ABSTRACT
Información sobre este síndrome congénito, en el que se presenta agenesia, atrofia o hipoplasia de los músculos de la pared abdominal, anomalías en el sistema urinario, criptorquidia bilateral, y otras malformaciones secundarias. Se considera a la ecografía el primer método de diagnóstico por imágenes que se debe efectuar inmediatamente al nacimiento, y a la vez se destaca la importancia del diagnóstico prenatal precoz.
Subject(s)
Prune Belly Syndrome/diagnosis , Prune Belly Syndrome/etiology , Prune Belly Syndrome/therapy , Prune Belly Syndrome , UltrasonographyABSTRACT
El síndrome de prune belly es una rara enfermedad congénita de causa desconocida. Se presenta el caso de un recién nacido a término, del sexo masculino y de un día de nacido, que fue remitido al servicio de urología pediátrica por presentar ausencia de los músculos de la pared anterior del abdomen (rectos anteriores), criptorquidia bilateral y gran globo vesical. A partir de los hallazgos del examen físico se planteó el diagnóstico de síndrome de prune belly. Se encontraron anomalías asociadas como escoliosis y agenesia del pie derecho. En el estudio radiológico del tracto urinario se confirmaron malformaciones congénitas como valva de uretra posterior y megavejiga con uretero-hidronefrosis bilateral. Los análisis de laboratorio confirmaron la afectación de la función renal y una infección urinaria asociada. Se practicó una cistostomía a cielo abierto. El paciente falleció a los 10 días a causa de las complicaciones de la insuficiencia renal(AU)
The prune belly syndrome is a congenital rare disease of unknown origin. The case of a one-day-old full- term male newborn infant that was referred to the pediatric urology service for presenting absence of the muscles of the anterior abdomen wall (anterior rectus muscle), bilateral cryptochordism and big vesical globe, was presented. Starting from the findings of the physical examination, the prune belly syndrome was diagnosed. Associated abnormalities such as scoliosis and agenesis of the right leg were found. In the radiological study of the urinary tract, congenital malformations as posterior urethra valve and megabladder with bilateral ureterohydronephrosis were confirmed. The lab tests corroborated the affectation of the renal function and an associated urinary infection. The patient died at 10 days as a result of the complications of renal failure(AU)
Subject(s)
Humans , Male , Infant, Newborn , Prune Belly Syndrome/diagnosis , Congenital Abnormalities/diagnosis , Renal Insufficiency/complications , Renal Insufficiency/mortalityABSTRACT
Posterior urethral valves [PUVs]. The most common congenital cause of lower urinary tract obstruction, have been described to occur in identical and nonidentical twins. Until now, reports have been published on 15 cases of PUVs. We report a new case of concordant PUVs in one set of male monochorionic twins with secondary Prune Belly Syndrome. The twins were born by elective cesarean section at 38 weeks of gestation to a 36-year-old mother, gravida 6, para 6. On ultrasound perfomed at 18 weeks's gestation, both fetuses showed signs of PUVs. At birth, physical examination of both revealed a secondary Prune Belly Syndrome [PBS]. Postnatal renal ultrasound confirmed the diagnosis of PUV. The two infants underwent transurethral resection of the valves after a cystoscopic evaluation of the urethra. Since this procedure. their voiding has been unremarkable with stable renal function and sterile urine until their discharge. We have documented a rare association between VUP and PBS in two monochiorionic twins. More studies are needed to throw light on the significance of the present associated anomalies
Subject(s)
Humans , Male , Twins, Monozygotic , Prune Belly Syndrome/diagnosis , Urethral Obstruction/congenitalABSTRACT
A retrospective investigation was conducted to describe clinical features and birth outcomes among babies with prune belly syndrome detected ante-natally and at birth at the University Teaching Hospital in Cameroon. A total of 11 cases were identified over a period of 13 years, and the majority of affected fetuses were male (10 of 11). Most of the mothers were less than 30 years of age, had singleton pregnancies, and about two-thirds were complicated by placenta praevia, genital infections, preeclampsia, and anaemia associated with hookworm infestations. The most prominent findings related to prune belly syndrome among our patients were clubfoot (45), pulmonary hypoplasia (27), Potter facies (27), imperforate anus (27), and arthrogryposis (18). About two-thirds of the affected pregnancies did not reach the age of viability either due to voluntary termination (three of seven cases) or spontaneous abortion (four of seven cases). Of the remaining four viable pregnancies, two cases each of stillbirth and neonatal death were recorded. Among pregnancies that were not voluntarily terminated (n = 8), multiple concomitant anomalies and bilateral renal lesions were apparently the cause of fetal/infant death in the majority of cases (75). In conclusion, Cameroonian babies with prune belly syndrome presented with clinical features that were comparable to those observed in developed nations
Subject(s)
Humans , Male , Female , Pregnancy , Infant, Newborn , Adult , Prune Belly Syndrome/etiology , Abnormalities, Multiple/diagnosis , Abnormalities, Multiple/mortality , Maternal Welfare , Infant Welfare , Pregnancy Complications/diagnosis , Pregnancy Complications/mortality , Statistics , Retrospective Studies , Fetus/abnormalities , Gestational Age , Infant Mortality , Muscle, Skeletal/abnormalities , Developed Countries , Developing Countries , Pregnancy Outcome , Prune Belly Syndrome/diagnosis , Prune Belly Syndrome/mortalityABSTRACT
Frecuencia: Uno en 50.000 nacimientos. Principales manifestaciones: Agenesia o Atresia de musculatura abdominal; Agenesia o Atresia de uretra; Megavejiga. Etiología: Algunos autores consideran una consecuencia de un defecto mesodérmico primario. Caso clínico: Controles obstétricos normales hasta la semana 16, con diagnóstico ecográfico de Megavejiga. Se realizan punciones con evacuación de orina fetal. Se realiza estudio cromosómico y bioquímico. Consideraciones terapéuticas: 1) Punciones con evacuación de orina para disminuir la presión retrógada del tracto urinario; 2) Derivación de vejiga a líquido amniótico, que trata además el oligoamnios y mejora el desarrollo pulmonar; 3) Corregir las alteraciones morfológicas. Conclusiones: Formar un equipo médico interdisciplinario. Informar a los padres y prevenir que a pesar del tratamiento puede ocurrir fallo renal o pulmonar irreversible. Importancia del diagnóstico ultrasónico precoz.
