A Síndrome de Prune Belly é uma condição rara com a qual o bebê já nasce (congênita). Ela faz parte do grupo de obstruções (bloqueios) das vias urinárias inferiores do feto (conhecidas como LUTO). Essa síndrome causa um alargamento variável (dilatação) das vias urinárias inferiores. Ela vem acompanhada pela ausência (falta) parcial ou total dos músculos da parte lateral e inferior da parede da barriga. E, em meninos, os testículos não descem para a bolsa escrotal e não podem ser sentidos (não palpáveis) em ambos os lados.
Introdução
O que você precisa saber de cara
A Síndrome de Prune Belly é uma condição rara com a qual o bebê já nasce (congênita). Ela faz parte do grupo de obstruções (bloqueios) das vias urinárias inferiores do feto (conhecidas como LUTO). Essa síndrome causa um alargamento variável (dilatação) das vias urinárias inferiores. Ela vem acompanhada pela ausência (falta) parcial ou total dos músculos da parte lateral e inferior da parede da barriga. E, em meninos, os testículos não descem para a bolsa escrotal e não podem ser sentidos (não palpáveis) em ambos os lados.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Entender a doença
Do básico ao detalhe, leia no seu ritmo
Preparando trilha educativa...
Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 15 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 40 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
1 gene identificado com associação a esta condição. Padrão de herança: Autosomal dominant, Not applicable, X-linked recessive.
The muscarinic acetylcholine receptor mediates various cellular responses, including inhibition of adenylate cyclase, breakdown of phosphoinositides and modulation of potassium channels through the action of G proteins. Primary transducing effect is Pi turnover
Cell membranePostsynaptic cell membraneBasolateral cell membraneEndoplasmic reticulum membrane
Prune belly syndrome
A syndrome characterized by thin abdominal musculature with overlying lax skin, cryptorchism, megacystis with disorganized detrusor muscle, and urinary tract abnormalities.
Variantes genéticas (ClinVar)
66 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 64 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
3 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Síndrome "prune belly"
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
Pesquisa e ensaios clínicos
15 ensaios clínicos encontrados, 1 ativos.
Publicações mais relevantes
Management of an infant with prune belly syndrome under caudal anaesthesia undergoing laparoscopic surgery.
Prune belly syndrome (PBS) is a rare congenital disorder predominantly affecting male infants and is characterised by deficient abdominal musculature, urinary tract anomalies and bilateral cryptorchidism. Its multisystem involvement presents significant anaesthetic challenges. We report the anaesthetic management of a male infant with PBS posted for laparoscopic first-stage orchidopexy. Examination revealed a flabby, wrinkled abdomen with bulging flanks, easily palpable enlarged hydronephrotic kidneys, a hypoplastic scrotum and absent testes. After obtaining written informed consent and ensuring appropriate fasting, caudal anaesthesia was administered in the lateral position under ketamine sedation. Surgery was performed laparoscopically in the supine position. Adequate surgical conditions were achieved without airway instrumentation or neuromuscular blockade, and the perioperative course was uneventful. This case highlights that in carefully selected infants undergoing short, uncomplicated laparoscopic procedures, high-volume dilute caudal anaesthesia with sedation can be a safe and effective alternative to general anaesthesia in PBS.
Functional Reconstruction of Abdominal Wall in Prune Belly Syndrome Using Vastus Lateralis Muscle Flaps: A Prospective Observational Study.
