Lisieux Eyer de Jesus

Priapism in children: review of pathophysiology and treatment

OBJECTIVE Priapism may cause serious sequelae concerning the future sex life of the patient, as it can determine impotence, erectile dysfunction or psychogenic sexual aversion. It is a common symptom of sickle cell disease in children and adolescents. There are few good quality evidence manuscripts about the problem in current medical literature. SOURCES Literature review on the databases MEDLINE and LILACS covering the period from 1966 to 2008. SUMMARY OF THE FINDINGS The basis for the treatment of low flow priapism includes treating sickle cell disease and the usage of intracavernous adrenergic agents as necessary. Surgery is indicated in a minority of cases. The treatment of pediatric cases demands dose adjustments, adequate drug choice and sedoanalgesia to cover procedures involving pain or trauma. CONCLUSIONS A new physiopathologic theory concerning sickle cell disease, which questions the traditional vascular blockage mechanisms by deformed red cells and proposes that endothelial inflammatory activation is the main cause of clinical problems, allows to propose new therapeutic maneuvers to solve sickle cell priapism. The absence of good quality evidence to treat sickle cell priapism suggests the necessity to conduct good prospective multicenter protocols to investigate the condition.

Priapismo em crianças: revisão de fisiopatologia e tratamento

OBJETIVOS: O priapismo pode causar sequelas graves em relacao a vida sexual futura do paciente, pois pode determinar impotencia, disfuncao eretil ou aversao sexual psicogenica. E um sintoma comum da doenca falciforme em criancas e adolescentes. Ha poucos estudos com evidencias de qualidade sobre esse problema na literatura medica atual. FONTES DOS DADOS: Revisao da literatura utilizando as bases de dados MEDLINE e LILACS no periodo de 1966 a 2008. SINTESE DOS DADOS: A base para o tratamento de priapismo de baixo fluxo inclui o tratamento da doenca falciforme e o uso de agentes adrenergicos por administracao intracavernosa conforme necessario. Indica-se cirurgia em uma minoria de casos. O tratamento de casos pediatricos exige ajuste de doses, escolha adequada de medicamentos e sedoanalgesia para procedimentos envolvendo dor ou trauma. CONCLUSOES: Uma nova teoria fisiopatologica relativa a doenca falciforme, que questiona os mecanismos tradicionais de bloqueio vascular por hemacias deformadas e propoe que a ativacao inflamatoria endotelial e a principal causa de problemas clinicos, permite propor novas manobras terapeuticas para lidar com o priapismo na doenca falciforme. A ausencia de evidencia de qualidade para tratar o priapismo na doenca falciforme sugere a necessidade de conduzir bons protocolos prospectivos multicentricos para investigar essa condicao.

Ensinar cirurgia: como e para quem?

The autor discusses the crisis concerning education and professional life for doctors and surgeons making a review of contemporary data concerning professional epidemiology. We reflect on the modern ethical and social references to the medical profession. There is a decline in medical schools preparing doctors and surgeons, and a progressive ¨feminization¨ of the profession. Young professionals want quality of life and professional autonomy as their main professional objectives. Burn-out syndromes are common between doctors, and loss of professional autonomy may be the main cause of the disease. The crisis that attains the medical profession in our days has been ethical, social and financial roots. It is essential to discuss new paradigms in medical education and professional routine, concerning the modern society.

Síndrome do notocórdio fendido, variante rara do cisto neuroentérico

OBJECTIVE: We present a case of split notochord syndrome, an extremely rare form of spinal dysraphism. DESCRIPTION: We treated a 2 month-old boy presenting with an extensive lumbosacral deformity, hydrocephalus and apparent enteric segments in the dorsal midline, accompanied by an enteric fistula and imperforated anus. The malformation was diagnosed as split notochord syndrome. The baby died as a result of sepsis before surgical treatment could be attempted. COMMENTS: Split notochord syndrome is the rarest form of neuroenteric cyst described until this moment (< 25 cases in the literature). It is frequently associated with anorectal malformation, intestinal fistulae and hydrocephalus. Prognosis is not necessarily poor and survival is possible if digestive malformations, hydrocephalus and the dysraphism itself are treated simultaneously.

