-
Indian Journal of Plastic Surgery :... Sep 2011The aim of this study was to investigate the efficacy of the rectus abdominis myo-peritoneal flap (RAMP) technique for the closure and augmentation of small,...
BACKGROUND
The aim of this study was to investigate the efficacy of the rectus abdominis myo-peritoneal flap (RAMP) technique for the closure and augmentation of small, non-elastic, non-compliant bladder exstrophies.
MATERIALS AND METHODS
The RAMP technique was used in three boys with bladder exstrophy who presented late with small, non-elastic, non-compliant bladder. The clinical outcome, imaging, cystoscopy, biochemical and microbiological studies were assessed during a follow-up of 36 months.
RESULTS
Bladder closure and augmentation was achieved in all patients without any complications. There were no urinary tract infections, metabolic problems or electrolyte disturbances and the kidney function remained normal in all patients. Radiography confirmed intact function and anatomy of the urinary tract and cystoscopy showed complete coverage of the inner peritoneal layer of RAMP with uroepithelium. No stone formation or mucous production was detected.
CONCLUSIONS
The RAMP technique is a good alternative for closing bladder exstrophies and achieves an increase in bladder capacity and compliance. The technique is indicated in the closure of large bladder defects, bladder exstrophies with small, inelastic, non-compliant bladder remnants and failed primary closures.
PubMed: 22279277
DOI: 10.4103/0970-0358.90817 -
Journal of the Royal Society of Medicine Jan 1996Exstrophy of the bladder is rare and the incidence of bladder exstrophy is calculated to be from 1 per 30,000 to 50,000 live births with male to female ratio ranging... (Review)
Review
Exstrophy of the bladder is rare and the incidence of bladder exstrophy is calculated to be from 1 per 30,000 to 50,000 live births with male to female ratio ranging from 1.5-5 to 1(1-4). It was found that persistence or overgrowth of the cloacal membrane on the lower anterior abdominal area, prevents normal mesenchymal ingrowth. This causes divergence of the lower abdominal muscular structures and forces the genital ridges to fuse caudal to the cloacal membrane. The stage of ingrowth of the urorectal septum at the time of rupture determines whether one will produce an exstrophic urinary tract alone (classic bladder exstrophy or epispadias) or cloacal exstrophy with the hindgut interposed between the hemibladders.
Topics: Adolescent; Adult; Age Factors; Bladder Exstrophy; Child; Child, Preschool; Epispadias; Female; Fetal Diseases; Humans; Infant, Newborn; Male; Mental Disorders; Pregnancy; Prenatal Diagnosis
PubMed: 8709084
DOI: 10.1177/014107689608900112 -
Innovative Surgical Sciences Jun 2018Tissue engineering is defined as the combination of biomaterials and bioengineering principles together with cell transplantation or directed growth of host cells to... (Review)
Review
Tissue engineering is defined as the combination of biomaterials and bioengineering principles together with cell transplantation or directed growth of host cells to develop a biological replacement tissue or organ that can be a substitute for normal tissue both in structure and function. Despite early promising preclinical studies, clinical translation of tissue engineering in pediatric urology into humans has been unsuccessful both for cell-seeded and acellular scaffolds. This can be ascribed to various factors, including the use of only non-diseased models that inaccurately describe the structural and functional modifications of diseased tissue. The paper addresses potential future strategies to overcome the limitations experienced in clinical applications so far. This includes the use of stem cells of various origins (mesenchymal stem cells, hematopoietic stem/progenitor cells, urine-derived stem cells, and progenitor cells of the urothelium) as well as the need for a deeper understanding of signaling pathways and directing tissue ingrowth and differentiation through the concept of dynamic reciprocity. The development of smart scaffolds that release trophic factors in a set and timely manner will probably improve regeneration. Modulation of innate immune response as a major contributor to tissue regeneration outcome is also addressed. It is unlikely that only one of these strategies alone will lead to clinically applicable tissue engineering strategies in pediatric urology. In the meanwhile, the fundamental new insights into regenerative processes already obtained in the attempts of tissue engineering of the lower urogenital tract remain our greatest gain.
PubMed: 31579774
DOI: 10.1515/iss-2018-0011 -
Journal of Indian Association of... 2022The aim of this study is to report surgical outcomes of the neonates who have undergone various surgical procedures.
PURPOSE
The aim of this study is to report surgical outcomes of the neonates who have undergone various surgical procedures.