Subject(s)
Humans , Female , Pregnancy , Infant, Newborn , Fetus/abnormalities , Prenatal Diagnosis , Prune Belly Syndrome/complications , Prune Belly Syndrome/diagnosis , Prune Belly Syndrome/epidemiology , Prune Belly Syndrome/etiology , Prune Belly Syndrome/physiopathology , Prune Belly Syndrome/mortality , Prune Belly Syndrome/therapy , Prune Belly Syndrome , Ultrasonography, Prenatal , Abnormalities, Multiple , Diagnosis, Differential , Patient Care TeamABSTRACT
Revisando la literatura nacional encontramos escasa información al Síndrome de Prune Belly o Síndrome de Abdomen en Ciruela Pasa, lo que motivó nuestro interés en la revisión del tema a propósito de la presentación del primer caso hospitalizado en el Hospital "Dr. Plácido D. Rodríguez R.". Este síndrome se caracteriza por la ausencia o hipoplasia congénita de la pared abdominal, severa dilatación no obstructiva del tracto urinario y criptorquídia bilateral (1). Siendo una patología de etiología aún obscura y de díficil manejo, es importante su estudio dado la gravedad de los trastornos fisiopatológicos que ocasiona este complejo malformativo. Se presenta el caso de neonato masculino quien nació con dificultad respiratoria, hipoplasia de la pared abdominal, criptorquidia bilateral y la dilatación del tracto urinario. Se realizaron estudios ultrasonográficos y radiológicos que confirmaron el diagnóstico. La anomalía no urológica más importante fue dextrocardia.
Subject(s)
Male , Infant, Newborn , Infant, Newborn , Radiology , Prune Belly Syndrome/diagnosis , Ultrasonography , Urinary TractABSTRACT
En este informe se describen dos casos de la variante letal del síndrome de Prune Belly en que la obstrucción de la uretra produjo graves alteraciones del sistema urinario con distensión vesical y ureteral con repercusión secundaria en la pared abdominal y daño renal bilateral por displasia. Se comenta la etiopatogenia de este síndrome de acuerdo a la teoría embriológica del mesodermo, así como de la teoría de la secuencia de la obstrucción uretral en las etapas tempranas del desarrollo que repercuten en la pared abdominal. En estos casos descritos, el grado de obstrucción del tracto urinario repercutió en el desarrollo de malformaciones letales
Subject(s)
Humans , Male , Infant, Newborn , Urinary Tract/abnormalities , Urinary Tract/pathology , Urethral Obstruction/diagnosis , Urethral Obstruction/pathology , Pathology, Clinical/methods , Prune Belly Syndrome/diagnosis , Prune Belly Syndrome/pathologyABSTRACT
Megalourethra, a rare congenital disorder involving the anterior urethra, is subdivided into two types: Fusiform and scaphoid. Two cases of scaphoid type megalourethra are reported. The first patient, a 5-weeks-old infant diagnosed at birth as having the prune belly syndrome was admitted to the hospital with vomiting and failure to thrive. Intravenous pyelogram revealed marked dilatation of the left ureter and a bladder diverticulum. A penile urethrostomy was performed. The second patient, born to healthy parents after an uneventful pregnancy and delivery, was found to have an enlarged and deformed penis. The baby voided with a poor stream and a concomitant swelling of the penis was noted. Retrograde uretrography showed a sac-like dilatation of the penile urethra. Surgical revision was carried out a 2 stage procedure and was completed (Nesbitt's operation) after 4 months. The patient did well postoperatively and voided with a normal stream without any abnormality of the penile shaft.
Subject(s)
Cystoscopy , Diagnosis, Differential , Female , Humans , Infant , Infant, Newborn , Male , Penis/abnormalities , Pregnancy , Prune Belly Syndrome/diagnosis , Urethra/abnormalities , UrographyABSTRACT
Consideramos importantes presentar el síndrome de abdomen en ciruelas pasa (Prune-Belly), porque a pesar de ser fácil de diagnosticar, puede pasar desapersibido al clínico debido su baja incidencia, conociéndose solamente 300 casos en la Literatura Mundial
Subject(s)
Infant, Newborn , Humans , Male , Prune Belly Syndrome , Prune Belly Syndrome/diagnosisABSTRACT
Se presenta un caso de Síndrome de Prune-Belly cuyo examen ecográfico pre-natal muestra hidrops, ascitis fetal transitoria y anomalías del sistema urinario. El diagnóstico del Síndrome fue hecho al nacer y confirmado por los exámenes de laboratorio. Se revisa la literatura y se enfatiza la posibilidad de sospechar este diagnóstico, cuando ciertos signos ecográficos aparecen