Challenges in the surgical treatment of a 8-year old child living with Prune Belly syndrome: case report / Desafios no tratamento tardio da síndrome de Prune Belly: relato de caso

Prune Belly syndrome, also known as Eagle-Barret syndrome, is a rare disease, with a prevalence of 3.8 live births per 100,000 births. Its main characteristic is the hypoplasia of the abdominal muscles, giving rise to the name "prune belly syndrome". The gold standard treatment is surgery, ideally with correction of cryptorchidism and phimosis between 6 to 18 months of life. Correction of urinary malformations and abdominoplasty should be performed up to 4 years of age. Little evidence exists in the literature about late treatment and its implications for prognosis. The context mentioned above led us to present an uncommon case of a six years old child in which a surgical approach was performed later than usual.

Introdução: A síndrome de Prune Belly é uma doença rara, com prevalência de 3,8 nascidos vivos a cada 100 mil nascimentos. Tem como principal caraterística a hipoplasia da musculatura abdominal gerando a origem do nome "síndrome da barriga de ameixa". O tratamento padrão ouro é cirúrgico, idealmente com correção da criptorquidia e fimose entre 6 a 18 meses e a correção das malformações urinárias e abdominoplastia devem ser realizadas até os 4 anos. Aqui descreve-se um caso ainda menos comum, em que foi realizada abordagem cirúrgica de forma mais tardia que o habitual. Há pouca evidência na literatura acerca do tratamento tardio e suas implicações em relação ao prognóstico.

Case report of prune belly syndrome: An airway challenge too?

Prune belly syndrome (PBS) is a rare congenital disease presenting a characteristic triad of abdominal muscle deficiency, urinary tract abnormality, and cryptorchidism with associated anomalies of various systems. We report the anesthetic management of an infant with PBS with no obvious facial deformity in whom we encountered an unanticipated difficult airway. Multiple attempts to secure the airway with different supraglottic airway devices were unsuccessful and the trachea could be intubated only on the second attemp

Pregnant patient with prune belly syndrome: case report

ABSTRACT Prune belly syndrome is a rare congenital disease of unknown etiology that is present in one in every 40 thousand live births, and predominantly affects males, at a ratio of 4:1. In males, it presents with anomalies in the urinary system, absence of abdominal muscles, bilateral cryptorchidism, and infertility. In women, the syndrome has variable presentations, but fertility is preserved. Searching the medical literature, we found only one case of prune belly syndrome in pregnant women. Therefore, the patient in this report is the second case. She was primiparous, 25-years-old, with no abdominal muscles, severe congenital kyphoscoliosis, and pulmonary restriction. Elective cesarean section was performed at 37 weeks of gestation due to maternal risk of uterine rupture by transverse presentation and fetal risk of intrauterine growth restriction. The pre-anesthetic approach defined that general anesthesia might have more risks for the patient due to severe maternal lung disease compared to ultrasound-guided locoregional anesthesia. During prenatal care, there were some maternal complications, such as asthma exacerbations, abdominal pain, and constipation. The newborn was born small for gestational age and this can possibly be explained by maternal restrictive lung capacity. The newborn presented with Apgar score 8/9 and tachypnea, but improved after two hours of life.

Síndrome de Prune-Belly. Presentación de un caso / Prune-Belly Syndrome. Presentation of a case / Síndrome de Prune-Belly. Apresentação de um caso

RESUMEN Introducción: el síndrome Prune-Belly (SPB) o síndrome de "abdomen en ciruela pasa", también conocido como el síndrome de Eagle Barrett, es una forma de uropatía obstructiva fetal (UOF). Su incidencia se calcula entre 1/40 000 y 1/50 000 nacidos vivos. Presentación de caso: es un caso de síndrome de Prune-Belly en un lactante de 36 días de nacido, parto extrahospitalario. Clínicamente presentó abdomen distendido con piel arrugada o en ciruela pasa, criptorquidia bilateral, signos y síntomas de insuficiencia respiratoria. En la ultrasonografía se constató la ausencia de musculatura en la pared abdominal, megacisto, dilatación quística de los uréteres, riñones con cambios quísticos y displásicos. Discusión: en cuanto a la supervivencia, en numerosos estudios se encuentra que el porcentaje de mortalidad en los primeros meses de vida es del 27%. Está caracterizado por una clásica tríada: ausencia congénita de la musculatura de la pared abdominal; anormalidades del tracto urinario y criptorquidia bilateral, descrita en la literatura revisada, lo que coincide con las características clínicas del caso presentado,con una proporción masculino femenino de 20:1. El mayor riesgo se ha descrito en gemelos y afroamericanos. Conclusiones: está caracterizado por una clásica tríada: ausencia congénita de la musculatura de la pared abdominal; anormalidades del tracto urinario y criptorquidia bilateral. Es más frecuente en el sexo masculino. El diagnóstico del SPB puede realizarse durante la gestación a través de la ecografía obstétrica. La mortalidad en los primeros meses de vida es alta.

