Urethral Duplication Research Articles

Urethral duplication associated with complex chordee: a narrative review of literature and report of a case

BackgroundUrethral duplication (UD) is reportedly rare. It is rarer in females. Knowledge on this anomaly comes from isolated report of cases. The aim of this review is to summarize information available on this anomaly thereby revealing gaps in knowledge, and to appropriately situate a recently managed case.MethodsPublications on UD in English language from 2001 to 2021 were searched for in the literature. Of importance were age at presentation, class of UD, nature of associated penile deformity and other structural anomalies. Available information was used to synthesize opinions after descriptive analyses using SPSS® version 21 (IBM Co., Armonk, NY, USA). In addition, a recently managed case of UD was reported and appropriately situated in the discourse.ResultIn all, 115 articles met the inclusion criteria. Majority (75.7%) were individual case reports, while the rest were retrospective review of case series. These provided a total of 269 reported cases. Of this lot, 38 cases were excluded because they either had no Effmann’s class or were not described well enough for an Effmann’s class to be assigned. Ultimately, 231 cases formed the basis for this review. Male to female ratio was 12:1. Types III and IIB had more females. Types IIA 2 (26.0%), IIA 2 “Y” (26.4%) and IA (22.5%) were frequently reported. About 61.8% males and 68.4% females had no associated defects. Isolated dorsal chordee was prevalent (7.1%), especially among type IA (16.7%) UD. Reported in 10.4%, vesicoureteric reflux may not be attributable always to high pressure voiding.A boy who presented at the age of 18 years with Effmann type IA UD and an associated complex chordee of the penile shaft was reported. The complex nature of the chordee adds to the challenge of explaining associated penile defects in UD.ConclusionUD is rare, but reported from all parts of the globe. There are a number of associated defects involving the external genitalia and other organs reported in UD in the male. The mechanisms of these defects are yet to be fully understood.

Urethral duplication as cause of incontinence in a 5-years-old female: Surgical approach

Duplication of the urethra is a rare congenital anomaly in females which, if left unidentified, might cause refractory urinary incontinence. This video demonstrates its identification and treatment. A 5-year-old female was referred for primary refractory diurnal incontinence without any dry moment since birth. Initial clinical examination showed permanent urine leakage from an opening cranial to the clitoris. Ultrasound did not show any bladder or kidney abnormalities. A diagnostic cystoscopy under anesthesia revealed an accessory meatus located cranially to the orthotopic meatus and to the clitoris. Lying in dorsal decubitus under general anesthesia, catheters were inserted in respectively the orthotopic and accessory meatus. The accessory meatus and urethra were dissected away into the bladder dome from a circumferential skin incision. Defects were closed and bladder drainage per urethram was left in situ for 10 days. P ostoperative recovery was uncomplicated. Complete resolution of the incontinence was immediately achieved at catheter removal. Daytime and night-time continence are observed with 3 years follow-up. Urethral duplication is a rare finding in females but should be excluded during the workup of primary incontinence. In girls with isolated urethral duplication, resection of the accessory urethra is the best surgical option.

Anorectal malformation, urethral duplication, occult spinal dysraphism (ARM-UD-OSD): a challenging uncommon association.

Urethral duplication (UD) is a rare malformation, which can be associated with other anomalies, like anorectal malformations (ARM). ARM has been described with occult spinal dysraphism (OSD). No ARM-UD-OSD combination has been reported. To share our experience andto discuss the management of ARM-UD-OSD association. We retrospectively reviewed records of five boys with UD. Four of these had ARM-UD-OSD association. ARM was the first diagnosis in all; OSD and UDwas detected during screening for associated malformation. All patients underwent ARM correction, 3 after colostomy. All reached fecal continence, 3 are performing bowel management. Three patients underwent UD surgical correction. Because of symptoms' worsening, 2 children had detethering surgery. At a mean follow-up of 9.5years, all patients have normal renal function, 3 are on clean intermittent catheterization (CIC) for neurogenic bladder (1 has a cystostomy, another one an appendicostomy). UD and OSD should be considered in patients with ARM. Children with these conditions associated must be centralized in a third-level Center and management carefully planned; in particular, urethral reconstruction should be weighed, considering CIC could be required. Suspicion of neurogenic bladder must be present in OSD patient.

