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Rare association of prune belly syndrome with pouch colon
M Ragavan,U Haripriya,PV Pradeep,et al
Pediatric Health, Medicine and Therapeutics , 2011,
Abstract: M Ragavan1, U Haripriya1, PV Pradeep1, J Sarvavinothini21Department of Endocrine Surgery, 2Department of Anaesthesia, Narayana Medical College and Superspeciality Hospital, Nellore, Andhra Pradesh, IndiaAbstract: Prune belly syndrome is a triad characterized by abdominal wall musculature deficiency, cryptorchidism and urinary tract abnormalities, and is often associated with other anomalies. Although associated anorectal anomalies have been reported with this syndrome, only two cases of pouch colon, a rare type of anorectal malformation, have been reported. We report a case of prune belly syndrome with pouch colon presenting with retention of urine.Keywords: prune belly, triad syndrome, pouch colon, anorectal malformation
Rare association of prune belly syndrome with pouch colon
M Ragavan, U Haripriya, PV Pradeep, et al
Pediatric Health, Medicine and Therapeutics , 2011, DOI: http://dx.doi.org/10.2147/PHMT.S16269
Abstract: re association of prune belly syndrome with pouch colon Case report (2543) Total Article Views Authors: M Ragavan, U Haripriya, PV Pradeep, et al Published Date January 2011 Volume 2011:2 Pages 9 - 12 DOI: http://dx.doi.org/10.2147/PHMT.S16269 M Ragavan1, U Haripriya1, PV Pradeep1, J Sarvavinothini2 1Department of Endocrine Surgery, 2Department of Anaesthesia, Narayana Medical College and Superspeciality Hospital, Nellore, Andhra Pradesh, India Abstract: Prune belly syndrome is a triad characterized by abdominal wall musculature deficiency, cryptorchidism and urinary tract abnormalities, and is often associated with other anomalies. Although associated anorectal anomalies have been reported with this syndrome, only two cases of pouch colon, a rare type of anorectal malformation, have been reported. We report a case of prune belly syndrome with pouch colon presenting with retention of urine.
Prune Belly Syndrome
Koyye Ravindranath Tagore,Asok Kumar S. Ramineni,A. R. Vijaya Lakshmi,Bhavani N.
Case Reports in Pediatrics , 2011, DOI: 10.1155/2011/121736
Abstract: Prune belly syndrome is a rare congenital disorder of the urinary system, characterized by a triad of abnormalities. The aetiology is not known. Many infants are either stillborn or die within the first few weeks of life from severe lung or kidney problems, or a combination of congenital anomalies.
Síndrome de Prune Belly: Presentación de un caso y revisión de la literatura
Guerrero,Albert Franz; Cuadros,Carlos Augusto; Archila,Diana Carolina; Beltrán,Sandra Milena; Cuadros,Gustavo Adolfo;
Revista de la Universidad Industrial de Santander. Salud , 2010,
Abstract: introduction: prune-belly syndrome, also known as eagle-barrett syndrome is characterized by a triad of anomalies that include varying degrees of abdominal musculature hypoplasia, urinary tract anomalies, and bilateral cryptorchidism. objective: we describe the case of a male patient with prune belly syndrome and we review the literature on this rare disease. conclusions: the characteristic wrinkled, prune-like abdomen, gives the name to the syndrome. can also be associated with cardiovascular, respiratory, orthopedic and gastrointestinal anomalies. salud uis 2010; 42: 78-85.
Prune belly syndrome (sequenze): a case report  [cached]
A. Vani,S. Saritha,K. Sangeetha,G. Supriya
International Journal of Research in Medical Sciences , 2013, DOI: 10.5455/2320-6012.ijrms20130523
Abstract: 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. [Int J Res Med Sci 2013; 1(2.000): 148-152]
Prune-Belly Syndrome: A Case Report from Rwanda
E Ngendahayo, P Kyamanywa, L Mutesa, J Gashegu
East and Central African Journal of Surgery , 2012,
Abstract: Background: Prune-Belly syndrome, Eagle-Barret syndrome and triad syndrome, all refer to congenital anomalies involving abdominal musculature, urinary tract and testicles. The syndrome consists of a triad of abdominal muscle aplasia, massive ureteral and bladder dilatation and cryptorchidism. Kidneys are often affected by secondary hydronephrosis or by polycystic dysplasia. The full manifestation of the syndrome occurs almost exclusively in boys. Available reports on the epidemiology and outcome show a high perinatal mortality due to related prematurity and associated pulmonary complications. The management of a prune-belly patient has been controversial. However nowadays, the tendency is to assist primarily prune – belly neonates in respiratory failure, and to limit radical urologic interventions. Case Report: We report hereby a full term prune-belly neonate who succumbed from this condition before being adequately investigated. Our purpose is to call clinicians’ attention to early recognition, investigation and management of the syndrome. Even though infants with a full-blown syndrome have a poor prognosis for long term survival, all patients need careful evaluation and individualized management according to the spectrum of the syndrome. In this paper, the morphogenesis and the developmental biology of the abdominal wall will be also recalled, and literature reviewed.
The Phenotypic and Pathological Features of Prune-Belly Syndrome  [cached]
Davut ?AH?N,Handan ?ET?NER,Nermin KO?