ABSTRACT Introduction: Prune-Belly syndrome (PBS) or "prune abdomen" syndrome, also known as Eagle Barrett syndrome, is a form of fetal obstructive uropathy (UOF). Its incidence is estimated between 1/40,000 and 1/50,000 live births. Case presentation: it is a case of Prune-Belly syndrome in a 36-day-old infant, out-of-hospital delivery. Clinically, she presented a distended abdomen with wrinkled or prune skin, bilateral cryptorchidism, signs and symptoms of respiratory failure. Ultrasonography revealed the absence of musculature in the abdominal wall, megacyst, cystic dilatation of the ureters, kidneys with cystic and dysplastic changes. Discussion: regarding survival, numerous studies have found that the percentage of mortality in the first months of life is 27%. It is characterized by a classic triad: congenital absence of the muscles of the abdominal wall; urinary tract abnormalities and bilateral cryptorchidism, described in the reviewed literature, which coincides with the clinical characteristics of the case presented, with a male-female ratio of 20: 1. The highest risk has been described in twins and African Americans. Conclusions: it is characterized by a classic triad: congenital absence of the muscles of the abdominal wall; urinary tract abnormalities and bilateral cryptorchidism. It is more common in males. The diagnosis of PBS can be made during pregnancy through obstetric ultrasound. Mortality in the first months of life is high.

RESUMO Introdução: A síndrome de Prune-Belly (PBS) ou síndrome do "abdômen de ameixa", também conhecida como síndrome de Eagle Barrett, é uma forma de uropatia obstrutiva fetal (UOF). Sua incidência é estimada entre 1 / 40.000 e 1 / 50.000 nascidos vivos. Apresentação do caso: trata-se de um caso de síndrome de Prune-Belly em lactente de 36 dias, parto fora do hospital. Clinicamente, apresentava abdome distendido com pele enrugada ou ameixada, criptorquidia bilateral, sinais e sintomas de insuficiência respiratória. A ultrassonografia revelou ausência de musculatura na parede abdominal, megacisto, dilatação cística dos ureteres, rins com alterações císticas e displásicas. Discussão: em relação à sobrevida, vários estudos constataram que o percentual de mortalidade nos primeiros meses de vida é de 27%. É caracterizada por uma tríade clássica: ausência congênita dos músculos da parede abdominal; anormalidades do trato urinário e criptorquidia bilateral, descritos na literatura revisada, que coincidem com as características clínicas do caso apresentado, com relação homem-mulher de 20: 1. O maior risco foi descrito em gêmeos e afro-americanos. Conclusões: caracteriza-se por uma tríade clássica: ausência congênita dos músculos da parede abdominal; anormalidades do trato urinário e criptorquidia bilateral. É mais comum em homens. O diagnóstico de PBS pode ser feíta durante a gravidez por meio de ultrassom obstétrico. A mortalidade nos primeiros meses de vida é alta.

The role of intra-abdominal pressure in human testicular migration

ABSTRACT Objectives: This review aims to study the role of the abdominal wall in testicular migration process during the human fetal period. Materials and Methods: We performed a descriptive review of the literature about the role of the abdominal wall in testicular migration during the human fetal period. Results: The rise in intra-abdominal pressure is a supporting factor for testicular migration. This process has two phases: the abdominal and the inguinal-scrotal stages. The passage of the testis through the inguinal canal occurs very quickly between 21 and 25 WPC. Bilateral cryptorchidism in Prune Belly syndrome is explained by the impaired contraction of the muscles of the abdominal wall; mechanical obstruction due to bladder distention and structural alteration of the inguinal canal, which hampers the passage of the testis during the inguinoscrotal stage of testicular migration. Abdominal wall defects as gastroschisis and omphaloceles are associated with undescended testes in around 30 to 40% of the cases. Conclusions: Abdominal pressure wound is an auxiliary force in testicular migration. Patients with abdominal wall defects are associated with undescendend testis in more than 30% of the cases probably due to mechanical factors; the Prune Belly Syndrome has anatomical changes in the anterior abdominal wall that hinder the increase of intra-abdominal pressure which could be the cause of cryptorchidism in this syndrome.