Acquired Labial Sinechiae and Hydrocolpos Secondary to Stevens-Johnson Syndrome

Stevens-Johnson syndrome frequently affects the genitalia. Vaginal problems have been recognized in female patients; however, reports about the problem and its treatment are rare. Labial sinechiae have not yet been reported as sequelae of Stevens-Johnson syndrome. Amenorrhea, cyclical abdominal pain, and a hypogastric mass in girls affected by Stevens-Johnson syndrome could indicate acquired vaginal obstruction. Extensive labial sinechiae in such patients can cause dysuria, urinary tract infection, and sexual dysfunction. After a diagnosis of Stevens-Johnson syndrome in girls, it is prudent to schedule a prepubertal genital examination to diagnose genital disease preemptively and avoid obstructed menstruation and future sexual problems.

Psychosocial and respiratory disease related to severe bladder dysfunction and non-monosymptomatic enuresis

OBJECTIVE Complicated bladder dysfunctions (BD) (associated with infections/urological complications or irresponsive to treatment) are a small proportion of all cases, but are highly morbid, clinically and psychosocially. Our aim is to describe a cohort of complicated pediatric BD, using subgroup analysis to compare presentations and responses to treatment among genders, age groups, and patients with or without non-monosymptomatic enuresis (NME). We also relate severe BD to other health conditions or to social/behavioral problems and report treatment results. METHOD Thirty-five cases of complicated BD were reviewed. Neurogenic bladders and anatomical urological problems were excluded. Justifications for referral, comorbidities, and social aspects/familial dynamics were studied. Overactive bladders were primarily treated with oxybutynin. Transcutaneous parasacral neuromodulation was used in case of insufficient response or unbearable side effects. For infrequent voiders, timed voiding and transcutaneous neuromodulation were counseled. RESULTS Incontinence/enuresis were the motives for referral in only a third of the cases. UTI (42.9%) was the main reason for referral. Hydronephrosis was observed in 8.6% of the children. Respiratory/ENT problems, obesity, and precocious puberty were highly prevalent. Schooling problems and neuropsychiatric disease were common. Social problems were common. Five patients presented urological problems secondary to BD (hydroureteronephrosis, VUR, trabeculated bladder). Twenty percent of cases required high anticholinergic doses and 37.1% transcutaneous electrostimulation. Eight (22.9%) patients abandoned but later resumed therapy, and 14.6% did not follow treatment. Boys tended to be older than girls and presented NME, respiratory, and behavioral problems more often, with a significant difference for asthma and anxiety/depression. Associated health problems and neuropsychiatric treatment tended to be more frequent among those presenting NME. Non-enuretic children tended to show better results from treatment (see Table). CONCLUSION The social characteristics of our population (severe cases, socially deprived, very poor, not well educated, and with limited access to health care) determine a very specific sampling. Our research demonstrated that even severe cases of BD affecting socially deprived children may be treated, with adhesion to treatment and results comparable with other cohorts of BD, although the children need multidisciplinary attention and close follow-up. Boys, older children, and NME are more difficult to treat and often have other associated health and behavioral problems. Stress-related conditions were common in severe BD. A relatively high occurrence of precocious puberty was an unexpected finding in our research.

Adolescents and adults in pediatric urology clinics

OBJECTIVE Adolescents represent a significant proportion of the patients seen by pediatric urologists (PU). Adolescents that require long-term follow-up (FU) eventually need to be transferred to adult care. This research aims to describe the disease profile of adolescent and adult patients from a referral PU clinic, in order to allow future medical education and cost planning. PATIENTS AND METHODS A retrospective review was made of cases seen in a PU referral clinic from January 1st to December 31st 2011. Patients were classified as child (≤12 years old), adolescent (12 < age ≤ 18 years old) or adult (>18 years old). Diagnoses and presentations were analyzed. RESULTS 521 patients were seen: 404 children (77.5%), 103 adolescents (19.8%) and 14 adults (2.7%). Clinical profiles differed between the three groups. 43.7% of the adolescents and all adults were FU cases or patients with late sequelae of congenital diseases. Some diseases predominated largely in adolescents. CONCLUSIONS Adolescents represent a significant proportion of our PU clinics. Half of them are FU cases or present sequelae from previous treatments/diseases. Some adolescents do not adapt to adult care, and persist being followed up by PU.

