Artificial urinary sphincter for urinary incontinence after radical prostatectomy: a historical cohort from 2004 to 2015

ABSTRACT This study aimed to retrospectively evaluate a cohort of patients with prostate cancer and persistent urinary incontinence after radical prostatectomy. From January 2004 to December 2015, eighty-six individuals were identified to have received an AUS implant, provided by a private nonprofit HMO operating in Belo Horizonte, Brazil. On total, there were 91 AUS implants, with a median interval between radical prostatectomy and AUS implant of 3.6 years (IQR 1.9 to 5.5). The rate of AUS cumulative survival, after a median follow-up of 4.1 years (IQR 1.7-7.2 years), was 44% (n=40). The median survival of AUS implants was 2.9 years (IQR 0.5-7.9 years). Thirty-seven AUS implants (40.7%) resulted in grade III surgical complications. There were 5 deaths at 2.1, 4.7, 5.7, 5.7 and 6.5 years of follow-up, but none due to causes directly associated to the AUS implant. Persistent severe incontinence was documented in 14 (15.3%) additional patients. From the 51 AUS implants which resulted in grade III surgical complications or persistent severe incontinence, 24 (47.1%) underwent surgical revisions. Explantation of the sphincter or its components was observed in 6 cases (25.0%). Mechanical failure, described as fluid loss and/or inability to recycle the AUS device, was observed in 4 devices (16.7%). In conclusion, although AUS implants are recommended as the gold-standard treatment of severe urinary incontinence after prostatectomy, the observed high rates of malfunction and grade III adverse events are a matter of concern warranting further assessment on the safety and efficacy of these devices.

Neurofibromatosis: part 2 – clinical management / Neurofibromatoses: parte 2 – manejo clínico

Part 1 of this guideline addressed the differential diagnosis of the neurofibromatoses (NF): neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2) and schwannomatosis (SCH). NF shares some features such as the genetic origin of the neural tumors and cutaneous manifestations, and affects nearly 80 thousand Brazilians. Increasing scientific knowledge on NF has allowed better clinical management and reduced rate of complications and morbidity, resulting in higher quality of life for NF patients. Most medical doctors are able to perform NF diagnosis, but the wide range of clinical manifestations and the inability to predict the onset or severity of new features, consequences, or complications make NF management a real clinical challenge, requiring the support of different specialists for proper treatment and genetic counseling, especially in NF2 and SCH. The present text suggests guidelines for the clinical management of NF, with emphasis on NF1.

A primeira parte desta diretriz abordou o diagnóstico diferencial das neurofibromatoses (NF): neurofibromatose do tipo 1 (NF1), neurofibromatose do tipo 2 (NF2) e schwannomatose (SCH). As NF compartilham algumas características, como a origem neural dos tumores e sinais cutâneos, e afetam cerca de 80 mil brasileiros. O aumento do conhecimento científico sobre as NF tem permitido melhor manejo clínico e redução da morbidade das complicações, resultando em melhor qualidade de vida para os pacientes com NF. A maioria dos médicos é capaz de realizar o diagnóstico das NF, mas a variedade de manifestações clínicas e a dificuldade de se prever o surgimento e a gravidade de complicações, torna o manejo da NF um desafio para o clínico e envolve diferentes especialistas para o tratamento adequado e aconselhamento genético, especialmente a NF2 e a SCH. O presente texto sugere algumas orientações para o acompanhamento dos portadores de NF, com ênfase na NF1.

Neurofibromatoses: part 1 diagnosis and differential diagnosis / Das neurofibromatoses: parte 1 diagnostico e diagnostico diferencial

Neurofibromatoses (NF) are a group of genetic multiple tumor growing predisposition diseases: neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2) and schwannomatosis (SCH), which have in common the neural origin of tumors and cutaneous signs. They affect nearly 80 thousand of Brazilians. In recent years, the increased scientific knowledge on NF has allowed better clinical management and reduced complication morbidity, resulting in higher quality of life for NF patients. In most cases, neurology, psychiatry, dermatology, clinical geneticists, oncology and internal medicine specialists are able to make the differential diagnosis between NF and other diseases and to identify major NF complications. Nevertheless, due to its great variability in phenotype expression, progressive course, multiple organs involvement and unpredictable natural evolution, NF often requires the support of neurofibromatoses specialists for proper treatment and genetic counseling. This Part 1 offers step-by-step guidelines for NF differential diagnosis. Part 2 will present the NF clinical management.

Neurofibromatoses (NF) constituem um grupo de doenças genéticas com predisposição ao crescimento de múltiplos tumores: tipo 1 (NF1), tipo 2 (NF2) e schwannomatose (SCH). Estas doenças têm em comum a origem neural dos tumores e os sinais cutâneos. Afetam cerca de 80 mil brasileiros. O maior conhecimento científico sobre as NF tem permitido melhor manejo clínico, redução da morbidade das complicações e melhor qualidade de vida. Na maioria dos casos, os especialistas em neurologia, dermatologia, genética clínica, oncologia e medicina interna estão capacitados a realizar o diagnóstico diferencial e identificar suas principais complicações. Devido à sua variabilidade fenotípica, curso progressivo, multiplicidade de órgãos acometidos e evolução imprevisível, as NF frequentemente necessitam de especialistas em NF para o acompanhamento. A Parte 1 deste texto oferece orientações para o diagnóstico de cada tipo de NF e discute os diagnósticos diferenciais com outras doenças. A Parte 2 oferecerá orientações em relação ao manejo clínico das NF.