Seudo síndrome prune belly / Pseudo Prune-belly syndrome

Introducción: El síndrome prune belly es una rara y compleja anomalía congénita y el seudo prune belly es aún más raro y en muchas ocasiones no se diagnostica como tal. Objetivo: Describir un paciente con las características de este síndrome. Presentación del caso: Feto de sexo masculino que en la semana 23 del embarazo el ultrasonido prenatal detectó dilatación pélvica bilateral y se propuso la interrupción del embarazo que no fue aceptada por los padres. Al nacer se observa criptorquidia bilateral, el ultrasonido renal a las 34 horas de nacido confirma dilatación pélvica bilateral severa, se indica profilaxis con cefalexina, y a los cuatro dias de nacido es enviado a consulta de nefrología. Al llegar a consulta se observa la ausencia de testículos en ambas bolsas escrotales y el ultrasonido abdominal a los 16 dias confirma dilatación pélvica renal derecha severa y uréter dilatado en su tercio superior con 6 mm de diámetro y ureteropielocaliectasia severa del lado izquierdo; no se visualiza testículo derecho y el izquierdo en canal inguinal mide 7 × 10 mm. La gammagrafía estática demostró hipocaptación marcada del radiofármaco por el riñón izquierdo y disminución de la función renal relativa y en la uretrocistografía miccional se encontró dilatación vesical y reflujo vesicoureteral con ureterohidronefrosis bilateral. Conclusiones: El seudo síndrome prune belly presenta alteraciones complejas del tracto urinario que son las que establecen el pronóstico en estos casos(AU)

Introduction: Prune belly syndrome is a rare and complex congenital anomaly and the pseudo prune belly is even rarer and often not diagnosed as such. Objective: To describe a patient with the characteristics of this syndrome. Presentation of the case: Male fetus that in the 23rd week of pregnancy it was detected a bilateral pelvic dilatation in the prenatal ultrasound; and it was proposed the termination of pregnancy which was not accepted by the parents. At birth it is observed bilateral cryptorchidism; the renal ultrasound at 34 hours after birth confirmed bilateral severe pelvic dilation, it is indicated prophylaxis with cephalexin, and at four days after birth, the newborn is remitted to the Nephrology consultation. In the consultation it was noticed the absence of scrotal sacs in both testicles and the abdominal ultrasound at 16 days after birth confirmed severe right renal pelvic dilatation and dilated ureter in its upper third with 6 mm of diameter and severe uretero-pielocaliectasy in the left side; it is not visualized the right testicle and the left in the inguinal channel measured 7 × 10 mm. Static scintigraphy showed marked hypocaptation of the radiopharmaceutical by the left kidney and decreased relative renal function, and in the voiding cystourethrogram it was found bladder dilation and vesicoureteral reflux with bilateral ureterohydronephrosis. Conclusions: The pseudo prune belly syndrome presents complex abnormalities of the urinary tract that are those which establish the prognosis in these cases(AU)

Trasplante renal en paciente con pared abdominal compleja: síndrome de abdomen en ciruela pasa / Kidney transplant in a patient with a complex abdominal wall: Prune belly syndrome

Introducción. Se presenta un paciente con falla renal crónica secundaria a hidroureteronefrosis bilateral, con antecedente de síndrome de abdomen en ciruela pasa (prune belly), en quien se realiza trasplante renal. El objetivo de este reporte de caso es exponer una anomalía congénita, con baja incidencia mundial y en menor proporción asociado a trasplante renal, con énfasis en la dificultad para la técnica quirúrgica dada por la hipoplasia de los músculos de la pared abdominal. Métodos. Revisión de Historia clínica, Consentimiento Informado. Búsqueda de la literatura.Caso clínico. En cirugía se identifica ausencia de músculos de la pared abdominal y fibrosis de las venas Iliaca externa y común derecha. Se realiza abordaje bajo técnica de Gibson contralateral, con trasplante renal sin complicaciones. Presentó adecuada evolución en seguimiento ambulatorio. Discusión. El síndrome de abdomen en ciruela pasa (prune belly) es una anomalía congénita de baja incidencia, con pocos reportes asociados a trasplante renal. El reto quirúrgico está dado por la ausencia de músculos de la pared abdominal, que aumentan los riesgos por posibles complicaciones, como la lesión de los vasos epigástricos inferiores. Para el cierre de pared abdominal en trasplante renal, se describe en dos planos, que en este caso se realiza sobre fascia muscular y piel. Aun siendo un reto, el paciente presenta adecuada evolución postoperatoria

