RESUMO
Distal hypospadias is a common congenital urology anomaly for which numerous corrective procedures have been described. Over the last 40 years, the gold-standard operative technique for distal hypospadias has switched from the meatal advancement and glanuloplasty (MAGPI) procedure to the tubularized incised plate (TIP) urethroplasty. A modification to the MAGPI procedure, first described 30 years ago, is the M inverted V (MIV) glansplasty, which improved upon the MAGPI procedure to reduce instances of meatal retraction. The MIV glansplasty is unique compared to many commonly used procedures as it does not require a formal urethroplasty or incorporation of a dartos flap, and it does not always necessitate as extensive mobilization of the glans wings. We describe our updated technique and outcomes of the MIV glansplasty and delineate situations where the MIV is best employed.
Assuntos
Hipospadia , Seguimentos , Humanos , Hipospadia/cirurgia , Lactente , Masculino , Pênis/cirurgia , Retalhos Cirúrgicos , Resultado do Tratamento , Uretra/cirurgia , Procedimentos Cirúrgicos Urológicos Masculinos/métodosRESUMO
Neuroendocrine tumors (NETs) are rare tumors with varying clinical presentations. We describe the case of an 11-year-old female presenting with Cushingoid features in the setting of a left-sided flank mass. Her presentation and evaluation suggested a paraneoplastic ectopic ACTH syndrome. She underwent open left radical nephrectomy and final pathology confirming a high-grade NET with nodal metastasis. Although exceedingly rare, ACTH-secreting tumors of the kidney can cause significant morbidity and mortality and so we recommend it be included in the differential diagnosis of pediatric renal masses.
Assuntos
Hormônio Adrenocorticotrópico/metabolismo , Neoplasias Encefálicas/secundário , Neoplasias Renais/terapia , Neoplasias Pulmonares/secundário , Tumores Neuroendócrinos/terapia , Quimioterapia Adjuvante , Criança , Evolução Fatal , Feminino , Humanos , Neoplasias Renais/metabolismo , Neoplasias Renais/patologia , Metástase Linfática , Nefrectomia , Tumores Neuroendócrinos/metabolismo , Tumores Neuroendócrinos/secundárioRESUMO
OBJECTIVE: To determine if there are histologic differences relative to tobacco exposure in buccal mucosa. Substitution urethroplasty outcomes may be worse in tobacco users and we investigate if the buccal graft is inherently damaged due to chronic tobacco exposure. METHODS: Subjects undergoing substitution urethroplasty with buccal graft harvest were prospectively consented in this IRB approved study. Subjects with poor dentition were excluded. A detailed tobacco use history was obtained. Cotinine testing was performed day of surgery to confirm or exclude active tobacco use. Trimmed portions of harvested graft were sent for tissue processing. Standard hematoxylin and eosin staining was performed. A single blinded pathologist performed analysis of the slides. Using a scale of none, mild, moderate, or severe slides were analyzed for cytologic atypia, architectural complexity, inflammation, and keratinization. Evidence of vascular damage was noted and the type of inflammation if present was classified. RESULTS: Twenty-five buccal grafts were analyzed. No evidence of vascular damage or cytologic atypia were noted in any grafts. While mild architectural complexity and mild inflammation, typically lymphocytic, were noted in several of the buccal mucosa sections, this did not appear to correlate with tobacco exposure. The majority of grafts demonstrating increased keratinization correlated with significant tobacco exposure, but this was not consistently noted in all those with tobacco use. CONCLUSIONS: Buccal mucosa in patients with tobacco exposure did not show significant histologic alterations. Outcomes of substitution urethroplasty may be more impacted by persistent systemic exposure causing local ischemia as opposed to the graft tissue itself.
