ABSTRACT
INTRODUCTION: Bladder exstrophy (BE) is a rare, complex malformation. There are three major approaches to closure. Despite this choice, abdominal wall closure in such patients is usually a challenging procedure specially in large defects and redo cases. OBJECTIVE: Our aim is to present our ten first cases' results, using Anterior Component Separation (ACS) to abdominal wall closure in BE patients. STUDY DESIGN: Ten male patients with BE (median age 7 months, range from 3 to 24 months) were operated from March 2020 to March 2021 by a multi-institutional Brazilian group using the Kelly technique. In addition to BE correction, anterior component separation was performed for abdominal closure. RESULTS: Postoperative suprapubic fistulae occurred in two of ten patients, but both closed spontaneously. No evisceration, abdominal wall dehiscence, or herniation was observed at a mean follow-up time of 14 months (range from 10 to 22 months). A 3 cm extent of advancement is achievable upon traction in each side (Fig. 3). CONCLUSION: We proposed the use of anterior component separation as an alternative for abdominal closure after BE correction using the Kelly procedure. This new technique avoids mesh usage, loosens the abdominal wall tension, and reduces complications. Even However, further studies are required.
Subject(s)
Abdominal Wall , Bladder Exstrophy , Hernia, Ventral , Humans , Male , Infant , Child, Preschool , Bladder Exstrophy/surgery , Bladder Exstrophy/complications , Abdominal Muscles , Hernia, Ventral/complications , Hernia, Ventral/surgery , Urologic Surgical Procedures/methods , Abdominal Wall/surgery , Postoperative Complications , Retrospective StudiesABSTRACT
Early diagnosis of pediatric cancer is key for adequate patient management and improved outcome. Although multiparameter flow cytometry (MFC) has proven of great utility in the diagnosis and classification of hematologic malignancies, its application to non-hematopoietic pediatric tumors remains limited. Here we designed and prospectively validated a new single eight-color antibody combination-solid tumor orientation tube, STOT-for diagnostic screening of pediatric cancer by MFC. A total of 476 samples (139 tumor mass, 138 bone marrow, 86 lymph node, 58 peripheral blood, and 55 other body fluid samples) from 296 patients with diagnostic suspicion of pediatric cancer were analyzed by MFC vs. conventional diagnostic procedures. STOT was designed after several design-test-evaluate-redesign cycles based on a large panel of monoclonal antibody combinations tested on 301 samples. In its final version, STOT consists of a single 8-color/12-marker antibody combination (CD99-CD8/numyogenin/CD4-EpCAM/CD56/GD2/smCD3-CD19/cyCD3-CD271/CD45). Prospective validation of STOT in 149 samples showed concordant results with the patient WHO/ICCC-3 diagnosis in 138/149 cases (92.6%). These included: 63/63 (100%) reactive/disease-free samples, 43/44 (98%) malignant and 4/4 (100%) benign non-hematopoietic tumors together with 28/38 (74%) leukemia/lymphoma cases; the only exception was Hodgkin lymphoma that required additional markers to be stained. In addition, STOT allowed accurate discrimination among the four most common subtypes of malignant CD45- CD56++ non-hematopoietic solid tumors: 13/13 (GD2++ numyogenin- CD271-/+ nuMyoD1- CD99- EpCAM-) neuroblastoma samples, 5/5 (GD2- numyogenin++ CD271++ nuMyoD1++ CD99-/+ EpCAM-) rhabdomyosarcomas, 2/2 (GD2-/+ numyogenin- CD271+ nuMyoD1- CD99+ EpCAM-) Ewing sarcoma family of tumors, and 7/7 (GD2- numyogenin- CD271+ nuMyoD1- CD99- EpCAM+) Wilms tumors. In summary, here we designed and validated a new standardized antibody combination and MFC assay for diagnostic screening of pediatric solid tumors that might contribute to fast and accurate diagnostic orientation and classification of pediatric cancer in routine clinical practice.
ABSTRACT
PURPOSE: Surgical management of the high urogenital sinus remains challenging. The anterior sagittal transrectal approach provides optimal exposure, facilitates vaginal dissection and separation from the urethra, and allows reconstruction of the bladder neck musculature. In this study we report our initial experience with this technique. MATERIALS AND METHODS: We performed a retrospective review of a 6-year multi-institutional experience treating patients with a urogenital sinus anomaly using the anterior sagittal transrectal approach without preoperative colostomy or prolonged postoperative fasting. Variables analyzed included patient age, associated malformations, the need for additional procedures and surgical outcomes. RESULTS: A total of 23 children with a mean age of 2.3 years (range 3 months to 17 years) who underwent surgery between 2003 and 2010 were included in the study. Mean followup was 3.4 years (range 14 months to 7 years). All children had a high urogenital sinus with (16) or without (7) congenital adrenal hyperplasia. There were 3 isolated cases treated with additional procedures. Only 1 anterior sagittal transrectal approach related complication was encountered when a perineal infection developed in a child and required temporary diverting colostomy without compromising the repair. There were no postoperative urethrovaginal fistulas. All toilet trained patients were continent for feces and most were voiding normally per urethra (21), except for 2 with associated urological malformations. There were 15 patients who underwent followup examination under anesthesia, and demonstrated separate urethral and vaginal openings. CONCLUSIONS: The anterior sagittal transrectal approach provides excellent exposure for the management of a high urogenital sinus, facilitating the separation of urogenital structures. Good outcomes in terms of urinary/fecal continence as well as the absence of urethrovaginal fistulas were achieved in the majority of cases, supporting its consideration for the surgical management of this congenital abnormality.