Prune Belly syndrome: a case report
Keywords:
children, Prune Belly syndrome, diagnosis, therapyAbstract
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.
References
Alhawsawi AM, Aljiffry M, Walsh MJ, Peltekian K, Molinari M. (2009, Jan-Feb). Hepatic artery aneurysm associated with prune belly syndrome: a case report and review of the literature. J Surg Educ. 66(1): 43-47. https://doi.org/10.1016/j.jsurg.2008.10.004; PMid:19215897
Arlen AM, Nawaf C, Kirsch AJ. Pr Arlen AM, Nawaf C, Kirsch AJ. (2019). Prune belly syndrome: current perspectives. Pediatric Health Med Ther. 10: 75-81. https://doi.org/10.2147/PHMT.S188014; PMid:31496864 PMCid:PMC6689549
Bellah RD, States LJ, Duckett JW. (1996, Dec). Pseudoprune-Belly syndrome: imaging findings and clinical outcome. AJR Am J Roentgenol. 167(6): 1389-1393. https://doi.org/10.2214/ajr.167.6.8956564; PMid:8956564
Biard JM, Johnson MP, Carr MC, Wilson RD, Hedrick HL, Pavlock C et al. (2005, Sep). Long-term outcomes in children treated by prenatal vesicoamniotic shunting for lower urinary tract obstruction. Obstet Gynecol. 106(3): 503-508. https://doi.org/10.1097/01.AOG.0000171117.38929.eb; PMid:16135579
Boghossian NS, Sicko RJ, Giannakou A, Dimopoulos A, Caggana M, Tsai MY et al. (2018, Mar). Rare copy number variants identified in prune belly syndrome. Eur J Med Genet. 61(3): 145-151. https://doi.org/10.1016/j.ejmg.2017.11.008; PMid:29174092 PMCid:PMC5803418
Ehrlich RM, Lesavoy MA. (1993, Mar). Umbilicus preservation with total abdominal wall reconstruction in prune2belly syndrome. Urology. 41(3): 231-232. https://doi.org/10.1016/0090-4295(93)90562-O
Franco I. (2005, Jul). Laparoscopic assisted modification of the Firlit abdominal wall plication. J Urol. 174(1): 280-283. https://doi.org/10.1097/01.ju.0000161214.65458.c2; PMid:15947667
Furness PD 3rd, Cheng EY, Franco I, Firlit CF. (1998, Sep). The prune-belly syndrome: a new and simplified technique of abdominal wall reconstruction. J Urol. 160(3); 2: 1195-1197. discussion 1216. https://doi.org/10.1016/S0022-5347(01)62738-1
Lesavoy MA, Chang EI, Suliman A, Taylor J, Kim SE, Ehrlich RM. (2012, Jan). Long-term follow-up of total abdominal wall reconstruction for prune belly syndrome. Plast Reconstr Surg. 129(1): 104e-109e. https://doi.org/10.1097/PRS.0b013e3182362091; PMid:22186524
Lindgren BW, Franco I, Blick S, Levitt SB, Brock WA, Palmer LS et al. (1999, Sep). Laparoscopic Fowler-Stephens orchiopexy for the high abdominal testis. J Urol. 162(3); 2: 990-993. discussion 994. https://doi.org/10.1016/S0022-5347(01)68042-X
Lopes RI, Tavares A, Srougi M, Denes FT. (2015, Oct). 27 years of experience with the comprehensive surgical treatment of prune belly syndrome. J Pediatr Urol. 11 (5): 276.e12277. https://doi.org/10.1016/j.jpurol.2015.05.018; PMid:26143487
Massad CA, Cohen MB, Kogan BA, Beckstead JH. (1991, Dec). Morphology and histochemistry of infant testes in the prune belly syndrome. J Urol. 146(6): 1598-1600. https://doi.org/10.1016/S0022-5347(17)38178-8
Monfort G, Guys JM, Bocciardi A, Coquet M, Chevallier D. (1991, Aug). A novel technique for reconstruction of the abdominal wall in the prune belly syndrome. J Urol. 146(2); 2: 639-640. https://doi.org/10.1016/S0022-5347(17)37880-1
Noh PH, Cooper CS, Winkler AC, Zderic SA, Snyder HM 3rd, Canning DA. (1999, Oct). Prognostic factors for long-term renal function in boys with the prune-belly syndrome. J Urol. 162(4): 1399-1401. https://doi.org/10.1016/S0022-5347(05)68320-6
Nunn IN, Stephens FD. (1961, Dec). The triad syndrome: a composite anomaly of the abdominal wall, urinary system and testes. J Urol. 86: 782-794. https://doi.org/10.1016/S0022-5347(17)65261-3
Passerini2Glazel G, Araguna F, Chiozza L, Artibani W, Rabinowitz R, Firlit CF. (1988, Nov). The P.A.D.U.A. (progressive augmentation by dilating the urethra anterior) procedure for the treatment of severe urethral hypoplasia. J Urol. 140; 5; Pt 2: 1247-1249. https://doi.org/10.1016/S0022-5347(17)42015-5
Philip J, Mullassery D, Craigie RJ, Manikandan R, Kenny SE. (2011, Jul) Laparoscopic orchidopexy in boys with prune belly syndrome - outcome and technical considerations. J Endourol. 25(7): 1115-1117. https://doi.org/10.1089/end.2010.0257; PMid:21671759
Prune Belly Syndrome. (2017, January 10). National Organization for Rare Disorders. Available at https://rarediseases.org/rare-diseases/prune-belly-syndrome/.