Prune Belly Syndrome (PBS) is a rare congenital disorder characterized by severe abdominal wall muscle deficiency, leading to significant impairment of respiratory, urinary, and gastrointestinal function. Despite numerous reconstructive attempts, no technique has achieved full functional restoration of the anterior abdominal wall. This paper introduces a novel dynamic reconstructive approach using bilateral pedicled musculo-fascial Vastus Lateralis (VL) flaps for functional abdominal wall reconstruction in PBS. Unlike traditional abdominoplasty-based procedures that mainly address cosmetic deformity, this method focuses on restoring true muscle dynamics and physiological function. The technique involves harvesting innervated, vascularized VL flaps from both thighs and transposing them to the anterior abdominal wall through a subcutaneous tunnel while preserving their neurovascular pedicles. The muscles are anchored to the costal arch superiorly and the pubic symphysis inferiorly, effectively recreating the functional vector of the rectus abdominis. This configuration enables dynamic contraction of the abdominal wall and reinstates the ability to generate intra-abdominal pressure required for coughing, defecation, and postural control. Follow-up assessment, including electromyography (EMG) and motor testing, confirmed active muscle contraction and marked improvement in core function. Patients demonstrated restored abdominal wall activity, enhanced motor abilities, and reduced symptoms related to ineffective intra-abdominal pressure, such as constipation and respiratory infections. This study presents the first reproducible surgical solution achieving both anatomical reconstruction and functional reanimation of the abdominal wall in PBS. The described technique offers a transformative step in pediatric reconstructive surgery, combining microsurgical precision with physiological restoration to improve long-term quality of life.
Clinical Presentation and Long-Term Outcomes of Prune Belly Syndrome in a Tertiary Hospital.
Prune belly syndrome (PBS) is a rare congenital disorder defined by deficient abdominal musculature, urinary tract anomalies and cryptorchidism. Clinical presentation is variable, and extrarenal malformations are common. A retrospective descriptive study of seven patients diagnosed with PBS between 1990 and 2024 in a tertiary-care hospital was carried out. Clinical, biochemical, radiological, auxological and surgical data were reviewed. All patients had megacystis and abdominal muscle hypoplasia. Megalourethra was observed in three (43%), cryptorchidism in six (86%), hydronephrosis in all cases and renal dysplasia in four (57%). Extrarenal anomalies included congenital heart disease in three patients (43%), musculoskeletal defects in three (43%) and other malformations in two (28%). Mean serum creatinine level was 0.83 mg/dL at birth and 1.30 mg/dL at the last follow-up. Two patients (28%) required clean intermittent catheterisation, and one (14%) underwent renal transplantation at 14 years. Surgical procedures included orchidopexy in five patients (71%), vesicostomy in four (57%) and Mitrofanoff appendicovesicostomy in one patient. PBS is a rare disorder with a heterogeneous clinical spectrum. This cohort revealed a high prevalence of cardiac anomalies. Renal dysplasia, recurrent urinary tract infections and delayed surgical intervention were associated with progressive renal impairment. Management in centres with multidisciplinary teams is essential.
A Case Report of a Syndromic Triad of Persistent Urogenital Sinus, Herlyn-Werner-Wunderlich Syndrome, and Prune Belly Syndrome in a Neonate.
We present a case of a premature female neonate with a triad of persistent urogenital sinus with urinary ascites, bilateral hydrocolpos in a duplex uterus, and abdominal wall hypoplasia resembling Prune Belly-like syndrome, combined with severe bilateral cystic dysplastic kidneys and complex urinary obstruction. A female infant was born at 34 3/7 weeks' gestation via cesarean section due to prenatal detection of hydrops fetalis and massive urinary ascites. Prenatal ultrasound had shown oligohydramnios, ascites, megacystis, and hydrocolpos. Postnatally, urinary ascites, a persistent urogenital sinus, severe upper urinary tract dilation, bilateral dysplastic kidneys, and an obstructive vaginal septum were confirmed. Management included staged urinary drainage, vaginal septum incision, intensive respiratory and renal support, and multidisciplinary care. This unique combination of anomalies presents significant diagnostic and therapeutic challenges. Early recognition and individualized multidisciplinary management are essential to improve postnatal outcomes and guide long-term planning in such cases.
Clinical manifestations and management of prune-belly syndrome: A 20-year single center experience.