Клінічний випадок подвоєння уретри зі збереженням функції та єдиним сечовим міхуром

Urethral doubling is a rare congenital anomaly of the urinary system that is more common in boys. Less than 500 clinical cases of this disease have been described in the literature, which may signal a lack of awareness among physicians. Although there are many considerations regarding the embryology of this anomaly, the etiopathogenesis of its various forms remains unclear. Sometimes doubling of the urethra is combined with other malformations, such as epispadias, hypospadias, exstrophy of the bladder, anorectal malformation, doubling of the bladder and others. In this article, we present a clinical case of a 9-year-old boy with urethral duplication and additional urethral epispadias. We found the main and additional urethra, which goes on dorsal side of the penis and led to curvature of the penis and urinary incontinence. During the operation, urethrocystoscopy was performed before the main stage, and a complete doubling of the type 2-A urethra was established according to the Effman classification, after which the additional urethra was excised as far as the pubic bones. Urethral duplication is a rare anomaly, with several forms of clinical presentation, often accompanied by other anomalies, and sometimes with difficult diagnosis. The treatment of urethral duplication should be individualized, according to its type. In this case we find urethral duplication type IIa, which has been treated as classic epispadias. Therefore, in child with congenital malformation of penis need to perform obsrvetion like urethrography, cystoscopy. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors. Key words: urethral substitution, epispadias.

V08-08 MANAGEMENT OF URETHRAL DUPLICATION

You have accessJournal of UrologyCME1 May 2022V08-08 MANAGEMENT OF URETHRAL DUPLICATION Patrick Curtin, Yu Zheng, Jessica Connor, and Shumyle Alam Patrick CurtinPatrick Curtin More articles by this author , Yu ZhengYu Zheng More articles by this author , Jessica ConnorJessica Connor More articles by this author , and Shumyle AlamShumyle Alam More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000002606.08AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Urethral duplication is a rare congenital condition in which there is a complete or partial duplication of the urethra. As of 2016 there were less than 300 reported cases of this condition reported in the literature. This video sought to demonstrate the operative technique used to correct a Type 1B urethral duplication. METHODS: After informed consent was obtained photos and video footage were collected. RESULTS: This video includes epidimiologic information on urethral duplication, a patient presentation of Type 1B urethral duplication, and the surgical technique used to correct this. CONCLUSIONS: Urethral duplication is a rare congenital malformation that can be surgically corrected. The exact type of surgical correction is dependent on the type of duplication as well as any concomitant genitourinary abnormalities. Source of Funding: No funding © 2022 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 207Issue Supplement 5May 2022Page: e716 Advertisement Copyright & Permissions© 2022 by American Urological Association Education and Research, Inc.MetricsAuthor Information Patrick Curtin More articles by this author Yu Zheng More articles by this author Jessica Connor More articles by this author Shumyle Alam More articles by this author Expand All Advertisement PDF DownloadLoading ...

