Micropenis: Etiology, Diagnosis and Treatment Approaches

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Abstract

Micropenis is a medical diagnosis based on correct measurement of length. If stretched penile length is below the value corresponding to - 2.5 standard deviation of the mean in a patient with normal internal and external male genitalia, a diagnosis of micropenis is considered. Micropenis can be caused by a variety of factors including structural or hormonal defects of the hypothalamic-pituitary-gonadal axis. It can also be a component of a number of congenital syndromes. For the etiological evaluation, endocrinologic tests are important. This article reviews the etiology, diagnosis, treatment and management of micropenis.

Conflict of interest:None declared.

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Most cited references 45

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A window of opportunity: the diagnosis of gonadotropin deficiency in the male infant.

Melvin M. Grumbach (2005)

A common cause of micropenis is congenital hypogonadotropic hypogonadism, whether isolated or associated with multiple pituitary hormone deficiencies. The postnatal surge in FSH, LH, and testosterone in the male infant as a consequence of the continued function of the fetal GnRH pulse generator provides a 6-month window of opportunity to establish the diagnosis of hypogonadotropic hypogonadism and alert the clinician to the possibility of its association with multiple pituitary hormone deficiencies. When ACTH or GH deficiency or both deficiencies are present, hypoglycemia and cortisol deficiency can lead to neonatal and infantile death or increased morbidity. Establishing the diagnosis of hypogonadotropic hypogonadism in infancy preempts the uncertainties and delays in distinguishing constitutional delay in puberty from hypogonadotropic hypogonadism. Accordingly, hormone replacement therapy can be initiated at the normal age of pubertal onset. The ontogenesis of infantile testicular function, including the possible significance of the infantile surge in gonadotropins and testosterone, is reviewed. The molecular basis for certain developmental disorders associated with hypogonadotropic hypogonadism and micropenis is considered and the management and treatment of congenital hypopituitarism discussed.

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Penile reconstruction: is the radial forearm flap really the standard technique?

Stan Monstrey, Piet Hoebeke, Gennaro Selvaggi … (2009)

The ideal goals in penile reconstruction are well described, but the multitude of flaps used for phalloplasty only demonstrates that none of these techniques is considered ideal. Still, the radial forearm flap is the most frequently used flap and universally considered as the standard technique. In this article, the authors describe the largest series to date of 287 radial forearm phalloplasties performed by the same surgical team. Many different outcome parameters have been described separately in previously published articles, but the main purpose of this review is to critically evaluate to what degree this supposed standard technique has been able to meet the ideal goals in penile reconstruction. Outcome parameters such as number of procedures, complications, aesthetic outcome, tactile and erogenous sensation, voiding, donor-site morbidity, scrotoplasty, and sexual intercourse are assessed. In the absence of prospective randomized studies, it is not possible to prove whether the radial forearm flap truly is the standard technique in penile reconstruction. However, this large study demonstrates that the radial forearm phalloplasty is a very reliable technique for the creation, mostly in two stages, of a normal-appearing penis and scrotum, always allowing the patient to void while standing and in most cases also to experience sexual satisfaction. The relative disadvantages of this technique are the rather high number of initial fistulas, the residual scar on the forearm, and the potential long-term urologic complications. Despite the lack of actual data to support this statement, the authors feel strongly that a multidisciplinary approach with close cooperation between the reconstructive/plastic surgeon and the urologist is an absolute requisite for obtaining the best possible results.

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Lack of androgen receptor expression in Sertoli cells accounts for the absence of anti-Mullerian hormone repression during early human testis development.

Jean-Yves Picard, Kahina Boukari, Laetitia Martinerie … (2009)

Puberty is associated with increased testicular testosterone (TT) synthesis, which is required to trigger spermatogenesis and to repress anti-Mullerian hormone (AMH) production. However, testicular gonadotropin stimulation during fetal and newborn life neither initiates spermatogenesis nor represses AMH. We postulated that a lack of androgen receptor (AR) expression in Sertoli cells (SC) might explain why these processes do not occur during early human development. Using immunohistochemistry and quantitative PCR, we examined the relationship between AR, AMH, and FSH receptor expression in fetal, newborn, and adult human testis. The ability of testosterone to repress AMH secretion was evaluated in male newborns, neonates, and two adults with androgen insensitivity syndrome and also in vitro using SMAT1 SC. FSH receptor was present in SC at all developmental stages. In fetal and newborn testis, AR was expressed in peritubular and Leydig cells but not in SC. This coincided with the absence of spermatogenesis and with strong SC AMH expression. In adult testis, spermatogenesis was associated with AR expression and with a decrease in SC AMH content. Accordingly, AR mRNA expression was lower and AMH mRNA expression higher in fetal testes than in adult testes. In androgen insensitivity syndrome patients, combined gonadotropin stimulation induced an increase in circulating testosterone and AMH, a finding consistent with a failure of TT to repress AMH in the absence of AR signalling. Finally, direct androgen repression of AMH only occurred in AR-expressing SMAT1 cells. Functional ARs are essential for TT-mediated AMH repression in SC.

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Author and article information

Journal

Journal ID (nlm-ta): J Clin Res Pediatr Endocrinol

Journal ID (iso-abbrev): J Clin Res Pediatr Endocrinol

Journal ID (publisher-id): JCRPE

Title: Journal of Clinical Research in Pediatric Endocrinology

Publisher: Galenos Publishing

ISSN (Print): 1308-5727

ISSN (Electronic): 1308-5735

Publication date (Print): December 2013

Publication date (Electronic): 12 December 2013

Volume: 5

Issue: 4

Pages: 217-223

Affiliations

[1 ] Erciyes University Faculty of Medicine, Department of Pediatric Endocrinology, Kayseri, Turkey

Author notes

* Address for Correspondence: Erciyes University Faculty of Medicine, Department of Pediatric Endocrinology, Kayseri, Turkey Phone: +90 352 438 00 76 E-mail: nihalhatipoglu@ 123456yahoo.com

Article

Publisher Item ID: 1161

DOI: 10.4274/Jcrpe.1135

PMC ID: 3890219

PubMed ID: 24379029

SO-VID: 6e6b0975-8e60-4677-a942-0468b9700499

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This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Micropenis: Etiology, Diagnosis and Treatment Approaches

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Most cited references 45

A window of opportunity: the diagnosis of gonadotropin deficiency in the male infant.

Penile reconstruction: is the radial forearm flap really the standard technique?

Lack of androgen receptor expression in Sertoli cells accounts for the absence of anti-Mullerian hormone repression during early human testis development.

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