&lt;p&gt;Prune belly syndrome: current perspectives&lt;/p&gt;

Arlen, Angela M.; Nawaf, Cayce; Kirsch, Andrew J.

doi:10.2147/phmt.s188014

Cited by 40 publications

(62 citation statements)

References 68 publications

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“…Patient past medical history, family history and his mother's pregnancy history did not contribute to diagnosis BACKGROUND P rune Belly Syndrome (PBS) is a rare congenital disorder characterized by a triad of deficient abdominal wall muscles, cryptorchidism and urinary tract anomalies (including hydroureteronephrosis). The syndrome affects 3.8 per 100,000 live male births [1][2][3][4] . Over 95% of patients are boys 5 .…”

Section: Ethical Considerationsmentioning

confidence: 99%

“…Some studies have suggested that the abdominal muscle deficiency is due to foetal abdominal distension of different causes including urethral obstruction with enlarged bladder and hydronephrosis. Also mesenchymal developmental defects have been suggested as the underlying defect 1 . The cause of PBS is unknown 6 .…”

Section: Ethical Considerationsmentioning

confidence: 99%

“…and regular prenatal follow up 1 . We report two cases with clinical manifestations associated with PBS, including deficient abdominal wall muscles, hydronephrosis and cryptorchidism.…”

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confidence: 99%

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Two cases of Prune Belly Syndrome from Kagera Region Tanzania

Kessy¹,

Philemon

Hamel

2020

EAHRJ

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Prune Belly Syndrome is a rare congenital disorder with unknown aetiology, consisting of a triad of abdominal muscle wall weakness, undescended testes, and urinary tract abnormalities. We are unaware of any preceding report of Prune Belly Syndrome in Tanzania, and here we describe two cases reported in Kagera region. The first case is a 2 month old boy with the triad of Prune Belly Syndrome along with pectus carinatum who died due to septicaemia. This case posed a diagnostic challenge at birth and during the natal period. Paucity of comprehensive knowledge of congenital malformations at the peripheral health facilities may have also contributed to the diagnostic challenge in the first place. The second case is a neonate who was referred to regional referral hospital where he was diagnosed with Prune Belly Syndrome at the age of four weeks. Because of limited capacity to manage congenital malformations at the regional referral hospital, he was referred to an urologist at the zonal referral hospital. However, inadequacies in supporting systems to the parents compounded care of the neonate with Prune Belly Syndrome. High index of Prune Belly Syndrome suspicion is needed in a resource limited setting in order to timely make diagnosis. There is also a need to strengthen institutional and individual's capacity for prenatal screening to detect congenital anomalies at an early stage of foetus development. Multidisciplinary management approach is necessary in order to improve the quality of life for patients with Prune Belly Syndrome. Psychosocial and medical support systems should be put in place in order to enhance preparedness for patient care in resource limited settings including equipping the referral hospital with different specialists and ensuring availability of basic investigations for patients.

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Section: Ethical Considerationsmentioning

confidence: 99%

Section: Ethical Considerationsmentioning

confidence: 99%

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Two cases of Prune Belly Syndrome from Kagera Region Tanzania

Kessy¹,

Philemon

Hamel

2020

EAHRJ

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“…Існує гіпотеза, що розширена сечо видільна система або облітерація пахових каналів ускладнюють нормальне опускання яєчок або вони можуть бути прикріплені до сечоводу. Однак крипторхізм не спостеріга ється у дітей із розширеним сечовим міхуром іншої етіології [2,15,25].…”

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“…Клініка Читайте нас на сайті: http://med expert.com.ua Гаррісона) може бути у багатьох дітей із син дромом Пруне-Беллі. Також може спостері гатися звуження грудної клітки у поперечно му напрямку (килевидна деформація грудної клітки) [2,25].…”

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Prune Belly syndrome: a case report

Tsymbalista¹,

Ziniak²,

Kurtash³

et al. 2020

MPU

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Objective: to acquaint doctors of various specialties with clinical course, early diagnostics, and treatment strategy for a rare congenital anomaly — Prune Belly syndrome in children. Materials and methods. Inpatient medical records of municipal non-profit enterprise «Ivano-Frankivsk Regional Children's Clinical Hospital» of Ivano-Frankivsk Regional Council. Results. The paper presents the clinical course of a rare congenital anomaly — Prune Belly syndrome in two children. The disease occurs chiefly among boys manifesting itself as multiple congenital malformations including deficiency or aplasia of abdominal muscles, congenital anomalies of the kidneys and urinary tract (hydronephrosis, megaureter, hydroureter, megacystis, vesicoureteral reflux), bilateral cryptorchidism. In addition, Prune Belly syndrome can coexist with urethral pathology, as well as skeletal, cardiovascular, respiratory, gastrointestinal malformations. Conclusion. Modern views on the development, clinical course, and diagnosis of a rare congenital disease with multiple organ malformations — Prune Belly syndrome have been presented for doctors of various specialties. Prenatal diagnosis, detection of potential congenital malformations after birth allows selecting the correct algorithm for monitoring vital organ functions, syndromic treatment and making timely decision on internal organ transplantation. The study was carried out in accordance with the ethical principles of the Declaration of Helsinki. The study protocol was approved by the Local Ethics Committee of municipal non+profit enterprise «Ivano-Frankivsk Regional Children's Clinical Hospital» of Ivano-Frankivsk Regional Council. The informed consent to participate in the research study was obtained from all the children's parents. Conflict of Interest. The authors declare they have no conflict of interest. Key words: children, Prune Belly syndrome, diagnosis, therapy.

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Prenatal hydronephrosis: Bridging pre‐ and postnatal management

et al. 2022

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Urinary tract dilation (UTD), including hydronephrosis (HN) is the most common prenatally detected anomaly and affects up to 1%–2% of pregnancies. Postnatally, it resolves without surgical intervention in approximately 75%–80% of children, however this may take several years to occur, necessitating repeated clinic visits and additional invasive testing for many. For the remainder, a surgical intervention will be offered to relieve obstruction or to correct vesicoureteral reflux. During the monitoring period, many of these infants will be offered continuous antibiotic prophylaxis for the prevention of urinary tract infections, however this remains a controversial topic among pediatric urologists and nephrologists. Herein, we present an up‐to‐date review of the early management of prenatally detected UTD including timing of postnatal imaging, the use of antibiotics, when circumcision may be beneficial and long term outcomes of the most common HN etiologies. We also propose a decision making tool to help guide the care of infants with UTD.

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<p>Prune belly syndrome: current perspectives</p>

Cited by 40 publications

References 68 publications

Two cases of Prune Belly Syndrome from Kagera Region Tanzania

Two cases of Prune Belly Syndrome from Kagera Region Tanzania

Prune Belly syndrome: a case report

Prenatal hydronephrosis: Bridging pre‐ and postnatal management

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