Scrotoschisis: An Extremely Rare Congenital Uropathy

Scrotoschisis, the congenital extrusion of the testis through the scrotal wall, is very rare. We describe a new case and review the pertinent published data. A male neonate presented with left scrotoschisis that was repaired successfully with a Denis-Browne trans-scrotal orchiopexy, with antibiotic coverage. The testes were intrascrotal and symmetric after 2 years of follow-up. Most cases of scrotoschisis are unilateral and affect normal males. The immediate prognosis is good, but long-term results are not available. The etiology of the disease is unknown, but it might be related to spontaneous healed fetal intestinal perforations with meconium peritonitis.

Formação e demanda do cirurgião pediátrico no Brasil

OBJECTIVE To define the distribution of Pediatric Surgeons in Brazil and the distribution of job offered for specialists in the country. To compare the professional profile of Brazilian Pediatric Surgeons with other countries. METHODS Statistical data derived from Brazilian Institute of Geography and Statistics (IBGE), Brazilian Medical Council (CFM) and Brazilian Society of Pediatric Surgery (CIPE) were used to define the professional profile of pediatric surgeons in Brazil. RESULTS Eight-hundred-and-five pediatric surgeons are needed in Brazil only to treat neonates and toddlers. Professionals are excessively concentrated in South and Southeast and insufficient numbers are found in North and Northeast. Data about the number of pediatric surgeons working in Brazil are conflicting: data obtained from FIOCRUZ differ from those from CFM and CIPE. The working routine of Brazilian pediatric surgeons is different from that of North-American and European specialists, from where the major part of our references is extracted. CONCLUSION Brazil needs a minimum of 805 pediatric surgeons working full time only to treat nenonates and toddlers. Specialists are not well distributed throughout the country. The southeast is a training center and exports specialists to the rest of the country. Epidemiological data concerning the distribution of professionals in Brazil are conflicting.

Pre-transplant management of valve bladder: A critical literature review

OBJECTIVE Indications, timing and problems related to augmentation cystoplasty (AC), in the context of posterior urethral valves (PUV) and renal transplantation (RT) are ill defined. Associated bladder dysfunction (BD) is not a stable condition and may cause the loss of the renal graft. Polyuria, accentuates BD and seems to improve after RT. The objective of this research is to critically review the available literature, aiming to rationalize the treatment of PUV with BD in the context of end stage renal disease (ESRD). MATERIALS AND METHODS A thorough literature review was performed. Pertinent papers were, critically analyzed and classified according to the level of evidence. RESULTS Data relating to PUV, RT and AC showed low levels of evidence. Results of RT in PUV cases with adequate management of BD were comparable to those suffering from other causes of ESRD. Bladder function can recover spontaneously after urinary undiversion. There were no established criteria to indicate AC in the context of ESRD and PUV or to define the ideal protocol to treat associated vesicoureteral reflux (VUR). Urinary tract infections (UTIs) were more frequent in transplanted PUV patients; this is possibly related to the inadequate control of BD, especially after AC. AC is feasible after RT with outcomes comparable to preemptive ones. CONCLUSION AC increases the risk of UTI after RT. Preemptive AC should be constructed only if the risks associated with increased bladder pressures exceed those associated with AC. Adequate management of BD is essential to improve bladder function and to minimize UTIs. AC is feasible after RT, with complication rates similar to the ones performed beforehand. Since a considerable number of PUV patients with high-pressure bladders eventually develop myogenic failure, it seems logical to postponing AC in this population, as long as they are under close surveillance.