V04-09 UNDOUBLED BLADDER IN THE SAGITTAL PLANE WITH ONE EXSTROPHIC BLADDER: A CASE REPORT ON RECONSTRUCTIVE TECHNIQUE

You have accessJournal of UrologyCME1 May 2022V04-09 UNDOUBLED BLADDER IN THE SAGITTAL PLANE WITH ONE EXSTROPHIC BLADDER: A CASE REPORT ON RECONSTRUCTIVE TECHNIQUE Wietse Claeys, Caroline Jamaer, Camille Berquin, Irina Verbakel, Achilles Ploumidis, Erik Van Laecke, Piet Hoebeke, and Anne-Françoise Spinoit Wietse ClaeysWietse Claeys More articles by this author , Caroline JamaerCaroline Jamaer More articles by this author , Camille BerquinCamille Berquin More articles by this author , Irina VerbakelIrina Verbakel More articles by this author , Achilles PloumidisAchilles Ploumidis More articles by this author , Erik Van LaeckeErik Van Laecke More articles by this author , Piet HoebekePiet Hoebeke More articles by this author , and Anne-Françoise SpinoitAnne-Françoise Spinoit More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000002560.09AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: The Exstrophy-Epispadias complex is a spectrum of rare congenital malformations that can involve multiple organ systems. It can range from a simple glandular defect to a complete multisystem malformation with cloacal exstrophy and musculoskeletal diastasis. We report on the case of an adult woman who was born with an exstrophy variant with bladder and urethral duplication in the sagittal plane. The aim of our study was to report our reconstructive approach in this rare case of bladder exstrophy with maximum use of available tissues. METHODS: A 27-year old female patient was referred to our center for the complaint complete incontinence since birth. Upon first clinical examination, we discovered an exstrophic bladder with blind ending ureteral orifices. Further evaluation revealed a urethral meatus caudal to the exstrophic bladder plate. Cystoscopy revealed a second non-exstrophic bladder in the sagittal plane with complete incontinence due to a broad open bladder neck. This bladder contained 2 orthotopic ureters with normal retrograde urethrography. Vaginoscopy revealed a short and small vaginal channel and an arcuate uterus. Plain radiography of the pelvis showed a diastasis of 5 centimeters. CT scanning could not reveal any aberrant anatomy in the upper urinary tract. Genetic testing showed a normal 46XX karyotype. A laparotomy incision is performed and the exstrophic bladder plate is dissected caudally up to the point where a ventral inlay in the non-exstrophic bladder can be performed as augmentation tissue. A Mitchell-type bladder neck reconstruction with fascia sling interposition is performed. Since we do not know if this non-exstrophic bladder will be capable of emptying or if the bladder neck reconstruction will remain patent, we chose for the interposition a continent Mitrofanoff-type vesicostomy. Genital reconstruction with re-anastomosis of the urethral meatus between the inner labia is performed. The patient was thought self-catheterization through the appendiceal channel. At this point the patient can hold around 250 ml in the bladder. She catheterizes 5 times a day and once at night. She is completely dry. RESULTS: We present our reconstructive technique in a step-by-step-video guide. Our main focus was to achieve continence by maximal use of available tissues with the best possible quality of life for the patient. CONCLUSIONS: No surgical technique will be suitable for all these varying anomalies. Therefore, reconstructive surgeons need to tailor their approach based on available tissues and functional goals. Source of Funding: none © 2022 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 207Issue Supplement 5May 2022Page: e369 Advertisement Copyright & Permissions© 2022 by American Urological Association Education and Research, Inc.MetricsAuthor Information Wietse Claeys More articles by this author Caroline Jamaer More articles by this author Camille Berquin More articles by this author Irina Verbakel More articles by this author Achilles Ploumidis More articles by this author Erik Van Laecke More articles by this author Piet Hoebeke More articles by this author Anne-Françoise Spinoit More articles by this author Expand All Advertisement PDF DownloadLoading ...

Voiding cysto-urethrography of complete urethral duplication in a child, a case report

BackgroundUrethral duplication is a very uncommon congenital anomaly that predominantly affects males. This condition is distinguished by the presence of two urethras, which might be incomplete or complete. The diagnosis of urethral duplication is very important, and missing this diagnosis leads to the delayed surgical correction of patients. Voiding cysto-urethrography is an important diagnostic method to diagnose the urethral duplication that can provide a standard view for surgical operation and determine the type of duplication.Case presentationIn the present study, we report a 5-year-old boy with complete urethral duplication diagnosed via voiding cysto-urethrography in our medical center. We reviewed the classification of this anomaly, indications of surgery, the importance of voiding cysto-urethrography, and the role of other imaging modalities to diagnose the urethral duplication.ConclusionFor children with a double urinary stream, a complete genital examination and radiological assessment are required not only to diagnose urethral duplication but also to determine the kind of urethral duplication and rule out related anomalies. These findings guide surgeons in planning the best treatment for the patients to prevent future complications. Voiding cysto-urethrography is one of the most useful and beneficial procedures to diagnose the urethral duplication which can be used as a first imaging modality, especially when there are double functional urethras.

Robotic-assisted laparoscopic repair of a congenital rectourethral fistula with duplicated urethra

To describe our experience with robot-assisted laparoscopic transperitoneal repair of a congenital rectourethral fistula in a pediatric patient with a urethral duplication. The patient is a 2-year-old male with a past medical history of Tetralogy of Fallot presenting with a febrile urinary tract infection (UTI). He was diagnosed with urethral duplication and a rectourethral fistula by voiding cystourethrogram (VCUG). The parents were counseled on various options and agreed to proceed with a robotic repair. Robotic-assisted transperitoneal rectourethral fistula repair was performed. The procedure time was 229min with an estimated blood loss (EBL) of 15mL. His postoperative course was unremarkable. At his 2-week follow-up, the urethral catheter was removed and the patient was voiding normally and having normal bowel movements. Congenital rectourethral fistula with urethral duplication is a rare anomaly with only a few reports in the literature. Pediatric robotic-assisted transperitoneal rectourethral fistula repair is a technically feasible approach in infants with minimal morbidity that allows for excellent visualization and avoids open repair.