Introduction. We present a patient with chronic renal failure secondary to bilateral hydroureteronephrosis, with a history of prune belly syndrome, who underwent a kidney transplant. The objective of this case report is to expose a congenital anomaly, with a low worldwide incidence and to a lesser extent associated with kidney transplantation, with emphasis on the difficulty for the surgical technique due to the hypoplasia of the abdominal wall muscles.Methods. Review of clinical history, Informed Consent. Literature search.Clinical case. Absence of abdominal wall muscles and fibrosis of the external iliac veins and right common veins were identified intraoperatively. An approach was performed under the contralateral Gibson technique, with uncomplicated kidney transplantation. He presented adequate evolution in outpatient follow-up.Discussion. Prune belly syndrome is a low-incidence congenital anomaly, with few reports associated with kidney transplantation. The surgical challenge is given by the absence of abdominal wall muscles, which increases the risks due to possible complications, such as injury to the inferior epigastric vessels. For the closure of the abdominal wall in kidney transplantation, it is described in two planes, which in this case is performed on muscle fascia and skin. Despite being a challenge, the patient has adequate postoperative evolution

Follow up of Patients with Eagle-Barett (Prune Belly) Syndrome Treated with Single-Stage Abdominoplasty and Total Genitourinary Tract Reconstruction / Seguimiento de pacientes con síndrome de Eagle-Barret (prune belly) tratados con abdominoplastia en un solo tiempo y reconstrucción total del tracto genitourinario

Introduction The Prune Belly Syndrome (PBS) is the deficiency or congenital hypoplasia of the abdominal muscles accompanied with disorders of the urinary tract. The surgical treatment for the uropathy of the PBS seeks to correct the anatomical defects that induce damage to the urinary tract, including the improvement of the bladder emptying. The correction of the abdominal wall defect must be considered as an important part of the treatment. Objective To describe of the most relevant clinic characteristics and outcomes of a group of eight patients with PBS managed with abdominoplasty and reconstructive urologic surgery. Methods Retrospective review of the medical charts of patients with PBS at Hospital Universitario San Ignacio, Hospital Militar Central of Bogotá, Clinica Infantil Colsubsidio and Fundación Oftalmológica Ardila Lulle of Bucaramanga, (Colombia) that were managed with reconstructive surgery of the urinary tract and Monfort technique abdominoplasty (between 2006 and 2016) by one of the authors (JPN). The evaluated variables included: phenotypic appearance, renal function, bowel movements, and urinary tract infection (UTI) episodes. These variables as well as the abdominal wall aspect, were evaluated postoperatively. Results There were 11 patients identified with PBS between 2006 and 2016. Eight (8) of them underwent reconstructive urologic surgery, orchidopexy and Monfort technique abdominoplasty. After the procedures, none of the patients presented deterioration of the renal function, and they reported a better quality of the stools according to the Bristol scale; there were also less episodes of UTI. The phenotypic aspect of the abdominal wall was qualified as satisfactory in all cases. Conclusions Reconstructive urologic surgery and abdominoplasty in patients with PBS is an excellent choice of management that reduces the number of UTIs, improves bowel movements according to the Bristol scale, and preserves the renal function. This management approach also improves the phenotypic aspect of the abdominal wall. All of this leads to higher survival rate and better quality of life.