Prune Belly Syndrome (PBS) is a rare congenital disorder characterized by abdominal wall musculature deficiency, cryptorchidism, and urinary tract abnormalities. Clinical presentation is highly variable, with multisystem involvement and a wide range of urologic and renal outcomes. Long-term data from contemporary pediatric cohorts remain limited. We conducted a retrospective review of pediatric patients diagnosed with PBS and followed at a tertiary pediatric urology center between 2002 and 2024. Demographic, clinical, surgical, and urodynamic data were extracted from electronic records. Renal function was assessed by estimated glomerular filtration rate and CKD staging. Comparative analyses were performed between subgroups using Student's t-test or Fisher's exact test, where appropriate. Multivariate logistic regression was performed to evaluate predictors of progression to CKD and renal transplant. Sixty-four patients (61 males, 3 females) with a median follow-up of 12.6 years were included. Hydronephrosis (87.5 %), vesicoureteral reflux (73.4 %), and recurrent UTIs (76.6 %) were common. Nearly all patients underwent surgical intervention, most frequently orchidopexy (83.6 %), vesicostomy (23.4 %), and appendicovesicostomy (35.9 %). Abdominal wall reconstruction was performed in 45.3 % of patients. CIC was utilized by 35.9 %, typically via a continent catheterizable channel. Urodynamic studies demonstrated large, compliant bladders with impaired emptying. CKD developed in 48.4 % of patients, and 17.2 % required renal transplantation, with nadir serum creatinine in infancy predictive of long-term outcomes. PBS requires lifelong, multidisciplinary care. Early identification of patients at risk for CKD, proactive bladder management, and coordinated reconstructive surgery are critical to optimizing outcomes. These results reinforce the importance of integrating functional and quality-of-life goals into care planning. Future prospective studies are needed to refine surgical timing, preserve renal function, and better characterize long-term quality-of-life outcomes.
Publicações recentes
Laparoscopic nephroureterectomy in Prune Belly Syndrome: a case report.
Vaginal delivery in a woman with prune belly syndrome: a case report.
🥉 Relato de casoFunctional Reconstruction of Abdominal Wall in Prune Belly Syndrome Using Vastus Lateralis Muscle Flaps: A Prospective Observational Study.
🥉 Relato de casoClinical Presentation and Long-Term Outcomes of Prune Belly Syndrome in a Tertiary Hospital.
🥉 Relato de casoA Case Report of a Syndromic Triad of Persistent Urogenital Sinus, Herlyn-Werner-Wunderlich Syndrome, and Prune Belly Syndrome in a Neonate.
🥉 Relato de caso📚 EuropePMC518 artigos no totalmostrando 163
Functional Reconstruction of Abdominal Wall in Prune Belly Syndrome Using Vastus Lateralis Muscle Flaps: A Prospective Observational Study.
Plastic and reconstructive surgeryClinical Presentation and Long-Term Outcomes of Prune Belly Syndrome in a Tertiary Hospital.
Archivos espanoles de urologiaA Case Report of a Syndromic Triad of Persistent Urogenital Sinus, Herlyn-Werner-Wunderlich Syndrome, and Prune Belly Syndrome in a Neonate.
European journal of pediatric surgery reportsClinical manifestations and management of prune-belly syndrome: A 20-year single center experience.
Journal of pediatric urologyManagement of an infant with prune belly syndrome under caudal anaesthesia undergoing laparoscopic surgery.
BMJ case reportsUreteral versus appendiceal Mitrofanoff channels: a retrospective analysis of functional outcomes and complications.
BMC surgeryA case of prune belly syndrome with patent urachus: Pediatric surgical aspects of a rare case report.
Radiology case reportsRuptured spinal dural arteriovenous fistula with subdural hematoma: A case report.
Surgical neurology internationalDiagnostic and management challenges of prune belly syndrome in a low-income country: a neonatal case report.
Journal of surgical case reportsUnique Dental and Craniofacial Manifestations of Hypoplastic Amelogenesis Imperfecta in a Patient With Prune Belly Syndrome: A Rare Case Report.
Case reports in dentistryCase Report: A rare case of prune belly syndrome with intraperitoneal cryptorchidism.
