WBR0316

Author	[[PageAuthor::Ogheneochuko Ajari, MB.BS, MS [1] (Reviewed by Yazan Daaboul, M.D.)]]
Exam Type	ExamType::USMLE Step 1
Main Category	MainCategory::Embryology
Sub Category	SubCategory::Reproductive
Prompt	[[Prompt::Following delivery, a 28-year-old woman states that her newborn boy is continuously dripping urine. On physical examination, the pediatrician notes the newborn has an abnormal opening of the penile urethra on the dorsal side of his penis. When asked, the physician explains that the condition is caused by the faulty positioning of the genital tubercle during development and states that the patient will require surgical management. Which of the following conditions is this patient's most likely diagnosis?]]
Answer A	AnswerA::Hydrocele of the testis
Answer A Explanation	[[AnswerAExp::Hydrocele of the testis results from the development of a fluid-filled sac within the scrotum. It is often caused by an incomplete obliteration of the processus vaginalis.]]
Answer B	AnswerB::Cryptorchidism
Answer B Explanation	[[AnswerBExp::Cryptorchidism results from failure of the testes to descend into the scrotum. Since the majority of cases do not spontaneously correct, early surgical correction is required before 6 months of age, given the strong association between cryptorchidism and testicular cancer. Postpubertal males are advised to undergo bilateral orchiectomy because approximately 20% of testicular cancers in cryptorchidism occur in the normally descended testis.]]
Answer C	AnswerC::Bifid scrotum
Answer C Explanation	[[AnswerCExp::Bifid scrotum results from the malunion of the labioscrotal folds that form the labia majora in females.]]
Answer D	AnswerD::Hypospadias
Answer D Explanation	[[AnswerDExp::Hypospadias results from an abnormal opening of the penile urethra on the ventral (inferior) side of the penis due to the failure of the urethral folds to close.]]
Answer E	AnswerE::Epispadias
Answer E Explanation	[[AnswerEExp::Epispadias in males results from the the faulty positioning of the genital tubercle that leads to an abnormally located dorsal (superior) urethra during development.]]
Right Answer	RightAnswer::E
Explanation	[[Explanation::Epispadias in males results from the the faulty positioning of the genital tubercle that leads to an abnormally located dorsal (superior) urethra during development. Due to the involvement of the urinary sphincter, patients typically present with urinary incontinence, which may include involuntary urine loss and continuous urine dripping through the meatus. Epispadias is associated with bladder and cloaca extrophy; the combination of both diseases is referred to as the extrophy-epispadias complex. Other genitourinary and abdominal anomalies may also be associated with the extrophy-epispadias complex. Diagnosis is usually made clinically at birth; but prenatal ultrasound may demonstrate a repeatedly empty bladder and may help in the early diagnosis. Management includes surgical genital reconstruction, but additional surgeries may be necessary in the presence of associated diseases. Educational Objective: Epispadias in males results from the the faulty positioning of the genital tubercle that leads to an abnormally located dorsal urethra during development. Due to the involvement of the urinary sphincter, patients typically present with urinary incontinence, which may include involuntary urine loss and continuous urine dripping through the meatus. Epispadias is associated with bladder and cloaca extrophy. References: Ebert AK, Reutter H, Ludwig M, et al. The extrophy-epispadias complex. Orph J Rare Dis. 2009;4:23. First Aid 2014 page 562]]
Approved	Approved::Yes
Keyword	WBRKeyword::Fetal development, WBRKeyword::Genital tubercle, WBRKeyword::Penis, WBRKeyword::Epispadias, WBRKeyword::Bladder extrophy, WBRKeyword::Cloaca extrophy, WBRKeyword::Genitourinary, WBRKeyword::Newborn, WBRKeyword::Congenital
Linked Question	Linked::
Order in Linked Questions	LinkedOrder::