Türk Patoloji Dergisi , 2010,
Abstract: Objective: Prune-belly syndrome is a rare congenital disorder characterized by musculature deficiency in the abdominal wall, lower urinary tract obstruction, other urinary tract anomalies, and bilateral cryptorchidism. The syndrome is commonly associated with pulmonary, skeletal, cardiac, and gastrointestinal defects. Over 95% of patients are male. Urinary tract disease is the major prognostic factor with the complications of pulmonary hypoplasia and end stage renal disease. The aim of this study was to determine phenotypic and pathologicalfeatures of fetuses with this syndrome.Material and Method: Six fetuses with prune-belly syndrome were evaluated by postmortem pathological investigation. Characteristic features of the fetuses with this syndrome as well as additional anomalies were evaluated.Results: Five fetuses were male while one was female. Gestational age ranged from 15 to 22 weeks. A urethral pathology that prevented urinary outflow from the bladder was present in all cases. Marked bladder distension with atrophy of the bladder smooth muscle and abdominal distension with muscular atrophy were also seen in all. Crypto-orchidism, Potter face, pes equinovarus, pulmonary hypoplasia and obstructive renal dysplasia were among the additional noteworthy anomalies.Conclusion: The pathogenesis of prune-belly syndrome is controversial. More studies are required on the inheritance, etiology and pathogenesis of the prune belly syndrome. Factors affecting the bilaminar and trilaminar germ layer during early 2-3. embryonic week may be considered to explain the pathogenesis of the anomalies seen with this syndrome.
Elective appendicovesicostomy in association with monfort abdominoplasty in the treatment of prune belly syndrome
Liguori, Riberto;Barroso Jr, Ubirajara;Matos, Joao T.;Ottoni, Sergio L.;Garrone, Gilmar;Demarchi, Guilherme T.;Ortiz, Valdemar;Macedo Jr, Antonio;
International braz j urol , 2006, DOI: 10.1590/S1677-55382006000600010
Abstract: objective: to evaluate the role of elective appendicovesicostomy in association with monfort abdominoplasty to avoid urinary tract infection (uti) and renal damage in the post-operative follow-up of patients with prune belly syndrome. materials and methods: we followed 4 patients operated in our institution (unifesp) (monfort, orchidopexy and mitrofanoff) and compared them to 2 patients treated similarly, but without an appendicovesicostomy, in a second institution (ufba). we evaluated postoperative clinical complications, uti and preservation of renal parenchyma. patients were followed as outpatients with urinalysis, ultrasonography (us) and occasionally with renal scintigraphy. results: mean follow-up was 23.5 months. immediate post-operative course was uneventful. we observed that only one patient with the mitrofanoff channel persisted with uti, while the 2 patients used as controls persisted with recurrent pyelonephritis (> 2 uti year). conclusion: our data suggest that no morbidity was added by the appendicovesicostomy to immediate postoperative surgical recovery and that this procedure may have a beneficial effect in reducing postoperative uti events and their consequences by reducing the postvoid residuals in the early abdominoplasty follow-up. however, we recognize that the series is small and only a longer follow-up with a larger number of patients will allow us to confirm our suppositions. we could not make any statistically significant assumptions regarding differences in renal preservation due to the same limitations.
Prune belly syndrome with pouch colon and absent dermatome  [cached]
Baba Aejaz,Hussain Syed,Shera Altaf,Patnaik Rekha
African Journal of Paediatric Surgery , 2010,
Abstract: Prune belly syndrome (PBS) is a rare congenital constellation of defects in pediatric surgical practice. Although anorectal anomalies have been reported in association with PBS, only few case of pouch colon with PBS has been reported. [1] In addition, our patient had deficient abdominal wall with absent dermatome in left upper quadrant, which has never been reported in the English literature. This association with abdominal wall deficiency and absent dermatome not only strengthens the theory of mesodermal arrest in the etiology of PBS but also points towards a defect in the ectodermal development.
Síndrome de Prune Belly: Presentación de un caso y revisión de la literatura Prune Belly Syndrome: Case report and review  [cached]
Albert Franz Guerrero,Carlos Augusto Cuadros,Diana Carolina Archila,Sandra Milena Beltrán
Revista de la Universidad Industrial de Santander. Salud , 2010,
Abstract: Introducción: El síndrome de Prune Belly (SPB), también conocido como el síndrome de Eagle Barrett, se caracteriza por una triada de anomalías que incluye grados variables de hipoplasia de la musculatura abdominal, anomalías del tracto urinario y criptorquidia bilateral. Objetivo: Se describe el caso de un paciente masculino con Síndrome de Prune Belly y se realiza una revisión de la literatura sobre esta rara enfermedad. Conclusión: La característica arrugada del abdomen similar a una ciruela pasa, le da el nombre al síndrome. Además, puede estar asociado a alteraciones cardiovasculares, respiratorias, ortopédicas y gastrointestinales. Salud UIS 2010; 42: 78-85 Introduction: Prune-belly syndrome, also known as Eagle-Barrett syndrome is characterized by a triad of anomalies that include varying degrees of abdominal musculature hypoplasia, urinary tract anomalies, and bilateral cryptorchidism. Objective: We describe the case of a male patient with Prune Belly Syndrome and we review the literature on this rare disease. Conclusions: The characteristic wrinkled, prune-like abdomen, gives the name to the syndrome. Can also be associated with cardiovascular, respiratory, orthopedic and gastrointestinal anomalies. Salud UIS 2010; 42: 78-85.
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