Frontiers in pediatricsBeyond borders: Prune belly syndrome unveiled in a nine-year-old Pakistani boy.
JPMA. The Journal of the Pakistan Medical AssociationGeneration of induced pluripotent stem cell line, NCHi028-A, from a male child with Prune Belly Syndrome.
Stem cell researchCo-occurring non-urinary congenital anomalies among cases with congenital anomalies of the kidney and urinary tract.
European journal of medical geneticsAnatomic and Functional Reconstruction of the Abdominal Wall in Prune Belly Syndrome: A Case Report.
CureusOutcomes of Growth-Friendly Surgical Treatment of Early Onset Scoliosis in Children With Prune Belly Syndrome: A Preliminary Report.
Journal of pediatric orthopedicsLong-term outcomes of kidney replacement therapy in Australians with prune belly syndrome.
Journal of paediatrics and child healthA new spectrum of neonatal urethral pathologies in the era of early vesicoamniotic shunting?
World journal of urologyPrune belly syndrome in a neonate with severe bilateral hydronephrosis: A rare case report from Somalia.
International journal of surgery case reportsPrune belly syndrome: A rare case report.
Clinical case reportsPrune-belly Syndrome: An Update.
Current pediatric reviewsThe challenging diagnosis and management of the prune belly syndrome: A case report.
SAGE open medical case reportsCombined Double-breasted Full-thickness Abdominal Flap Plication and Acellular Dermal Matrix in Prune-belly Syndrome Reconstruction.
Plastic and reconstructive surgery. Global openNovel minimally invasive abdominoplasty for selected cases of prune belly syndrome: Step-by-step technique description and clinical indications.
Journal of pediatric urologyFertility prospects for the prune-belly patient: A scoping review.
Journal of pediatric urologySyndromic and single gene disorders associated with fetal megacystis (I): Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS).
Taiwanese journal of obstetrics & gynecologyChromosomal abnormalities associated with fetal megacystis.
Taiwanese journal of obstetrics & gynecologyCase of Urethral Hypoplasia in Prune Belly Syndrome Successfully Managed With Vesicoamniotic Shunts and a Progressive Augmentation by Dilation Urethra Anterior Procedure.
UrologyPIEZO1 loss-of-function compound heterozygous mutations in the rare congenital human disorder Prune Belly Syndrome.
Nature communicationsDIS3L2 Gene Mutation Causes the Perlman Syndrome of Overgrowth and Wilms Tumor Susceptibility.
CureusVolume of the normal prostate gland in polish boys, aged 1-17 years: Based on transabdominal ultrasound - Prospective study.
Journal of pediatric urologyTroubleshooting Tips for Diagnosing Complex Fetal Genitourinary Malformations.
Radiographics : a review publication of the Radiological Society of North America, IncPrune Belly Syndrome in a Female Newborn following In Vitro Fertilization-Induced Pregnancy: A Case Report and Literature Review.
Case reports in urologyKidney function and transplants in prune belly syndrome: a scoping review.
Pediatric nephrology (Berlin, Germany)Simultaneous second-stage Fowler-Stephens Orchiopexy and microvascular testicular autotransplantation in a patient with prune belly syndrome with cryptorchidism.
Journal of surgical case reportsRapid reversal of left ventricular enlargement after percutaneous closure of pulmonary sequestration in a newborn with prune belly syndrome.
Turk gogus kalp damar cerrahisi dergisiLower Urinary Tract Obstruction in Newborns.
Advances in pediatricsUse of whole genome sequencing to determine the genetic basis of visceral myopathies including Prune Belly syndrome.
Journal of rare diseases (Berlin, Germany)The Toronto nomogram: A Bayesian meta-regression derived prenatal ultrasound index to predict lower urinary tract obstruction and prune belly syndrome.
Prenatal diagnosisSingle-center outcome analysis of 46 fetuses with megacystis after intrauterine vesico-amniotic shunting with the Somatex®intrauterine shunt.