Assuntos
Estreitamento Uretral , Masculino , Humanos , Estreitamento Uretral/cirurgia , Procedimentos Cirúrgicos Urológicos Masculinos , Uretra/cirurgia , Coleta de Tecidos e Órgãos , Mucosa Bucal/transplante , Uso de Tabaco/efeitos adversos , Resultado do TratamentoRESUMO
BACKGROUND: The technique of hypospadias repair with a dorsal inlay graft (ingraft) was initially reserved for boys with an unfavorable glans configuration or in previously failed repairs. Although the ingraft technique has been used for some time, there is scarce literature describing its longer term outcomes. Additionally, there is minimal data comparing inner preputial skin and buccal mucosal graft outcomes in primary and reoperative surgery. OBJECTIVE: To describe longer-term outcomes in the use of buccal mucosa and inner preputial skin ingrafts for primary and revisional hypospadias repairs. STUDY DESIGN: We conducted a single-center retrospective review of our hypospadias repairs employing ingrafts along with a review of the literature. RESULTS: A total of 47 patients met inclusion criteria. Primary repair was performed in 38 patients, all with unfavorable glans characteristics at a mean age of 16 months and redo repairs in 9 boys at a mean age of 110 months. We used a buccal mucosal graft (BMG) in 19 boys and the remaining 28 were grafted with inner preputial skin. The complication rate was 32% in primary repairs and amongst those, 6 of 28 patients (21%) with skin grafts and 6 of 10 patients (60%) with BMGs suffered a complication. Only BMGs were used in reoperative situations and complications occurred in 2 of 9 cases (22%) of those cases. Overall, 8 patients (42%) with BMG and 6 patients (21%) with preputial skin ingrafts experienced a complication, at an average time of 17 months (range: 0.4-66 months) and 24 months (range: 1.1-113 months), respectively. Surgical correction of the complications resulted in resolution of symptoms in a majority of patients. DISCUSSION: In our experience, BMGs used as salvage therapy in revisional hypospadias surgery, had lower observed complication rates when compared to its use in primary repair. Inner preputial skin ingrafts for primary repair yielded an acceptable complication rate. Our study describes some of the longest follow-up times in the literature with complications observed even up to 10 years postoperatively. This reinforces the need for active long-term follow-up in reporting outcomes in hypospadias surgery.
Assuntos
Hipospadia , Criança , Seguimentos , Humanos , Hipospadia/cirurgia , Lactente , Masculino , Estudos Retrospectivos , Resultado do Tratamento , Uretra/cirurgia , Procedimentos Cirúrgicos Urológicos MasculinosRESUMO
INTRODUCTION: Urethrorrhagia characterized by terminal hematuria and/or bloody spotting of the underwear between episodes of voiding is a common problem of childhood. We describe a series of boys with urethrorrhagia and associated clinical and endoscopic findings. METHODS AND MATERIALS: The records of boys presenting between 1990 and 2005 with urethrorrhagia were retrospectively reviewed for age, symptoms, symptom duration, physical examination, radiographic evaluation, laboratory data and endoscopic findings. RESULTS: Of 66 boys, 50 presented with classic symptoms of terminal hematuria and/or blood spotting, and 16 with atypical symptoms who underwent cystoscopy with similar findings. Endoscopic evaluation was performed in 55 (82%) patients for prolonged symptomatology or recurrent symptoms and revealed four distinct patterns: Group 1, three (5%) patients with a normal appearing urethra; Group 2, 27 (49%) patients who were found to have inflammation or hyperemia of the bulbar urethra; Group 3, 17 (31%) cases in which there was a white membranous exudate or mucosal irregularity; and Group 4, eight (14%) patients who were found to have stricture disease on cystoscopy. Stricture developed after initial cystoscopy in six cases. CONCLUSION: Consistent with previous studies, a subset of patients developed urethral stricture disease after presenting with urethrorrhagia. As eight patients had a urethral stricture on initial cystoscopy, and those with strictures are clinically indistinguishable symptomatically from those without, we feel that uroflow and selective cystoscopy are important tools for the work-up of severe and persistent cases of urethrorrhagia. Group 3 may represent a population at significant risk for the development of stricture disease warranting closer follow up and evaluation. The white membranous exudate found on cystoscopy may represent a pre-stricture lesion as evidenced by four cases that subsequently developed strictures.
RESUMO
The Malone antegrade continence enema (MACE) is a therapeutic option to treat chronic constipation and fecal incontinence in patients with neurogenic bowel. Previous reports have described the short-term success of this procedure, but no report has described the durability of the procedure during pregnancy. We present the case of a spinal cord injury patient who underwent an uncomplicated pregnancy after a MACE procedure with no stomal catheterization difficulties or leakage during or after the pregnancy.