An unusual Y-Type urethral duplication in a boy

Urethral duplication is a rare congenital anomaly but more common in males, it can take many forms and presentations due to the anatomical positions and histological epithelium of the urethrae. In sagittal duplications, the normal positioned dorsal urethra is hypoplastic and stenotic and the ventral one is almost always the more normal one. We present a case of a 4 years old boy and highlights a rare form of a dominant dorsal urethra and an accessory ventral one (type II A 2 Y according to effmann classification) and discuss its treatment.

Posterior Cloaca: Multicenter Experience of a Very Rare and Unusual Anorectal and Genitourinary Malformation.

Posterior cloacal malformations (PCMs) are distinguished from classical cloacal malformations by the posterior location of the common opening in the perineum. We aim to describe our experience of management of these rare and complex malformations. This study was a retrospective chart review of all patients with PCM who underwent treatment at Kalawati Saran Children's Hospital (KSCH), New Delhi, and Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, between 2013 and 2021. Individual anatomical variations and their impact on the clinical presentation, management, and final outcome (cosmesis and function) were recorded and analyzed. During the study period, four girls with a median age of 2 (range: 0-5) years presented to us with PCM. Three patients were referred to KSCH as ambiguous genitalia whereas one patient was born at SGPGIMS with an antenatal diagnosis of "urorectal septal defect." On examination, three patients had the urogenital sinus (UGS) opening immediately anterior to the normally located anal opening (2 orifices), whereas one had a single orifice at the normal location of the anus with the UGS opening in the anterior rectal wall. Associated anomalies included: (a) hydrocolpos (n = 3), which was managed by a tube vaginostomy; (b) urethral duplication with dorsal atretic urethra (n = 3); (c) uterine didelphys (n = 1); (d) bilateral grade 5 vesicoureteric reflux (n = 1); and (e) vaginal calculus (n = 1). Total urogenital mobilization (anterior sagittal approach) with feminizing genitoplasty was performed for patients with UGS and normal anus (n = 3). In the patient with a single opening at the normal location of the anus, posterior sagittal ano-recto-urethro-vaginoplasty was performed. At a median follow-up of 24 months, three patients are continent for urine and stool, whereas one patient is yet to be toilet trained. PCMs are unusual complex malformations that necessitate meticulous clinical examination, detailed diagnostic workup, and multistaged surgical management. Management should be tailored as per each individual patient's anatomy and clinical presentation for an optimal outcome.

Urorectal septum malformation sequence with a giant calculus in the colon cyst in an adult

Abstract Introduction: The present article reports urinary and digestive system abnormalities, including retrocaval ureter, anal atresia, and colon duct cyst with duplicate urethra carrying a giant calculus in a 40-year-old man. Patient concerns: A 40-year-old man visited the hospital complaining of acute pain in the right lower abdomen. Diagnosis: Cystoscopy showed that the patient had duplicated urethra and bladder, and pathology showed that the duplicated bladder consisted of colon tissue, which led to the diagnosis of a urorectal septum malformation sequence. Interventions and outcomes: The calculus was removed, and the retrocaval ureter was treated with laparoscopic ureteroureterostomy. We followed up for 3 months after the secondary surgery, and there were no complications. Conclusion: A urorectal septum malformation sequence with a giant calculus in a colon cyst is rare. Hence, clinicians should be aware of the possibility of calculus formation in patients with a urorectal septum malformation sequence.

Experiences in management of a Y-type urethral duplication

Urethral duplications are rare congenital malformations with a wide range of anatomic variants. Surgical management of Y-type urethral duplications is challenging and requires careful prior diagnostic workup and planning. In this article we report our case of a boy with steno-atretic Y-type urethral duplication who underwent transrectal resection of the ectopic urethra, failed progressive augmentation by dilating the orthotopic urethra and finally reconstruction of the orthotopic urethra with a buccal mucosa free graft. Our literature review highlights the difficulties in choosing a fitting individualized approach to achieve the best possible outcome while avoiding unnecessary procedures.

Congenital urethral fistula with a scrotal cyst in a seven-month-old male

Urethral duplication is a rare anomaly with a spectrum of anatomic types. The etiology is unknown and embriology needs to be more elucidated. It affects mainly boys and clinical presentation varies according to anatomic forms. We report an unusual Y-type urethral duplication with normal orthotopic urethra that presented with a scrotal cyst in a 7-month old boy. Our patient underwent only excision of the hypoplastic and non-functioning urethra with good functional and cosmetic result. In conclusion we suggest that on evaluating a patient with a scrotal cyst a Y-type urethral duplication should be suspected and a radiographic study must be done to recognize the normal urethra and choose the best surgical treatment.