Saxena AK, Brinkmann OA. (2007, Aug). Unique features of prune belly syndrome in laparoscopic surgery. J Am Coll Surg. 205(2): 217-221. https://doi.org/10.1016/j.jamcollsurg.2007.03.008; PMid:17660067
Seidel NE, Arlen AM, Smith EA, Kirsch AJ. (2015, Jan). Clinical manifestations and management of prune-belly syndrome in a large contemporary pediatric population. Urology. 85 (1): 211-215. https://doi.org/10.1016/j.urology.2014.09.029; PMid:25444629
Smith CA, Smith EA, Parrott TS, Broecker BH, Woodard JR. (1998, May). Voiding function in patients with the prune-belly syndrome after Monfort abdominoplasty. J Urol. 159(5):1675-1679. https://doi.org/10.1097/00005392-199805000-00089; PMid:9554391
Smith EA, Srinivasan A, Scherz HC, Tracey AJ, Broecker B, Kirsch AJ. (2017, Oct.). Abdominoplasty in prune belly syndrome: Modifications in Monfort technique to address variable patterns of abdominal wall weakness. J Pediatr Urol. 13 (5): 502.e12502.e6. https://doi.org/10.1016/j.jpurol.2017.02.020; PMid:28373000
Snyder HM, Harrison NW, Whitfield HN, Williams INNES. (1976). Urodynamics in the prune belly syndrome. Br J Urol. 48(7): 663-670. https://doi.org/10.1111/j.1464-410X.1976.tb06716.x; PMid:138465
Stephens FD. (1983). Four valves in prune belly syndrome. Congenital Malformations of the Urinary Tract. Philadelphia, Pa: Praeger Press.
Waldbaum RS, Marshall VF. (1970, May). The prune belly syndrome: a diagnostic therapeutic plan. J Urol. 103(5): 668-674. https://doi.org/10.1016/S0022-5347(17)62025-1
Weiser A, Schoor RA, Franco I. (2003, Nov). Annual Meeting Section of Urology of American Academy of Pediatrics. Health Related Quality of Life, Social and Sexual Function In Patients with Prune Belly Syndrome.
Woodhouse CR, Kellett MJ, Williams DI. (1979, Dec). Minimal surgical interference in the prune belly syndrome. Br J Urol. 51(6): 475-480. https://doi.org/10.1111/j.1464-410X.1979.tb03582.x; PMid:43757
Woodard JR, Parrott TS. (1978, Aug). Orchiopexy in the prune belly syndrome. Br J Urol. 50(5): 348-351. https://doi.org/10.1111/j.1464-410X.1978.tb03646.x; PMid:37974
Woodard JR, Parrott TS. (1978, Jun). Reconstruction of the urinary tract in prune belly uropathy. J Urol. 119(6): 824-828. https://doi.org/10.1016/S0022-5347(17)57644-2
Downloads
Published
Issue
Section
License
The policy of the Journal “MODERN PEDIATRICS. UKRAINE” is compatible with the vast majority of funders' of open access and self-archiving policies. The journal provides immediate open access route being convinced that everyone – not only scientists - can benefit from research results, and publishes articles exclusively under open access distribution, with a Creative Commons Attribution-Noncommercial 4.0 international license (СС BY-NC).
Authors transfer the copyright to the Journal “MODERN PEDIATRICS. UKRAINE” when the manuscript is accepted for publication. Authors declare that this manuscript has not been published nor is under simultaneous consideration for publication elsewhere. After publication, the articles become freely available on-line to the public.
Readers have the right to use, distribute, and reproduce articles in any medium, provided the articles and the journal are properly cited.
The use of published materials for commercial purposes is strongly prohibited.