Archives of gynecology and obstetricsFetal Megacystis: Associated Structural Abnormalities and Obstetric Outcomes.
Fetal and pediatric pathologyA case of prune belly syndrome: Experience from a regional hospital in Togo.
International journal of surgery case reportsTechnical details and long-term outcomes of P.A.D.U.A. for congenital urethral narrowing; a case series and review of the literature.
Journal of pediatric urologyPrune-belly syndrome with severe lordosis.
Prenatal diagnosisManagement of Abdominal Wall Defects.
The Surgical clinics of North AmericaMedium and Long-Term Clinical Outcomes of Kidney Transplantation in Patients With Prune Belly Syndrome: A Single-Centre Experience.
UrologyPregnant patient with prune belly syndrome: case report.
Einstein (Sao Paulo, Brazil)Dental treatment of patients with prune belly syndrome.
Special care in dentistry : official publication of the American Association of Hospital Dentists, the Academy of Dentistry for the Handicapped, and the American Society for Geriatric DentistryStudy of Penis Structure and Development in Fetus With Prune Belly Syndrome.
UrologyRobotic treatment of ureteropelvic junction obstruction in Eagle-Barrett Syndrome.
The Canadian journal of urologyPrune Belly Syndrome Associated with Interstitial 17q12 Microdeletion.
Case reports in urologyComments on the paper Surgical approach to prune belly syndrome: A review of our series and novel surgical technique.
Journal of pediatric urologyThird Renal Transplant in a Patient With Prune Belly Syndrome.
CureusBladder Cancer in a Long-term Survivor of the Prune Belly Syndrome.
UrologyCase Report: Novel Copy Number Variant 16p11.2 Duplication Associated With Prune Belly Syndrome.
Frontiers in pediatricsSurgical approach to prune-belly syndrome: A review of our series and novel surgical technique.
Journal of pediatric urologyMP44-09 UNDERSTANDING PRUNE BELLY SYNDROME AT SINGLE CELL RESOLUTION.
The Journal of urologyRobotic abdominoplasty for abdominal wall lateral bulging repair in an adult prune belly syndrome patient: a novel approach.
Hernia : the journal of hernias and abdominal wall surgeryPrune belly syndrome in Finland - A population-based study on current epidemiology and hospital admissions.
Journal of pediatric urologyHypoplasia of abdominal wall muscles following massive fetal persistent chylous ascites without anemia.
Oxford medical case reportsModern management of and update on prune belly syndrome.
Journal of pediatric urologyA rare case report of prune belly syndrome with malnutrition.
Oxford medical case reports[Cryptorchidism in Prune Belly syndrome].
Le Mali medicalRespiratory support in the absence of abdominal muscles: A case study of ventilatory management in prune belly syndrome.
Paediatric respiratory reviewsA novel technique of abdominoplasty for prune belly syndrome.
African journal of paediatric surgery : AJPSMegacystis-microcolon-intestinal hypoperistalsis syndrome associated with cystic fibrosis and meconium peritonitis in a female neonate 4 days of age - case report and review of the literature.
Medicine and pharmacy reportsUnusual variant of pseudo prune belly syndrome.
BMJ case reportsImpact of the COVID-19 pandemic on the surgical activity of Pediatric Urology: analysis of postoperative complications according to the Clavien-Dindo classification.
Actas urologicas espanolasSuccessful sperm retrieval in prune belly syndrome.
Asian journal of urologyFetal bladder outflow obstruction: Interventions, outcomes and management uncertainties.
Early human developmentPrune perineum surgical correction - Treatment of a rare syndrome.
Journal of pediatric urologyPrune-Belly syndrome, a rare case presentation in neonatology: about one case in Yaounde, Cameroon.
The Pan African medical journalChallenging cases in urology: Hematuria in a man with prune belly syndrome.
Urology timesThe role of intra-abdominal pressure in human testicular migration.
International braz j urol : official journal of the Brazilian Society of UrologyAre pressure pop-offs beneficial to the bladder in boys with posterior urethral valves?