Prevalência de um fenótipo inédito na Neurofibromatose do Tipo 1 - Dados preliminares / Prevalence of a new phenotype in neurofibromatosis type 1 - Preliminary data

O diagnóstico precoce da neurofibromatose do tipo 1 (NF1) é útil no manejo clínico das complicações da doença: dificuldade de aprendizado, tumores no sistema nervoso, displasias ósseas, distúrbios auditivos e neurofibromas. No entanto, ao nascimento, a maioria dos pacientes com novas mutações apresenta somente manchas café-com-leite (MCL), ou seja, apenas um dos sete critérios diagnósticos adotados em consenso internacional. Portanto, qualquer outro sinal congênito de NF1 poderia ajudar a esclarecer o diagnóstico de NF1 e, consequentemente, o cuidado precoce às crianças. No atendimento de cerca de 600 pacientes com NF1 tem-se observado com certa frequência determinado sinal no segundo dedo dos pés (SSDP), consistindo em discreta elevação do segundo dedo e acavalamento sobre o terceiro dedo. Verificando que esse fenótipo ainda não fora descrito em pessoas com ou sem NF1, decidiu-se estudar se o SSDP seria manifestação fenotípica exclusiva ou mais prevalentena população com NF1. O método utilizado foi o envio de questionário pelo correio para 445 pessoas com pelo menos três critérios diagnósticos para NF1 e por correio eletrônico para 86 autodeclaradas sem NF1. O questionário continha foto com o SSDP, desenho explicativo e envelope selado com endereço para retorno e a pergunta se os pés do entrevistado seriam parecidos com a foto e/ou o desenho. Foram recebidas 167 respostas (39,2%). Nos pacientes com NF1 a prevalência de SSDP foi de 11,9% (11,1% em homens e 12,9% em mulheres, p>0,05),mas nove respostas manifestavam dúvidas (2%). Foram recebidas 60 respostas por e-mail de pessoas autodeclaradas sem NF1, nas quais a prevalência de SSDP foi de 3,3% (apenas dois homens ? pai e filho). Usando-se o teste do qui-quadrado, a diferença de prevalências do SSDP entre pacientes com NF1 parece ser maior do que nos controles (p=0,0503). Após o estudo, as imagens obtidas dos pés dos dois homens sem NF1, mas com SSDP positivo, mostraram hálux valgus em ambos os pés do pai (o que seria a causa do desvio dos dedos) e alteração similar ao SSDP apenas no pé esquerdo do filho. Em conclusão, se o SSDP semostrar verdadeiro sinal da NF1 em estudos subsequentes, ele será útil por estar presente ao nascimento (previamente ao aparecimento das efélides, do glioma óptico, dos neurofibromas cutâneos e dos nódulos de Lisch) e com mais prevalência do que as displasias ósseas.

The early diagnosis of neurofibromatosis type 1 (NF-1) is useful in the clinical management of the disease complications: learning difficulties, tumors in the nervous system, bone dysplasia, hearing disorders, and neurofibromas. However, at birth, most patients with new mutations present only café au lait spots (CLS), i.e. only one of the seven diagnosis criteria adopted in the international consensus. Therefore, any other NF-1 congenital signal could help clarifying its diagnosis and consequentlythe children?s early care. Following the attendance of about 600 NF-1 patients, we have frequently seen a determined signal in the second toe (SST), which consists of a discrete elevation of the second toe andsuperposition on the third one. After verifying that this kind of phenotype has not yet been described in people with or without NF-1, we have decided to study whether the SST would be a phenotypic manifestation that is exclusive or more prevalent in NF-1 population. It was sent a questionnaire as the applied method: a) via mailing to 445 people with at least 3 diagnostic criteria for NF-1; b) by forwarding the same questionnaire through e-mail for 86 people who self-declared being without NF-1. This instrument had a picture of the SST, an explanatorydrawing, and a sealed envelope with the correspondence address and the question if the interviewed person?s feet were alike to the picture and/or drawing. A total of 167 replies (39.2%) were received. In NF-1 patients, the SST prevalence was of 11.9% (11.1% for men and 12.9% for women, p>0.05), but nine answers had doubts (2%). We received 60 replies via e-mail from individuals who self-declared being without NF-1,wherein the SST prevalence was of 3.3% (only two men, father and son). By applying the chi-square test, the SST prevalence difference among patients with NF-1 seems to be higher than in controls (p=0.0503). After the study, the images that were acquired from the feet of both men without NF-1, but with positive SST, showed bunion in both feet of the father (which would be the cause of toe deviation), and a similar change inSST only on the son?s left foot. In conclusion, if the SST proves to be a real NF-1 sign in subsequent studies, it will be useful for being present at birth (before appearance of ephelides, optic glioma, cutaneous neurofibromas, and Lisch nodules), and for having a higher prevalence than the bone dysplasias.