MATERIALS AND METHODS
In this retrospective study, 39 neonates who have undergone a surgical procedure in Mogadishu Somalia Turkey Recep Tayyip Erdoğan Training and Research Hospital, between October 2018 and March 2019 were included. Data regarding age, gender, diagnosis, surgical procedure, length of hospital stay, mortality, and cause of mortality were recorded.
RESULTS
Of 39 neonates, 12 were female (30.7%) and 27 were male (69.3%). The mean age of the neonates at admission was 7.7 ± 7.6 days (1-30 days) days. The most common diagnoses were anal atresia ( = 12, 30.8%), esophageal atresia ( = 9, 23.1%), and pyloric stenosis ( = 5, 12.8%). The most common surgical procedures were colostomy creation ( = 10, 25.6%), esophageal anastomosis ( = 9, 20.5%), primary closure of anterior abdominal wall defects including bladder exstrophy ( = 6, 15.4%), and pyloromyotomy ( = 5, 12.8%). Mortality rate was 17.9%, and mortality causes were sepsis ( = 4, 57.1%) and congenital heart disease ( = 3, 42.9%). Neonates with the highest mortality by underlying primary surgical diagnosis were esophageal atresia (n=4, 57.1%).
CONCLUSION
The mortality rate from the surgical procedures of the neonates in Somalia is extremely high when compared with the developed countries. Employment of experienced pediatric surgeons and well-trained nurses, strict attention to the sanitary measures and shortening the time from birth to presentation might improve the surgical outcomes of the neonates in Somalia.
PubMed: 35937105
DOI: 10.4103/jiaps.JIAPS_349_20 -
Anatomy & Cell Biology Dec 2023Bladder exstrophy is a rare congenital condition of the pelvis, bladder, and lower abdomen that opens the bladder against the abdominal wall, produces aberrant growth,... (Review)
Review
Bladder exstrophy is a rare congenital condition of the pelvis, bladder, and lower abdomen that opens the bladder against the abdominal wall, produces aberrant growth, short penis, upward curvature during erection, wide penis, and undescended testes. Exstrophy affects 1/30,000 newborns. The bladder opens against the abdominal wall in bladder exstrophy, a rare genitourinary condition. This study is vital to provide appropriate therapy choices as a basis to improve patient outcomes. This study may explain bladder exstrophy and provide treatment. Epispadias, secretory placenta, cloacal exstrophy, and other embryonic abnormalities comprise the exstrophy-spades complex. The mesenchymal layer does not migrate from the ectoderm and endoderm layers in the first trimester, affecting the cloacal membrane. Embryological problems define the exstrophy-aspidistra complex, which resembles epimedium, classic bladder, cloacal exstrophy, and other diseases. Urogenital ventral body wall anomalies expose the bladder mucosa, causing bladder exstrophy. Genetic mutations in the Hedgehog cascade pathway, Wnt signal, FGF, BMP4, Alx4, Gli3, and ISL1 cause ventral body wall closure and urinary bladder failure. External factors such as high maternal age, smoking moms, and high maternal body mass index have also been associated to bladder exstrophy. Valproic acid increases bladder exstrophy risk; chemicals and pollutants during pregnancy may increase bladder exstrophy risk. Bladder exstrophy has no identified cause despite these risk factors. Exstrophy reconstruction seals the bladder, improves bowel function, reconstructs the vaginal region, and restores urination.
PubMed: 37649128
DOI: 10.5115/acb.23.056 -
Urology Case Reports May 2022Bladder exstrophy is a severe malformation characterized by the lack of the anterior sub-umbilical abdominal wall, and the front wall of the bladder. We present a rare...
Bladder exstrophy is a severe malformation characterized by the lack of the anterior sub-umbilical abdominal wall, and the front wall of the bladder. We present a rare case of a 26-year-old woman without any previous medical or surgical history, that we treated for bladder exstrophy. We performed an iliac osteotomy, bladder enlargement using the ileum and a Monti-type continent urinary derivation and a Promentofixation. A vesico-cutaneous fistula was diagnosed after surgery and we failed to manage it after two surgical revision. Therefore, we performed a cystectomy and a non-continent Bricker external urinary derivation.
PubMed: 35116225
DOI: 10.1016/j.eucr.2022.102001 -
International Braz J Urol : Official... 2022
Topics: Abdominal Muscles; Bladder Exstrophy; Hernia, Ventral; Humans; Infant; Retrospective Studies; Urinary Bladder; Urologic Surgical Procedures
PubMed: 35838513
DOI: 10.1590/S1677-5538.IBJU.2022.05.02 -
The Indian Journal of Radiology &... Sep 2022Early prenatal diagnosis of bladder exstrophy is challenging because of its variable size and presentation. This article brings forth new signs on color Doppler (CD) to...