Journal of pediatric urologyDemographics and baseline care among newly transitioning adult congenital urology patients.
Journal of pediatric urologyA rare case of peritonitis in a young woman on peritoneal dialysis.
CEN case reportsGonadal Function and Reproductive System Anatomy in Postpubertal Prune-Belly Syndrome Patients.
UrologyChallenges in pediatric urologic practice: a lifelong view.
World journal of urologyPrune belly syndrome in surviving males can be caused by Hemizygous missense mutations in the X-linked Filamin A gene.
BMC medical geneticsAnterior and posterior rectus abdominis sheath stiffness in relation to diastasis recti: Abdominal wall training or not?
Journal of bodywork and movement therapiesPrune Belly Syndrome in the Pig (Sus scrofa domesticus).
Journal of comparative pathologyTwo cases of Prune Belly Syndrome from Kagera Region Tanzania.
The East African health research journalStudy of prostate growth in prune belly syndrome and anencephalic fetuses.
Journal of pediatric surgeryRenal Arterial Doppler Resistive Index in Differentiating Obstructive and Non-Obstructive Hydronephrosis in Children at Tikur Anbessa Specialized Hospital, Ethiopia, October, 2018.
Ethiopian journal of health sciencesPrune belly syndrome: current perspectives.
Pediatric health, medicine and therapeuticsLong term follow-up in a patient with prune-belly syndrome - a care compliant case report.
MedicineDo prune-belly syndrome and neural tube defects change testicular growth? A study on human fetuses.
Journal of pediatric urologyChallenging diagnosis of prune belly syndrome antenatally: a case report.
Journal of medical case reportsPseudo Prune Belly syndrome: a case report with unilateral abdominal defect.
Radiology case reportsA Neonate with Prune Belly Syndrome and Limb Reduction Defect.
Indian journal of pediatricsThe Cost of Uncertainty: A Patient's Perspective.
Journal of the American College of Radiology : JACRCongenital Disorders of the Human Urinary Tract: Recent Insights From Genetic and Molecular Studies.
Frontiers in pediatricsPfeiffer Syndrome Type 3 and Prune Belly Anomaly in a Female: Case Report and Review.
Fetal and pediatric pathologyLong-term Outcome of Renal Transplantation in Patients with Congenital Lower Urinary Tract Malformations: A Multicenter Study.
TransplantationDifferences in Linea Alba Stiffness and Linea Alba Distortion Between Women With and Without Diastasis Recti Abdominis: The Impact of Measurement Site and Task.
The Journal of orthopaedic and sports physical therapyPrenatal imaging of genital defects: clinical spectrum and predictive factors for severe forms.
BJU internationalAn Autopsy Case Report: Prune Belly Syndrome with Overlapping Presentation of Partial Urorectal Septum Malformation Sequence.
Turk patoloji dergisiA potential serious complication in infants with congenital obstructive uropathy: Secondary pseudohypoaldosteronism.
JPMA. The Journal of the Pakistan Medical AssociationPrune belly syndrome: Approaches to its diagnosis and management.
Intractable & rare diseases researchExploration of the fetus with gross anomaly: a case of pseudo prune belly syndrome.
Anatomy & cell biologyCopy number variations in a population with prune belly syndrome.
American journal of medical genetics. Part ALiving-related kidney transplantation with catheterizable urinary conduit in prune belly syndrome: A case report.
International journal of surgery case reportsCorrigendum: Vesicoamniotic Shunting Improves Outcomes in a Subset of Prune Belly Syndrome Patients at a Single Tertiary Center.
Frontiers in pediatricsPhenotypic severity scoring system and categorisation for prune belly syndrome: application to a pilot cohort of 50 living patients.
BJU internationalPrenatal renal parenchymal area as a predictor of early end-stage renal disease in children with vesicoamniotic shunting for lower urinary tract obstruction.
Journal of pediatric urologyManagement of Congenital Urethral Strictures In Infants. Case Series.
