Genital Abnormality - Hypospadia: Difference between revisions

Revision as of 12:09, 14 December 2010

Introduction

Hypospadias are the most common penis abnormality (1 in 300) and result from a failure of male urogenital folds to fuse in various regions, the normal process is described as virilization of the external genitalia. This in turn leads to resulting in a proximally displaced urethral meatus or opening. The cause is unknown, but suggested to involve many factors either indivdually or in combination including: familial inheritance, low birth weight, assisted reproductive technology, advanced maternal age, paternal subfertility and endocrine-disrupting chemicals.^[1] Infants with hypospadias should not undergo circumcision.

Some Recent Findings

Correction of Distal Penile Hypospadias
- "The major aims of correction of hypospadias include construction of a neourethra, removal of the chordee, and reduction of the probability of postoperative fistulization. Here, we describe a new technique that can be easily used in cases with subcoronal distal penile hypospadias (even those with chordee) for the construction of the urethra using meatus-based transverse flaps."^[2]
- "Meatal mobilization (MEMO) by distal urethral preparation has been demonstrated to be an efficient surgical technique for the correction of distal hypospadias offering excellent short-term success rates."^[3]

Hypospadia Classification

Classification of Hypospadias^[1]

The condition is classified by the general location of the relocated opening (meatus) and lack of fusion.

Hypospadia Classification	Meatus Opening	Image
Anterior	on inferior surface of glans penis	A
Coronal	in balanopenile furrow	B
Distal	on distal third of shaft	C
Penoscrotal	at base of shaft in front of scrotum	D
Scrotal	on scrotum or between the genital swellings	E
Perineal	behind scrotum or genital swellings	F

Surgical Repair

Depending on the class of hypospadias there are a number of different surgical repair techniques including: orthoplasty or penile straightening, urethroplasty, meatoplasty and glanuloplasty, scrotoplasty (oscheoplasty) and skin coverage.

Maternal Factors

Data from a case-control study in Sweden and Denmark (2000 - 2005) identified a number of maternal factors that may be associated with hypospadias.^[4]

Maternal diet during pregnancy lacking both fish and meat - more than 4-fold increased risk
Maternal obesity - more than 2-fold increased risk
Maternal hypertension - 2.0-fold increased risk
Maternal absence of nausea - 1.8-fold increased risk
Maternal nausea in late pregnancy - also appeared to be positively associated with increased risk

Molecular Factors

chromosomal abnormalities
HOX, FGF, Shh - genes of penile development
WT1, SRY - testicular determination
luteinizing hormone (LH) receptor
5alpha reductase, androgen receptor - action of androgen
CXorf6
ATF3

Links: OMIM - CXorf6

Related Genetic Conditions

X-linked isolated hypospadias-1 HYSP1 OMIM 300633
X-linked isolated hypospadias-2 HYSP2 OMIM 300758
X-linked isolated hypospadias-3 HYSP3 OMIM 146450
Johanson-Blizzard syndrome^[5] - hypospadias, failure to thrive, exocrine pancreatic deficiency, short stature and developmental delay, cutis aplasia on the scalp, aplasia of alae nasi, hypothyroidism, myxomatous mitral valve, and patent ductus arteriosus.

References

↑ ^1.0 ^1.1 <pubmed>11713004</pubmed>
↑ <pubmed>20548222</pubmed>
↑ <pubmed>20450394</pubmed>
↑ <pubmed>18709149</pubmed>
↑ <pubmed>20556423</pubmed>

Reviews

Articles

Search Pubmed

June 2010 "Hypospadia" All (4698) Review (377) Free Full Text (413)

Search Pubmed: Hypospadia | Hypospadia Surgical Repair

External Links

EuroDSD programme

Glossary Links

Glossary: A | B | C | D | E | F | G | H | I | J | K | L | M | N | O | P | Q | R | S | T | U | V | W | X | Y | Z | Numbers | Symbols | Term Link

Cite this page: Hill, M.A. (2024, May 1) Embryology Genital Abnormality - Hypospadia. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/Genital_Abnormality_-_Hypospadia

What Links Here?

[PMID11713004-1] 1.0 ^1.1 <pubmed>11713004</pubmed>

[2] <pubmed>20548222</pubmed>

[3] <pubmed>20450394</pubmed>

[4] <pubmed>18709149</pubmed>

[5] <pubmed>20556423</pubmed>

[1]

[2]

[3]

[4]

[5]

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 * '''Correction of Distal Penile Hypospadias'''
-**<ref><pubmed>20548222</pubmed></ref> "The major aims of correction of hypospadias include construction of a neourethra, removal of the chordee, and reduction of the probability of postoperative fistulization. Here, we describe a new technique that can be easily used in cases with subcoronal distal penile hypospadias (even those with chordee) for the construction of the urethra using meatus-based transverse flaps."
+** "The major aims of correction of hypospadias include construction of a neourethra, removal of the chordee, and reduction of the probability of postoperative fistulization. Here, we describe a new technique that can be easily used in cases with subcoronal distal penile hypospadias (even those with chordee) for the construction of the urethra using meatus-based transverse flaps."<ref><pubmed>20548222</pubmed></ref>
-** <ref><pubmed>20450394</pubmed></ref> "Meatal mobilization (MEMO) by distal urethral preparation has been demonstrated to be an efficient surgical technique for the correction of distal hypospadias offering excellent short-term success rates."
+** "Meatal mobilization (MEMO) by distal urethral preparation has been demonstrated to be an efficient surgical technique for the correction of distal hypospadias offering excellent short-term success rates."<ref><pubmed>20450394</pubmed></ref>
 |}