Pattern Recognition of Abdominal Vasculature on Color Doppler in the Fetus as a Tool for Early Diagnosis of Bladder Exstrophy in the First and Early-Second Trimester: Initial Observations.
Early prenatal diagnosis of bladder exstrophy is challenging because of its variable size and presentation. This article brings forth new signs on color Doppler (CD) to help establish the diagnosis in a suspected case. Two cases of omphalocele-exstrophy-imperforate anus-spinal defects complex presenting as a solid-cystic ventral mass at 11 weeks and a solid lower abdominal wall mass at 20 weeks, with nonvisualization of the urinary bladder, were studied by gray-scale and CD in sagittal and transverse-bladder views of the abdomen. The sagittal view on CD revealed an altered intrafetal course of umbilical artery (UA), widened UA-aorta angle (K angle), a break in the intersection of UA, and umbilical vein (UV) at the umbilicus-broken "X-sign" with distortion of the equilateral triangle normally formed by aorta, UA, and UV. The transverse-bladder view showed an altered divergent course of single-UA. Combination of these findings substantiated early diagnosis of bladder exstrophy, thus facilitating prenatal counseling. Early prenatal diagnosis of bladder exstrophy is possible by pattern recognition of abdominal vasculature on color Doppler.The new signs-"X" and "Y" derived on color Doppler, as a clue to early diagnosis of bladder exstrophy-have not been identified in the current literature that makes this article unique.
PubMed: 36177283
DOI: 10.1055/s-0042-1754363 -
African Journal of Paediatric Surgery :... 2023Classical bladder exstrophy is a congenital anomaly whose management and outcome has advanced over years. Management and outcome are better when management starts at the...
Classical bladder exstrophy is a congenital anomaly whose management and outcome has advanced over years. Management and outcome are better when management starts at the newborn period. This was the management of a neglected bladder exstrophy in a male presenting at 16 years of age. We report our challenges, management and outcome to highlight the rarity of this presentation, and the adaptation to the usual protocol of care. The patient presented at 16 years of age with classic bladder exstrophy. The bladder plate was contracted and had cystitis. The patient had a modification of complete primary repair of exstrophy (CPRE) with bilateral pelvic osteotomy stabilised with a 7-hole plate and 4 screws, then bladder neck reconstruction + bladder augmentation + cross-trigonal neocystoureterostomy in a 12-h procedure. He had surgical site infection, superficial wound breakdown and vesicocutaneous fistula that all healed with dressing and prolonged suprapubic cystostomy drainage. He achieved some degree of urinary continence and ability to void, though he still has stress incontinence and frequency at 6 months of follow-up. He has a micturition interval of 60-120 min, and is expected to improve. Presentation and repair of classic bladder exstrophy in the adolescent is very rare in the literature and therefore no known standard of care. This report adds to the body of knowledge. Again, this experience lends credence to the proponents of CPRE in reducing the number of procedures required to treat exstrophy.
Topics: Infant, Newborn; Humans; Male; Adolescent; Bladder Exstrophy; Urinary Incontinence; Urologic Surgical Procedures; Urination; Plastic Surgery Procedures; Treatment Outcome; Epispadias
PubMed: 37470562
DOI: 10.4103/ajps.ajps_172_21 -
Frontiers in Pediatrics 2019Today, there are few indications for the use of bowel in pediatric urology. This is in large extent due to the successful conservative therapy in patients with... (Review)
Review
Today, there are few indications for the use of bowel in pediatric urology. This is in large extent due to the successful conservative therapy in patients with neurogenic bladder and the improved success of primary reconstruction in patients with the bladder exstrophy-epispadias complex. Only after the failure of the maximum of conservative therapy or after failure of primary reconstruction, bladder augmentation, or urinary diversion should be considered. Malignant tumors of the lower urinary tract (e.g., rhabdomyosarcomas of the bladder/prostate) are other rare indications for urinary diversion. Replacement or reconstruction of the ureter with a bowel segment is also a quite rarely performed procedure. In this review, the advantages and disadvantages of the different options for the use of bowel segments for bladder augmentation, bladder substitution, urinary diversion, or ureter replacement during childhood and adolescence are discussed.
PubMed: 31245339
DOI: 10.3389/fped.2019.00236