Urology journalVesicoamniotic Shunting Improves Outcomes in a Subset of Prune Belly Syndrome Patients at a Single Tertiary Center.
Frontiers in pediatricsCollagen I and III in women with diastasis recti.
Clinics (Sao Paulo, Brazil)Minimal Invasive Linea Alba Reconstruction for the Treatment of Umbilical and Epigastric Hernias with Coexisting Rectus Abdominis Diastasis.
Journal of laparoendoscopic & advanced surgical techniques. Part ADiastasis recti abdominis - a review of treatment methods.
Ginekologia polskaThree cases of prune belly syndrome at the Lagos State University Teaching Hospital, Ikeja.
Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi ArabiaPediatric patient with end-stage kidney disease secondary to Eagle-Barrett syndrome and metastatic unresectable hepatoblastoma treated successfully with chemotherapy and liver-kidney transplant.
Pediatric transplantationQuadratus lumborum block for post-operative pain relief in patient with Prune belly syndrome.
Indian journal of anaesthesiaPrune belly syndrome: A report of 15 cases from Sudan.
Sudanese journal of paediatricsUnusual presentation of prune belly syndrome: a case report.
Journal of medical case reportsRare copy number variants identified in prune belly syndrome.
European journal of medical geneticsA newborn with abdominal distension.
Journal of paediatrics and child health"Pseudo-prune belly syndrome": An under-reported phenotypic variant of prune belly syndrome.
Journal of pediatric urologyCombined laparoscopic and open technique for repair of congenital abdominal hernia: A case report of prune belly syndrome.
MedicineDiastasis of rectus abdominis muscles in low back pain patients.
Journal of back and musculoskeletal rehabilitationOutcomes of renal replacement therapy in boys with prune belly syndrome: findings from the ESPN/ERA-EDTA Registry.
Pediatric nephrology (Berlin, Germany)Type V Pouch Colon, Prune Belly Syndrome, and Congenital Anterior Urethrocutaneous Fistula.
Journal of neonatal surgeryStudy of Testicular Structure in Fetuses with Prune Belly Syndrome.
Advances in urologyStructural study of the bladder in fetuses with prune belly syndrome.
Neurourology and urodynamicsPrune Belly Syndrome with Sacrococcygeal Teratoma.
Indian journal of pediatricsPseudo Prune Belly Syndrome: Diagnosis Revealed by Imaging - A Case Report and Brief Review.
Polish journal of radiologyPrenatal detection of megacystis: not always an adverse prognostic factor. Experience in 25 consecutive cases in a tertiary referral center, with complete neonatal outcome and follow-up.
Journal of pediatric urologyAbdominoplasty in prune belly syndrome: Modifications in Monfort technique to address variable patterns of abdominal wall weakness.
Journal of pediatric urologyFetal megacystis: A systematic review.
Journal of pediatric urology[Video-assisted sigmoidectomy as an option for Sigmoid volvulus management in the pediatric population].
Cirugia pediatrica : organo oficial de la Sociedad Espanola de Cirugia PediatricaPrune Belly Syndrome with Situs Inversus Abdominus.
Journal of neonatal surgeryIt's not All Doom and Gloom: Prune Belly Syndrome Associated with VACTERL.
Journal of neonatal surgeryPATTERN AND OUTCOME OF RENAL DISEASES IN HOSPITALIZED CHILDREN IN TIKUR ANBESSA SPECIALIZED TEACHING HOSPITAL, ADDIS ABABA, ETHIOPIA.
Ethiopian medical journalWhat is the outcome of paediatric gastrocystoplasty when the patients reach adulthood?
BJU internationalFurther evidence of the etiology of prune belly syndrome provided by a transient massive intraabdominal cyst in a female.
Journal of pediatric surgeryClinical manifestations of prune belly syndrome.
Clinical medicine (London, England)Regarding commentary to: Detailed evaluation of the upper urinary tract in patients with Prune Belly Syndrome using magnetic resonance urography.
