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  • Title: Surgical management of penoscrotal hypospadias in a child with Opitz G/BBB syndrome: a case report.
    Author: Ahmed F, Altam A, Alyhari Q, Badheeb M, Aljbri W, Al-Wageeh S, Al-Naggar A, Ghabisha S, Al-Shami E.
    Journal: Pan Afr Med J; 2023; 44():103. PubMed ID: 37250678.
    Abstract:
    Opitz G/BBB syndrome is a rare condition characterized by three significant anomalies; hypertelorism, cleft lip and palate, and hypospadias. However, other anomalies may be associated. Herein, we report a 4-year-old child presented with penoscrotal hypospadias. On examination, hypertelorism and cleft lip and palate were noticed, suggesting a diagnosis of Opitz G/BBB syndrome. The cleft lip was corrected in the first year, and a two-staged surgical approach was implemented for penoscrotal hypospadias. In the first stage, the chordee was corrected and urethral plate was reconstructed using a tabularized incised plate urethroplasty and testicular tunica vaginalis flap. In the second stage, the remanent hypospadias was corrected, and the meatal opening reached its normal location. In conclusion, a two-staged surgical approach for the treatment of penoscrotal hypospadias associated with Opitz G/BBB syndrome may provide excellent outcomes in early-recognized cases. The urologist should pay attention to abnormal facial characteristics in patients with hypospadias.
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