE gross appearance (P = .033) and identification of bacteriologic agent by culture or Gram stain (P = .023). Biochemical parameters (pH ≤7.1 and glucose ≤40 mg/dL) were associated with increased odds of receiving a more invasive treatment. For pH, the odds ratio was 9.614 (95% CI, 1.952-47.362; P = .005); and for glucose, 9.201 (95% CI, 1.333-63.496; P = .024).nnnCONCLUSIONSnPrevious use of antibacterial agents affected the bacteriologic analysis of pleural fluid in this pediatric sample admitted for PPE. However, it did not interfere significantly with biochemical parameters of pleural fluid.

Congenital Rupture of Scrotum: An Unusual Complication of Meconium Peritonitis

Congenital rupture of the scrotum caused by meconium peritonitis occurred in a newborn with clinical findings of the Beckwith-Wiedemann syndrome. Ruptured omphalocele and jejunal atresia were present. Surgical repair 3 hours after birth was successful.

Diagnosis and management of congenital rectourethral fistula in a child with long tubular duplication of the colon and Klippel-Feil syndrome

We describe a unique association of congenital rectourethral fistula with long tubular duplication of the colon in a boy with Klippel-Feil syndrome and Sprengel deformity. He presented with a rectourethral fistula after surgical repair of a tubular duplication of the terminal ileum, colon, and proximal rectum. Preoperative identification of the fistula was challenging and was only achieved after cystoscopy with injection of methylene blue under pressure through Foley catheters placed into the anus and distal stoma of a colostomy. Surgical repair was performed through the posterior sagittal approach. The patient is doing well after 4 years of follow-up.