Journal of pediatric urologyGenetics of Vesicoureteral Reflux.
Current genomicsTrends in Renal Transplantation Rates in Patients with Congenital Urinary Tract Disorders.
The Journal of urologyCommentary to "Detailed evaluation of the upper urinary tract in patients with prune belly syndrome using magnetic resonance urography".
Journal of pediatric urologyProposed technique for open repair of a small umbilical hernia and rectus divarication with self-gripping mesh.
Hernia : the journal of hernias and abdominal wall surgeryDetailed evaluation of the upper urinary tract in patients with prune belly syndrome using magnetic resonance urography.
Journal of pediatric urologyIsolated hypoplasia of abdominal wall muscles associated with fetal ascites.
Congenital anomaliesCorset Usage for Gastrointestinal and Respiratory Problems in a Newborn with Prune Belly Syndrome.
Indian journal of pediatricsOUTCOMES OF POSTERIOR SAGITTAL ANORECTOPLASTY FOR HIGH ANORECTAL MALFORMATION IN BENIN CITY, NIGERIA.
Journal of the West African College of SurgeonsMegaureter detection through renal scintigraphy scan: Prune-Belly syndrome.
Revista espanola de medicina nuclear e imagen molecularHealth-related Quality of Life in Children With Prune-belly Syndrome and Their Caregivers.
UrologySuspected Urine Leak in a Pediatric Renal Transplant Patient With Prune Belly Syndrome.
Clinical nuclear medicineOutcomes of living donor renal transplantation in children with lower urinary tract dysfunction: a comparative retrospective study.
BJU international[Multiple bladder diverticula caused by occipital horn syndrome].
Archives de pediatrie : organe officiel de la Societe francaise de pediatrieCommentary to "27 years of experience with the comprehensive surgical management of prune belly syndrome".
Journal of pediatric urologyImpact and frequency of extra-genitourinary manifestations of prune belly syndrome.
Journal of pediatric urologyAbdominoplasty in Prune Belly Syndrome.
Journal of pediatric urology[Diastasis Recti Abdominis and Pregnancy].
PraxisCommentary to '27 years of experience with the comprehensive surgical management of prune belly syndrome'.
Journal of pediatric urology27 years of experience with the comprehensive surgical treatment of prune belly syndrome.
Journal of pediatric urologyClear cell adenocarcinoma of the bladder with intravesical cervical invasion.
BMJ case reportsThree-dimensional magnetic resonance imaging overlay to assist with percutaneous transhepatic access at the time of cardiac catheterization.
Annals of pediatric cardiologySeminoma in Cryptorchid Testis in Prune Belly Syndrome.
The Journal of urologyA First Description of Prune Belly Syndrome in Central Africa.
Pediatrics and neonatologyUrachal bladder in kidney transplanted patient with Prune Belly syndrome and patent urachus.
Revista espanola de medicina nuclear e imagen molecularAnesthetic experience of a patient with Prune-belly syndrome.
Korean journal of anesthesiologyAssociações
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Referências e fontes
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Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Management of an infant with prune belly syndrome under caudal anaesthesia undergoing laparoscopic surgery.
- Functional Reconstruction of Abdominal Wall in Prune Belly Syndrome Using Vastus Lateralis Muscle Flaps: A Prospective Observational Study.
- Clinical Presentation and Long-Term Outcomes of Prune Belly Syndrome in a Tertiary Hospital.
- A Case Report of a Syndromic Triad of Persistent Urogenital Sinus, Herlyn-Werner-Wunderlich Syndrome, and Prune Belly Syndrome in a Neonate.
- Clinical manifestations and management of prune-belly syndrome: A 20-year single center experience.
- Laparoscopic nephroureterectomy in Prune Belly Syndrome: a case report.
- Vaginal delivery in a woman with prune belly syndrome: a case report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:2970(Orphanet)
- OMIM OMIM:100100(OMIM)
- MONDO:0007032(MONDO)
- GARD:7479(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q250354(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
