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Bifid clitoris

MedGen UID:
234652
Concept ID:
C1389795
Congenital Abnormality
HPO: HP:0030911

Definition

Two clitorides located side by side. [from HPO]

Conditions with this feature

Bladder exstrophy-epispadias-cloacal extrophy complex
MedGen UID:
374033
Concept ID:
C1838703
Disease or Syndrome
Bladder exstrophy and epispadias complex (BEEC) is an anterior midline defect with variable expression involving the infraumbilical abdominal wall including the pelvis, urinary tract, and external genitalia (Gearhart and Jeffs, 1998). BEEC is one of the most severe urologic birth defects because of its profound impact on continence, sexual function, and morbidity due to the effect of chronic and recurrent infections on renal function. The term 'exstrophy,' derived from the Greek work ekstriphein, which literally means 'turn inside out,' was first used by Chaussier in 1780. Martinez-Frias et al. (2001) emphasized that exstrophy of the cloaca and exstrophy of the bladder are 2 different expressions of a primary developmental field defect. Cloacal exstrophy is a feature of the OEIS (omphalocele-exstrophy-imperforate anus-spinal defects) complex (258040). Exstrophy of the cloaca includes the persistence and exstrophy of a common cloaca that receives ureters, ileum, and a rudimentary hindgut and is associated with failure of fusion of the genital tubercles and pubic rami, incomplete development of the lumbosacral vertebrae with spinal dysraphism, imperforate anus, cryptorchidism and epispadias in males and anomalies of the mullerian duct derivatives in females, and a wide range of urinary tract anomalies. Omphalocele is common, and most patients have a single umbilical artery. Reutter et al. (2016) reviewed the epidemiology, potential mechanisms, and animal models for BEEC. They described BEEC as a spectrum of component malformations of variable severity, including epispadias as the mildest phenotype and classic bladder exstrophy as the most common, with cloacal exstrophy representing the most severe form. In approximately one-third of cases, urologic malformations are present, including ectopic kidney, renal agenesis, and/or hydronephrosis. Other malformations involving the gastrointestinal, skeletal, spinal, and genitourinary systems, including cryptorchidism and ambiguous genitalia, are reported frequently. The authors noted that cloacal exstrophy is considered by some to have a different embryologic origin from classic bladder exstrophy.

Professional guidelines

PubMed

Frimberger D
Semin Pediatr Surg 2011 May;20(2):85-90. doi: 10.1053/j.sempedsurg.2011.01.003. PMID: 21453851

Recent clinical studies

Etiology

Shetty MV, Bhaskaran A, Sen TK
Afr J Paediatr Surg 2011 May-Aug;8(2):215-7. doi: 10.4103/0189-6725.86066. PMID: 22005369
Frimberger D
Semin Pediatr Surg 2011 May;20(2):85-90. doi: 10.1053/j.sempedsurg.2011.01.003. PMID: 21453851
Kajbafzadeh AM, Talab SS, Elmi A, Tourchi A
BJU Int 2011 Oct;108(7):1200-7. Epub 2011 Jan 11 doi: 10.1111/j.1464-410X.2010.10022.x. PMID: 21223473
VanderBrink BA, Stock JA, Hanna MK
J Plast Reconstr Aesthet Surg 2010 Dec;63(12):2141-5. Epub 2010 Mar 29 doi: 10.1016/j.bjps.2010.02.005. PMID: 20303329
Ekenze SO, Mbadiwe OM, Ezegwui HU
J Paediatr Child Health 2009 Oct;45(10):610-3. Epub 2009 Sep 14 doi: 10.1111/j.1440-1754.2009.01574.x. PMID: 19751373

Diagnosis

Ali S, Moosa H, Khan SA
J Coll Physicians Surg Pak 2020 Apr;30(4):438-439. doi: 10.29271/jcpsp.2020.04.438. PMID: 32513370
Shetty MV, Bhaskaran A, Sen TK
Afr J Paediatr Surg 2011 May-Aug;8(2):215-7. doi: 10.4103/0189-6725.86066. PMID: 22005369
Frimberger D
Semin Pediatr Surg 2011 May;20(2):85-90. doi: 10.1053/j.sempedsurg.2011.01.003. PMID: 21453851
Tantibhedhyangkul J, Copland SD, Haqq AM, Price TM
Fertil Steril 2008 Nov;90(5):2017.e1-3. Epub 2008 Mar 7 doi: 10.1016/j.fertnstert.2007.12.055. PMID: 18314106Free PMC Article
Allen L, Rodjani A, Kelly J, Inoue M, Hutson JM
BJU Int 2004 Sep;94(4):613-5. doi: 10.1111/j.1464-4096.2004.04920.x. PMID: 15329123

Therapy

Ekenze SO, Mbadiwe OM, Ezegwui HU
J Paediatr Child Health 2009 Oct;45(10):610-3. Epub 2009 Sep 14 doi: 10.1111/j.1440-1754.2009.01574.x. PMID: 19751373

Prognosis

Shetty MV, Bhaskaran A, Sen TK
Afr J Paediatr Surg 2011 May-Aug;8(2):215-7. doi: 10.4103/0189-6725.86066. PMID: 22005369
Kajbafzadeh AM, Talab SS, Elmi A, Tourchi A
BJU Int 2011 Oct;108(7):1200-7. Epub 2011 Jan 11 doi: 10.1111/j.1464-410X.2010.10022.x. PMID: 21223473
Damario MA, Carpenter SE, Jones HW Jr, Rock JA
Int J Gynaecol Obstet 1994 Mar;44(3):245-53. doi: 10.1016/0020-7292(94)90174-0. PMID: 7909763

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