Introducción El Síndrome de Prune Belly (SPB) es la deficiencia o hipoplasia congénita de los músculos abdominales acompañada de desórdenes del tracto genitourinario. El manejo quirúrgico de la uropatía del SPB busca corregir los defectos anatómicos que inducen daño al tracto urinario, incluyendo la mejora del vaciamiento vesical. La corrección del defecto de la pared abdominal debe considerarse como una parte importante del manejo. Objetivos Describir las características clínicas más relevantes y el desenlace de un grupo de 8 pacientes con SPB manejados con abdominoplastia y cirugía urológica reconstructiva. Materiales y Métodos Revisión retrospectiva de las historias clínicas de los pacientes con SPB del Hospital Universitario San Ignacio, Hospital Militar Central de Bogotá, Clínica Infantil Colsubsidio, y Fundación Oftalmológica Carlos Ardila Lulle de Bucaramanga (Colombia), manejados con cirugía reconstructiva de las vías urinarias y abdominoplastia tipo Monfort entre los años 2008 y 2016 por uno de los autores (JPN). Las variables incluidas fueron: aspecto fenotípico, función renal, hábito intestinal y características de las deposiciones, y episodios de infección urinaria. El desenlace de estas variables y el aspecto de la pared abdominal fueron evaluados en el postoperatorio. Resultados se identificaron 11 pacientes con SPB entre 2006 y 2016. Ocho (8) fueron sometidos a cirugía reconstructiva de las vías urinarias, orquidopexia y abdominoplastia tipo Monfort. Después de los procedimientos, ninguno de los pacientes presentó deterioro de la función renal; los pacientes refieren mejor calidad de las deposiciones según la escala de Bristol; se presentaron menos episodios de infección de vías urinarias. El aspecto fenotípico fue calificado como satisfactorio en todos los casos. Conclusiones La cirugía urológica reconstructiva acompañada de abdominoplastia en pacientes con SPB es una excelente forma de manejo que reduce el número de infecciones urinarias, mejora la calidad del hábito intestinal según la escala de Bristol, y conserva la función renal. Este manejo mejora además el aspecto fenotípico de la pared abdominal. Todo esto lleva a una mayor sobrevida y mejor calidad de vida.

Exploration of the fetus with gross anomaly: a case of pseudo prune belly syndrome / 대한해부학회지

Prune belly syndrome is a rare congenital anomaly usually presented with triad characteristic feature of deficient abdominal muscles, cryptorchidism, and urinary tract anomalies. Here, we present a case with all the characteristic features of prune belly and the associated secondary features which were observed on detailed dissection and exploration of the fetus.

Primer reporte de caso de Síndrome de Prune Belly a 3320 msn. Huancayo-Perú

Se describe a un neonato con Síndrome Prune belly, de sexo masculino, nacido de 39 semanas edad gestacional y peso 3570gr. Al examen físico presentó fascies no características, tórax alado, abdomen asimétrico, con redundancia de piel que va hacia ambos lados, no se aprecia tono muscular, se palpa fácilmente asas intestinales, presencia de borborismos y ruidos hidroaéreos conservados, ausencia de testículos en bolsas escrotales, el examen cardiovascular y de extremidades fueron aparentemente normales. La ecografía mostró hidronefrosis renal bilateral y testículos intraabdominales.

We describe a neonate with Prune belly syndrome, male, born 39 weeks gestational age and 3570gr weight. Physical examination showed non-characteristic fascities, winged chest, asymmetrical abdomen, with skin redundancy going to both sides, no muscle tone, intestinal handles easily palpated, presence of borborisms and preserved airflow sounds, absence of testicles in scrotal sacs, Cardiovascular and limb examination were apparently normal. Ultrasound showed bilateral renal hydronephrosis and intra-abdominal testes.

Imaging analysis of ultrasound in the diagnosis of Prune belly syndrome during the early second-trimester / 中华医学超声杂志（电子版）

Objective To investigate the sonographic characteristics of prune belly syndrome (PBS) in early period of second trimester. Methods A total of 16 pregnancies with diagnosis of PBS were enrolled between January 2014 and March 2017. Their sonographic characteristics and autopsy outcomes were analyzed. Results Overdistension of fetal bladder and flimsy fetal abdominal wall were found in all 16 cases. And there were 5 cases found prenatally to be associated with other abnormalities, including single umbilical artery (2 cases), sacrococcygeal teratoma (1 case), spina bifida manifesta (1 case) and strephenopodia (1 case). All women decided to terminatethe pregnancy, and deficiency of the abdominal musculature and increased collagen fiber were found by autopsy. Because of the confusion of the huge bladder, one case associated with imperforate anus was missed by prenatal ultrasonography. Conclusion Prenatal diagnosis of PBS in early period of second trimester is very important, because the amniotic fluid volume is still enough to evaluate the fetal structure.

