Cases reported "Hydronephrosis"

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1/78. hydronephrosis in pregnancy: simultaneous depiction of fetal and maternal hydronephrosis by magnetic resonance urography.

    Magnetic resonance urographic (MRU) techniques possess image quality and diagnostic capability that are improving with increasingly sophisticated imaging sequences and shorter scanning times. We describe the application of a fast breath-hold MR sequence (HASTE) in the assessment of ureteric obstruction in pregnancy. In the patient presented, HASTE MRU was successful in depicting ureteral anatomy and demonstrated dilation of both ureters below the level of the pelvic brim. This observation suggested distal ureteral obstruction rather than simple hydronephrosis of pregnancy. As a result, bilateral nephrostomies were performed and neonatal prematurity was avoided. Interestingly, in this patient, HASTE MR imaging also showed evidence of concurrent fetal hydronephrosis.
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2/78. Spontaneous resolution of an in utero perirenal urinoma associated with posterior urethral valves.

    Ultrasound imaging of a 26-week-gestation fetus demonstrated a large, nonemptying bladder. At 27 weeks, a distended, thick-walled bladder, left hydronephrosis, and a perirenal urinoma were present, without ascites. observation was undertaken, as the amniotic fluid volume was normal. At 29 weeks, the left perirenal fluid collection persisted but, at 30 weeks, was absent. After delivery at 36 weeks, no ultrasound evidence for perirenal urinoma or ascites was present. Isotope renal scan showed preserved renal function bilaterally. This case illustrates that in utero urinomas associated with posterior urethral valves can resolve spontaneously, with preservation of renal function.
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3/78. Megacystis microcolon intestinal hypoperistalsis syndrome: bladder distension and pyelectasis in the fetus without anatomic outflow obstruction.

    We report a case of a 46,XY male infant with a history of normal amniotic fluid levels who was delivered by elective cesarean section at 38.5 weeks' gestation because of progressive bladder distension, hydroureteronephrosis, and what was thought to be a dilated posterior urethra. The child died at 19 days of age of cardiovascular complications. The autopsy revealed megacystis, bilateral megaureters and pyelocaliectasis, congenital absence of ganglion cells in the bladder wall, renal dysplasia, and a microcolon. No dilation or anatomic obstruction of the posterior urethra was found. These findings strongly suggest the diagnosis of megacystis microcolon intestinal hypoperistalsis syndrome. We discuss the ultrasound findings of in utero bladder distension with hydronephrosis and one of its rare etiologies.
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4/78. Cloacal outlet obstruction with an ectopic ureter.

    Cloacal malformation occurs in approximately 1 in 50,000 live female births. Prenatal ultrasound may lead to the diagnosis in selected cases. We report an unusual case of prenatally detected single-system hydronephrosis with a nonvisible bladder and worsening oligohydramnios. Labor was induced at 35 weeks' estimated gestational age. On physical examination, a single perineal opening was noted consistent with cloaca. endoscopy revealed an obstructed ectopic ureter at the level of the sphincter, an undeveloped bladder and vagina, and a fistula to the rectum. A low loop cutaneous ureterostomy and right upper quadrant loop colostomy were performed. The absence of a typical fluid-filled pelvic structure may confound the prenatal diagnosis of cloaca.
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5/78. Genitopatellar syndrome: a new condition comprising absent patellae, scrotal hypoplasia, renal anomalies, facial dysmorphism, and mental retardation.

