Cases reported "Urethral Obstruction"

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11/163. epidermolysis bullosa, pyloric atresia, and obstructive uropathy: a report of two case reports with molecular correlation and clinical management.

    The epidermolysis bullosa-pyloric atresia-obstructive uropathy (EB-PA-OU) association is a rare, but well-described multisystem disease. While the prognosis at this time is still poor, an increasing number of patients are surviving to adolescence with aggressive care. It is important to understand this syndrome in order to anticipate medical complications and offer preventive strategies where possible. Prompt and expectant management of obstructive uropathy is crucial in these patients. Evidence of ureterovesicular obstruction may require bowel diversion, as excision of the obstructed ureterovesicular junction with reimplantation is often associated with a high risk of reobstruction. Many newborns succumb to sepsis or dehydration and electrolyte imbalance. Those infants who survive need close monitoring for the development of obstructive uropathy, failure to thrive, protein-losing enteropathy, respiratory compromise, and increased susceptibility to invasive infections. Once a clinical diagnosis is made, mutational analysis can confirm it and facilitate genetic counseling, as recurrence risks are 25% for this autosomal recessive condition. Mutational analysis enables direct genetic testing and accurate prenatal diagnosis. As more patients are studied, genotype/phenotype correlations may be possible. ( info)

12/163. Posterior urethral valves with congenital megalo-urethra.

    A 3-year-old male child was admitted with complaints of dysuria and dribbling of urine since birth. There was a small plum sized swelling, on the ventral surface of penis, which increased in size during micturition and on compression of the swelling urine dribbled out. Voiding cysto-urethrogram showed posterior urethral valves with scaphoid megalo-urethra. Vesicostomy with fulguration of the valves and one year later reduction urethroplasty were performed. ( info)

13/163. urinary retention resulting from incarceration of a retroverted, gravid uterus.

    urinary retention resulting from urethral obstruction by a retroverted, gravid uterus is an uncommon disorder that is reported only once in the emergency medicine literature. Yet these patients may present in extreme distress and precipitate considerable confusion regarding the cause of and solution to this problem. No study evaluating outcome, risk of complications, or therapy exists. We present two cases that clarify diagnostic and therapeutic controversies and provide a better understanding of what is known about the pathophysiology and treatment alternatives. ( info)

14/163. Genetic homogeneity of the urofacial (Ochoa) syndrome confirmed in a new French family.

    The urofacial syndrome (UFS) or Ochoa syndrome has been reported as a rare autosomal recessive disorder comprising a uropathy and facial abnormalities. The gene was mapped on chromosome region 10q23-q24. We report the first European cases of UFS. Haplotype analyses in our French family were compared with those previously described in patients from Columbia and America (literature data). The results are compatible with the same localization of the critical region and favor the hypothesis of genetic homogeneity. ( info)

15/163. urethral stricture associated with malacoplakia: a case report and review of the literature.

    A 68-year-old man presented with obstructive and irritative lower urinary tract symptoms and microscopic hematuria. Cystourethroscopy showed a circumferential stricture in the bulbar urethra that bled easily on contact. biopsy revealed malacoplakia. There was also focal nonspecific cystitis. The patient improved symptomatically, but the microscopic hematuria persisted. Follow-up biopsies showed persistent urethral malacoplakia and stricture. malacoplakia of the male urethra is exceptionally rare, this being the second reported case. ( info)

16/163. prenatal diagnosis of fetal urinary ascites.

    We report on a rare in utero appearance of the rupture of the fetal bladder caused by low urinary tract obstruction with subsequent urinary ascites. The findings on prenatal sonography, postnatal X-ray examinations and postnatal surgical treatment are described and the literature is reviewed. ( info)

17/163. Bladder diverticula causing posterior urethral obstruction in children.

    Two cases of bladder diverticula causing posterior urethral obstruction, which was corrected surgically, are presented. Five similar cases have been found in the literature. ( info)

18/163. Anterior urethral valves--a rare cause of urethral obstruction.

    Anterior urethral valves is a very rare congenital anomaly of the anterior urethra. It produces symptoms of varied intensity with the most severe ones leading to Urosepsis and renal failure (similar to as in posterior urethral valves) to the milder ones which may not produce any obstruction at all. However unlike the Posterior urethral valves this anomaly has milder and reversible sequelae in majority of the cases. The clinical presentation is mainly in the form of obstructed stream of urine and UTI and the diagnosis depends essentially on Micturating Cystographic studies and on the awareness and a high level of suspicion of its existence among Paediatricians and general practitioners. ( info)

19/163. Simultaneous upper and lower urinary tract obstruction associated with severe genital prolapse: diagnosis and evaluation with magnetic resonance imaging.

    Genital prolapse causing both urethral and ureteral obstruction is an infrequent occurrence, especially in the absence of uterine prolapse. We report on a patient with massive genital prolapse causing both urethral and ureteral obstruction in whom magnetic resonance imaging demonstrated the level of obstructive uropathy and, after surgical repair of the prolapse, confirmed restoration of the normal pelvic and upper urinary tract anatomy. ( info)

20/163. Posterior urethral valves presented at birth despite normal prenatal ultrasound scans.

    Most patients with significant posterior urethral valves are identified antenatally. However, even with a normal antenatal ultrasound scan, posterior urethral valves may be detected after birth. We present a patient with significant upper urinary tract involvement identified 2 days after birth, despite a normal third-trimester antenatal ultrasound scan. ( info)
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