De las bases embriológicas a la clínica en el síndrome de Prune Belly / From the embryological basis to the clinical Prune Belly syndrome

El síndrome de Prune Belly es un trastorno congénito, que obedece según lo reportado actualmente a una base genética. Está caracterizado por la siguiente triada: deficiencia en grados variables de la musculatura abdominal, criptorquidia bilateral y anomalías del tracto urinario. Se identifican dos variantes del síndrome, una mortal y otra compatible con la vida(AU)

Prune Belly syndrome is a congenital disorder that is due, as currently reported, to genetic basis. It is characterized by the following triad: deficiency of abdominal muscles in varying degrees, bilateral cryptorchidism and urinary tract anomalies. Two variants of the syndrome are identified, a deadly one and another compatible with life(AU)

Síndrome de Prune Belly: Diagnóstico y manejo

El síndrome de Eagle-Barret o síndrome de Prune Belly también conocido como "síndrome de abdomen en ciruela pasa", es un cuadro cuya etiología aún se desconoce. Se caracteriza por la de ciencia de la musculatura de la pared abdominal, dilatación del tracto urinario y criptorquidia bilateral. La incidencia estimada es de 1 en 35,000 a 1 en 50,000 recién nacidos vivos, ocurriendo aproximadamente más del 95% de los casos en masculinos. Las manifestaciones clínicas de la triada de anormalidades presentes en esta entidad, van desde el defecto muscular presente en la pared abdominal, que consta solamente de piel y peritoneo, razón por la cual el tejido dérmico presenta una serie de estriaciones, que permiten la palpación fácil de las vísceras, asociándose además una alteración del sistema urogenital que se caracteriza por la dilatación de la vejiga como consecuencia de una obstrucción distal, con reflujo vesico-ureteral. Se describe un neonato con Síndrome de abdomen en ciruela pasa, de sexo masculino, nacido de 40 semanas de gestación y pesó 3 050 g al nacer. El examen clínico mostró un neonato con un abdomen globuloso, con redundancia de la piel que huelga a los costados rebosantes, la palpación del abdomen no descubre la tonalidad muscular. No se palpa visceromegalia y hay presencia normal de ruidos hidroaéreos. El examen físico del tórax y cardiovascular no reportó datos patológicos. Examen neurológico normal. La ecografía renal mostró alteraciones en el tracto urinario. Revela además criptorquidia bilateral. Este caso destaca la importancia del diagnóstico temprano y el manejo adecuado del paciente tanto a nivel primario como en el tercer nivel de atención; ya que si ofrecemos el manejo adecuado podemos brindarle a este tipo de pacientes un mejor pronóstico de vida.

Eagle-Barrett syndrome, formerly known as prune-belly syndrome, it's a disease whose etiology is still unknown. It's characterized by deficiency of abdominal wall musculature,a dilated urinary tract,and bilateral cryptorchidism. The estimated incidence is 1 in 35,000 to 1 in 50,000 live births, with more than 95% of cases occurring in males. Clinical manifestations of the triad of abnormalities present in this entity, ranging from muscle defect present in the abdominal wall, consisting only of skin and peritoneum, reason why the dermal tissue presents a series of striations, allowing easy palpation of the viscera, further associating an alteration of the urogenital system characterized by the expanded bladder following a distal obstruction, with vesicoureteral reflux. We report the case of a male neonate born after 40 weeks of gestation with a body weight of 3 050 g with Prune Belly syndrome. The physical examination revealed a distended abdomen with redundant skin that wrinkle like a prune, no visceromegaly was found and normal abdominal sounds were heard. The examination of thorax and cardiovascular systems were normal. The neurologic exam was normal. A renal ultrasound revealed alteration of the urogenital system. Bilateral cryptorchidism was also observed. This case highlights the importance of early diagnosis andappropriatemanagement both in primary health as tertiary,because if we bring the adequate treatment we will be able to o er to this patients a better life prognosis.