    We report on the association of absent patellae, genital and renal anomalies, dysmorphic features, and mental retardation in seven children (six boys and one girl) belonging to five unrelated families. Flexion deformities of the knees and hips with club feet and absent patellae were consistently observed and scrotal hypoplasia and cryptorchidism were present in all boys (6/6). Dysmorphic features included a coarse face, a large nose with a high nasal bridge, and microcephaly. Other features included renal anomalies (multicystic kidneys or hydronephrosis, 7/7), agenesis of the corpus callosum (4/7), swallowing difficulties, micrognathia (4/7), and pulmonary hypoplasia (3/7). Bilateral hypoplasia of the ischia and brachydactyly were also consistently observed (5/5). In two out of seven cases, prenatal ultrasound detection of microcephaly and renal anomalies led to termination of the pregnancy at 27 weeks. Three children died during the first years of life and the remaining two who survived exhibit severe developmental delay. High resolution cytogenetic studies performed on lymphocytes or fibroblasts or both were normal in all cases. recurrence in two families suggests an autosomal recessive mode of inheritance. We propose that this unusual association, similar to that observed in a 4 year old boy by Goldblatt et al, represents a new syndrome distinct from previously reported hypoplastic patella syndromes.
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6/78. Spontaneous rupture of hydronephrotic kidney during pregnancy: value of serial sonography.

    hydronephrosis during pregnancy is common but rarely results in renal rupture. We report an unusual case of spontaneous rupture of a hydronephrotic kidney during pregnancy. Although we could not predict the renal rupture on the basis of sonographic findings, serial sonography was useful in the early detection and management of the rupture.
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7/78. A case of walker-warburg syndrome.

    walker-warburg syndrome (WWS) is an autosomal recessive disorder characterized by type II lissencephaly, cerebellar and retinal anomalies, and congenital muscular dystrophy. We report a female diagnosed with WWS based on clinical criteria. This patient was found to have fetal hydrocephalus on ultrasonography at 29 weeks of gestation, and exhibited severe hypotonia, ocular malformations, and hydrocephalus at birth. MRI revealed type II lissencephaly, hydrocephalus, and other severe brain malformations. Genetic analysis was performed to distinguish WWS from severe Fukuyama-type congenital muscular dystrophy (FCMD), which has numerous findings in common. This revealed no expression of the founder haplotype or single-stranded conformation polymorphism (SSCP) abnormalities. Since the life expectancy of patients with FCMD is longer, differential diagnosis should be performed precisely.
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keywords = gestation
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8/78. Sacrococcygeal teratoma with hydrops fetalis and bilateral hydronephrosis.

    A 29-year-old Japanese primipara with fetal sacrococcygeal teratoma and hydronephrosis was referred to our department at 22 weeks of gestation. The preload index of the inferior vena cava (PLI) was 0.75 and fetal cardiac failure was suspected at 28 weeks of gestation. The sodium and chloride concentrations and osmolarity of the fetal urine were 96 mEq/L, 81 mEq/L and 204 mOsm/L, respectively, and we predicted a good renal function. Then, a vesico-amniotic shunting operation was performed at 28 weeks of gestation to keep the renal function and PLI decreased 0.41 after this operation. At 30 weeks of gestation, the fetus was delivered by a cesarean section owing to preterm PROM and a huge growing sacrococcygeal teratoma. The baby was female, weighing 2,020 g, and the 1 minute apgar score was 1. She died almost 7 hours after birth due to respiratory insufficiency. An autopsy confirmed bilateral hydronephrosis, urethral stenosis, sacrococcygeal immature teratoma, and pulmonary hypoplasia. The size of this tumor revealed 80 x 70 x 45 mm in intrapelvic cavity and 130 x 90 x 50 mm out of body, and this tumor was classified as Type II according to the American Academy of pediatrics Surgery Section classification.
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9/78. Reversible acute renal failure in association with bilateral ureteral obstruction and hydronephrosis in pregnancy.

    This is a case report of a 16-year-old girl with renal failure in pregnancy in association with bilateral ureteral obstruction and hydronephrosis. The presenting symptom was a common nonspecific type of abdominal pain. The diagnosis was made on incidental laboratory work to rule out preeclampsia. Renal function reverted back to normal after induced vaginal delivery.
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10/78. Ectopic hydronephrotic kidney masquerading as an ovarian cyst during pregnancy.

    An ectopic iliopelvic kidney with hydronephrosis causing fetal malpresentation is a rare occurrence. We describe this case for its unusual presentation which was mistaken for an ovarian cyst. The difficulty in diagnosis and the need for a high index of suspicion is highlighted.
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