Long term follow-up in a patient with prune-belly syndrome – a care compliant case report

Abstract

Rationale:Malformative uropathies represent a major cause of Chronic Kidney Disease (CKD) in children. Genitourinary system is the most frequent and sever affected in Prune-Belly syndrome cases. That is why the findings of early diagnosis and vigilant monitoring for these situations remain a major challenge for the medical team.Patient concerns:We present the clinical course of a 10 years old child with diagnosis of Prune-Belly syndrome. A urinary tract abnormality was suspected starting 25 weeks of gestation, when a routine ultrasound showed oligohydramnios, increased size urinary bladder, bilateral hydronephrosis and megaureters, thin abdominal wall.Diagnosis:Prenatal suspicion of Prune-Belly syndrome plays a deciding role in renal disease progression. A detailed clinical exam at birth established the diagnosis of Prune-Belly syndrome. Renal ultrasound confirmed bilateral grade III hydronephrosis and megaureters, with empty bladder, suggesting an obstruction at this level. A persistent urachus was confirmed by catheterization. Later it was used for imaging study that showed bilateral high grade reflux.Interventions:The main goal of any treatment is to preserve kidney function. Treatment options depend on the clinical picture. The pregnancy was closely monitorized, but fetal distress appeared so early labor was induced at 32 weeks. At beginning a temporary catheter was placed into the urachus which expressed urine. The urachus drain was left in place until the age of 6 weeks, when a bilateral ureterostomy was performed. Skeletal and genital malformations were present too; the child has undergone several surgeries to solve these abnormalities.Outcomes:At the age of 10 years, he is a well-adapted child. He has had fewer than 3 urinary tract infections per year. Long term follow-up showed a relatively slow decline in the estimated Glomerular Filtration Rate in our child (62 ml/1.73m2/min).Lessons:This case suggests that induced early labor could prove beneficial for early upper urinary tract decompression through earlier access to surgery. This is an option especially in situations or region where vesicoureteric or vesicoamniotic shunt placement is not available.

Syndrome De Prune-Belly : A Propos d'UN Cas Enregistre a Lubao, République Démocratique Du Congo, Et Revue De La Litterature

“…La prise en charge des cas de SPB n'est pas aisé 3,4 , surtout en cas de multiples malformations congénitales associées. La mortalité est dépendante des atteintes pulmonaires et rénales 4,26 , mais il existe des cas ayant survécus au moins dix ans 32 .…”

Síndrome de Prune Belly em paciente escolar

“…Outros pacientes possuidores da síndrome e também com maior idade já foram descritos na literatura. Cornel et al 10 relataram o caso de uma criança de 10 anos, com SPB, que foi internada na nefrologia pediátrica pela primeira vez com 3 dias de vida. A suspeita da doença teve início às 25 semanas de gestação em que foram visualizadas alterações características por meio da USG.…”

“…Além das malformações clássicas que compõem a tríade, outras patologias podem se desenvolver em associação. Dentre elas estão as alterações pulmonares, como hipoplasia pulmonar, pneumonias e atelectasia; alterações gastrointestinais, a exemplo da constipação crônica, ânus imperfurado e fístula retouretral; alterações musculoesqueléticas, como subluxação do quadril, escoliose e pé torto congênito; o atraso no desenvolvimento neuropsicomotor e as alterações cardiovasculares em menor escala 9,10 .…”

“…O diagnóstico da síndrome pode ser efetuado ainda durante o pré-natal da gestante, por meio da ultrassonografia (USG) no segundo trimestre. Em alguns casos, até a 12ª semana da gestação é possível identificar a doença, dependendo do grau de obstrução do trato urinário, da presença de oligodrâmnio e da experiência do examinador 10,11,12,13 .…”

“…O prognóstico das pessoas com a síndrome é reservado, sendo pequeno o número de indivíduos que sobrevivem além da infância. Apesar da evolução no cuidado neonatal, permanecem elevadas as taxas de mortalidade perinatal desses pacientes, variando entre 10% e 25% 4,10,14,15 .…”