Síndrome de abdomen en ciruela pasa y dextrocardia, asociación infrecuente / Prune belly syndrome and dextrocardia, an infrequent association

Se describe un neonato con Síndrome de abdomen en ciruela pasa, de sexo masculino, nacido de 36 semanas de gestación y pesó 3 650 g al nacer. El examen clínico mostró un neonato con un abdomen globuloso, con redundancia de la piel que huelga a los costados rebosantes, la palpación del abdomen no descubre la tonalidad muscular. No se palpa visceromegalia y hay presencia normal de ruidos hidroaéreos. El examen físico del tórax y cardiovascular fueron aparentemente normales. Normocéfalo y facies no peculiar. La radiografía de tórax muestra dextrocardía. La ecografía renal mostró dilatación pielocalicial. Revela además criptorquidia bilateral. (AU)

We report the case of a male neonate born after 36 weeks of gestation with a body weight of 3 650 g with Prune belly Syndrome. The physical examination revealed a distended abdomen with redundant skin that wrinkle like a prune, no visceromegaly was found and normal abdominal sounds were heard. The examination of thorax and cardiovascular systems were apparently normal. The neurologic exam was normal. The chest x-ray revealed dextrocardia. A renal ultrasound revealed pyelocalyceal dilation. Bilateral cryptorchidism was also observed. (AU)

Síndrome de Prune Belly: diagnóstico y manejo pre y posnatal. Presentación de dos casos / Prune Belly syndrome: diagnosis and prenatal and postnatal management. Report of two cases

El síndrome de Prune Belly es una anomalía congénita rara, caracterizada por presentar: hipoplasia de músculos de pared abdominal, anomalía del tracto urinario y criptorquidia bilateral, el cuadro clínico que desencadena en el recién nacido es variable, dependiendo sobre todo del grado de alteración del sistema urinario. La ultrasonografía es el método de elección en el diagnóstico prenatal. El manejo prenatal de este cuadro, está dirigido fundamentalmente a mejorar la función renal y pulmonar, siendo el tratamiento de elección la descompresión vesico-amniótico temprano a través de la colocación de un catéter doble pigtail. El siguiente artículo presenta 2 casos clínicos que muestran las principales formas clínicas de presentación, diagnóstico prenatal ultrasonográfico y manejo intraútero y postnatal de esta patología.

Prune belly syndrome is a rare congenital anomaly, characterized by having: hypoplasia of abdominal wall muscles, urinary tract anomalies and bilateral cryptorchidism, clinical triggers in newborns varies, depending primarily on the degree of disturbance urinary system. Ultrasonography is the method of choice in prenatal diagnosis. The prenatal management of this picture, is aimed primarily at improving renal function and lung being the treatment of choice vesico-amniotic decompression early through the placement of a double pigtail catheter. This article presents 2 cases showing the main clinical presentations, diagnostic ultrasound and prenatal intrauterine and postnatal management of this condition.

Prune belly syndrome (sequenze): a case report.

The Prune Belly syndrome (PBS) also known as Eagle Barret syndrome is a rare disorder. It is an abdominal muscles deficiency syndrome characterized by a Triad syndrome i.e. deficiency of abdominal wall muscles, failure of testicular descent and dilation of the urinary tract. This syndrome has derived its name from the wrinkled prune appearance of the abdominal wall. Prune Belly syndrome is a rare anomaly seen in one in 35,000-50,000 live births. It occurs in all races. Prune Belly syndrome almost exclusively occurs in males (M:F, 20:1). The diagnosis can be made in utero by ultrasonography at 21 weeks of gestation or in the Neonate with characteristic clinical findings. The present case was a dead male fetus of 20 weeks of gestation sent to Anatomy department after Medical termination of pregnancy, due to congenital anomalies identified in routine ultrasound examination during antenatal checkup.

Prune-belly syndrome detected by ultrasound in the first trimester and the usefulness of vesicocentesis as a modality of treatment

Prune-belly syndrome may be related to lower urinary tract obstruction (LUTO). LUTO in the early gestational age exacerbates fetal renal function and may require intrauterine intervention. If early developed LUTO causes bladder distension and abdominal musculature deficiency, it will result in prune belly syndrome. Therefore, early detection of the disease and proper treatment before the renal impairment is important. However, there are few literatures concerning the treatment of prune belly syndrome in the first trimester. We report a case of prune belly syndrome diagnosed at 11+6 weeks of gestation and the value of vesicocentesis as a modality of treatment. Ultrasound showed dilated fetal bladder and vesicocentesis was successful in reducing the volume of the bladder. However, the pregnancy was terminated upon request.

Malformaciones de la uretra masculina: [revisión] / Malformations of the male urethra: [review]

La patología de la vía urinaria es frecuente en la población pediátrica. Las anomalías uretrales son comunes y constituyen una importante causa de morbilidad. Reconocer las patologías y utilizar de manera óptima los métodos diagnósticos disponibles son pasos claves para un manejo ideal de estos pacientes y para la prevención de futuras morbilidades, que en ocasiones son de alto costo e implican una reducción significativa en la calidad de vida.

