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1.
Urologe A ; 59(7): 825-828, 2020 Jul.
Article in German | MEDLINE | ID: mdl-32472223

ABSTRACT

Aphallia is an extremely rare congenital malformation of unknown cause. The incidence is reported in the literature to be 1 in 10-30 million live births. Almost 100 cases have been described to date. Aphallia is associated with other congenital malformations (in particular urogenital and gastrointestinal anomalies) in >50% of cases. The diagnosis is made clinically and shows the complete absence of the corpora cavernosa and the corpus spongiosum with a urethral opening along the perineal midline (most frequently ventral to the anus and in the ventral rectal wall). Two case reports from the authors' department: The first child was a male newborn (46,XY) with penis agenesis and additional bilateral intraabdominal testis, an anorectal malformation (ARM) with a rectovesical fistula, as well as left renal duplication and grade III vesico-ureteral reflux on the right side. The second child was a male newborn (46,XY) with aphallia without further urological or anorectal malformation. Only right inguinal hernia was present. In the first patient, several corrective surgeries were performed in the further course in view of the additional malformations. With regard to the aphallia, the various temporary treatment options (scrotal or parascrotal phalloplasty or penis prosthesis) were discussed with the parents. Masculinizing surgery by means of definitive phalloplasty was planned once the patient has reached puberty. Due to the technical demands of phallus reconstruction, feminization is still favored in some countries in the literature, which nowadays, however, cannot be justified medically or legally.


Subject(s)
Abnormalities, Multiple/pathology , Anorectal Malformations , Genital Diseases, Male , Kidney/abnormalities , Penis/abnormalities , Urogenital Abnormalities/pathology , Androgens , Child , Humans , Infant, Newborn , Male , Scrotum , Sexual Maturation , Urethra
2.
Geburtshilfe Frauenheilkd ; 75(9): 945-948, 2015 Sep.
Article in English | MEDLINE | ID: mdl-26500372

ABSTRACT

Background: Female genital mutilation (FGM) occurs mainly in Africa, parts of the Arabian Peninsula and parts of Asia. It is commonly associated with acute complications as well as diverse late/delayed complications. One of the most common of these late complications is progressively enlarging painless cysts of the vulva. Case Report: An 8-year-old girl from Eritrea presented to our paediatric emergency department with a progressively enlarging mass of the vulva. She had undergone a clitoridectomy and partial removal of the labia minora as an infant in Eritrea. We performed surgical excision of the cyst and reconstruction of the labia. Histology showed a traumatic squamous epithelial inclusion cyst of the vulva. Conclusion: Epithelial or dermoid cysts of the vulva following FGM are extremely rare. Symptoms often require surgical intervention. Through increasing migration, more girls and female youths with FGM are likely to present to practices and hospitals in Germany. Thus increased knowledge and awareness of the medical complications of FGM and their treatment will be necessary in years to come.

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