Aarskog syndrome

Shinkawa, Tohru; Yamauchi, Yoshizumi; Osada, Yoshihito; Ishisawa, Nobuyuki

doi:10.1016/0090-4295(83)90311-4

Cited by 9 publications

(1 citation statement)

References 4 publications

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“…Two different studies by 2 studies conducted by Polat et al [1] and Donnahoo et al [7] revealed that about 30% of patients with chordee and normal meatus are categorized in groups 4 and 5. Peno-scrotal transposition, including complete and partial transposition, may accompany sex chromosomal abnormalities [8], Aarskog syndrome [9] and urinary tract anomalies like renal agenesis and dysplasia [10], so these patients should undergo KUB, sonography and VCUG. In the management of chordee without hypospadias, penile degloving and artificial erection should always be done in order to obtain an exact evaluation of chordee and possible causes [3].…”

Section: Discussionmentioning

confidence: 99%

A Patient with Third Corpus Cavernosum, Severe Penile Ventral Deviation and Partial Peno-scrotal Transposition: First Worldwide Report

2018

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Chordee – a variety of penile deviations with or without hypospadias – is a relatively prevalent condition. This is quite a unique kind of chordee and peno-scrotal transposition with a considerable tension effect on the ventral side of the penis due to an additional corpus cavernosum, and this condition is confirmed by histopathologic evaluation. We treated the patient by wide resection of the third corpus cavernosum with scrotoplasty and postponed the urethroplasty for a second stage repair. It was interesting that we did not come across a similar report as a result of our deep literature search.

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Section: Discussionmentioning

confidence: 99%

A Patient with Third Corpus Cavernosum, Severe Penile Ventral Deviation and Partial Peno-scrotal Transposition: First Worldwide Report

2018

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Aarskog syndrome: Report of a family with review and discussion of nosology

1993

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Five individuals in one family, including dizygotic male twins, a half brother and their mother, had Aarskog syndrome (AS). Phenotypic variability is wide not only between mother and sons but also between sibs. Collectively, the affected relatives have the full spectrum of findings seen in AS. Based on analysis of this family and others from the literature, we derive primary and secondary diagnostic criteria for AS. Primary criteria include: short stature, hypertelorism, short nose with anteverted nares, maxillary hypoplasia, a crease below the lower lip, mild interdigital webbing with short and broad hands, short fifth finger with clinodactyly, and shawl scrotum. Secondary criteria include: abnormal auricles with fleshy lobules, posteriorly angulated ears, widow's peak, ptosis, downward slant of palpebral fissures, joint hyperextensibility, broad feet with bulbous toes, cryptorchidism, inguinal hernia, and prominent umbilicus. Literature pertaining to the clinical manifestations and genetics of AS is reviewed and nosology of similar syndromes is discussed.

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