Pathology of the urinary tract is common in the pediatric population. Urethral anomalies are frequent and an important cause of morbidity. Recognizing these diseases and usingimaging modalities in an optimal way, is important for an ideal management of the different pathologies and as a prevention strategy of future associated complications, that can turn into high cost diseases with an important impact in the quality of life.

Hallazgos estomatológicos en insuficiencia renal crónica debido a síndrome de Prune Belly: reporte de caso / Oral findings in renal chronic failure from Prune Belly syndrome: case report

El síndrome de Prune Belly es una alteración congénita que puede conducir a insuficiencia renal crónica y alteraciones metabólicas como hiperparatiroidismo secundario. El presente caso describe un individuo masculino que padece insuficiencia renal crónica debido al síndrome de Prune Belly y presenta hallazgos estomatológicos como hipoplasia de esmalte, signos radiográficos de osteopenia a nivel de los maxilares y ausencia de lesiones de caries. Los exámenes radiográficos demostraron retardo de edad biológica en el paciente. Se brindó tratamiento odontológico preventivo.

Prune Belly's syndrome is a congenital alteration that can lead to chronic renal failure and metabolic alterations as secondary hyperparathyroidism. The present case describes a male who suffers chronic renal failure due to Prune Belly's syndrome and presents oral findings like hipoplasia of enamel, radiographic signs of osteopenia in the upper maxilla and absence of carious lesions. Radiographic analyses showed a delay in the biological age in the patient. Preventive treatment was offered to the patient.

Síndrome de prune belly (ciruela pasa): reporte de un caso / Prune belly syndrome: a case report

El síndrome de prune belly es una rara anomalía congénita. Se trata de una triada compuesta por ausencia, deficiencia o hipoplasia de la musculatura de la pared abdominal, criptorquidia bilateral y malformación del tracto urinario (dilatación). Se presenta el caso de un bebé, de sexo masculino y con diagnóstico imagenológico in utero de megavejiga, valvas uretrales posteriores parciales y dilatación de los uréteres. Luego del nacimiento, las características fenotípicas fueron sugestivas del síndrome de prune belly (ciruela pasa). Los estudios complementarios confirmaron la ausencia de testículos en escroto, reflujo vésico ureteral bilateral y estenosis de la uretra proximal. La uro resonancia magnética con reconstrucción en 3D reportó que ambos riñones presentaban alteración en su morfología con quistes en su interior, hipoplasia renal derecha, uréteres dilatados y tortuosos, vejiga distendida con divertículo del uraco y deficiencia de los músculos rectos abdominales. Como complicaciones se presentaron una infección urinaria febril y una enfermedad renal crónica secundaria. Una vez los eventos infeccioso y obstructivo fueron resueltos la función renal se estabilizó y se procedió a realizar vesicostomía. El paciente fue dado de alta en condición estables, con manejo de la vesicostomía, profilaxis para infección urinaria y seguimiento periódico. La reconstrucción de su tracto urinario se realizará al segundo año de vida y de acuerdo con la evolución de su función renal.

Prune belly syndrome is a rare congenital anomaly. It is a triad consisting of absence, deficiency or hypoplasia of wall abdominal muscles, bilateral cryptorchidy, and urinary tract (dilatation) malformation. As an example, take the case of a male baby with in utero diagnostic of megacystis, partial posterior urethral valves, and dilated ureters. After birth, the phenotypic characteristics were suggestive of prune belly syndrome. Complementary studies confirmed the absence of testes in scrotum, bilateralvesicourereteral reflux and stricture of the proximal urethra. Magnetic resonance with 3D reconstruction reported that both kidneys had alterations in their morphology with interior cysts, right renal hypoplasia, long and tortuous ureters, distendedbladder with diverticulum of urachus and deficiency of abdominal muscles. There were complications like febrile urinary tract infection and secondary chronic kidney disease. Once the infectious and obstructive events were resolved the renal function was stabilized and the surgical approach was vesicostomy. The patient was discharged in stable condition, with vesicostomy management, prophylaxis for urinary tract infection and regular follow. The urinary tract reconstruction will be in the second year of life and according to evolution of his kidney function.