Duplication of the urethra in an adult male presenting with scrotal fistula: a rare case report

Urethral duplication is a rare congenital abnormality with varied clinical manifestations; to this day, <300 cases were reported in the literature. It is affecting mainly males and is nearly always diagnosed in childhood or adolescence. It may be complete or incomplete, and the most widely accepted classification of the different types of urethral duplication was developed by Effman et al. Herein, we present a rare case of urethral duplication revealed by scrotal fistula in an adult man. A duplication is an unusual form of Y-type duplication: it is an incomplete urethral duplication urethra opening on the scrotum without communication with the urethra or bladder.

V03-08 TRUE DIPHALLIA: EVALUATION AND SURGICAL CORRECTION OF A RARE CASE OF COMPLETE DUPLICATED PENIS

You have accessJournal of UrologyPediatrics (V03)1 Sep 2021V03-08 TRUE DIPHALLIA: EVALUATION AND SURGICAL CORRECTION OF A RARE CASE OF COMPLETE DUPLICATED PENIS Amr A. Elbakry, Chad Crigger, and Osama Al-Omar Amr A. ElbakryAmr A. Elbakry More articles by this author , Chad CriggerChad Crigger More articles by this author , and Osama Al-OmarOsama Al-Omar More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000001991.08AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: We are presenting a rare case of true diphallia. In this video, we will present our evaluation and demonstrate the surgical technique for management of a patient with complete duplicated penis. METHODS: Our patient is an 11-month-old male who presented initially as 28-day-old that was referred for evaluation of abnormal male genitalia. Physical examination showed duplicated penis with penoscrotal transposition and large scrotal lipoma. VCUG was done and showed Y-shaped duplicated urethra, with normal right urethra and atretic narrow left urethra. Diagnosis was confirmed with US and MRI, that showed complete duplication of the penis with separate two corpora cavernous and a corpus spongiosum and urethra in each penis. We decided to proceed with surgical correction with amputation of the left penis as right penis has the normal urethra evidenced by the VCUG. We started our procedure with performing a cystourethroscopy that confirmed normal right urethra that in continuation with prostatic urethra and bladder neck. Next, bilateral sub-coronal circumferential incisions were made, and complete deglovig of both penises was completed. The lipoma of the scrotum was dissected excised. The left penis was amputated, and the stump was oversewn. Next, we turned our attention to correction of pent-scrotal transposition. The two hemiscrotums were mobilized at the level of the peso-scrotal junction, paying attention to maintain penile skin attached at peno-pubic junction to maintain blood supply. The scrotal flaps were brought ventrally and approximated in the midline in 3 layers. penile skin was trimmed and redistributed for penile coverage. penile skin was approximated ventrally in the midline and was sutured to the foreskin of the left penis. A catheter was left place and dressing was applied. RESULTS: Our procedure was successful with total operative time of 4 hours, minimal blood loss and the patient was discharged home on the same day. Urethral catheter was removed at POD7. There was no reported Complications, no fistula, no wound dehiscence or discharge. Pathology confirmed the diagnosis of accessory penis with normal left and right corpora cavernosum and atretic urethra. CONCLUSIONS: We showed a case of a complete duplicated penis which is a rare congenital anomaly of the genitalia. We demonstrated a surgical technique for correction and reconstruction of the male genitalia in this rare case. Source of Funding: None © 2021 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 206Issue Supplement 3September 2021Page: e224-e224 Advertisement Copyright & Permissions© 2021 by American Urological Association Education and Research, Inc.MetricsAuthor Information Amr A. Elbakry More articles by this author Chad Crigger More articles by this author Osama Al-Omar More articles by this author Expand All Advertisement Loading ...

Surgical Correction of True Diphallia in a Newborn Male

Diphallia is an exceedingly rare anomaly characterized by partial or complete duplication of the phallus. Approximately 100 cases have been reported worldwide since its initial documentation, and incidence is estimated at 1 in 5 to 6 million live births. Therapeutic management is dependent on the extent of the anomaly, ranging from phallic excision to complex reconstructive procedures in cases of broader systemic involvement. We present the case of congenital true diphallia with associated penoscrotal transposition, bifid scrotum, partial urethral duplication, ventral chordee, large scrotal lipoma and sacral dimple. We further present a review of available literature pertaining to diphallia.

URETHRAL DUPLICATION A RARE CAUSE OF EVEN RARER DORSAL CHORDEE

Chordee is a condition in which the penile head has a curvature. It can be towards ventral or dorsal aspect. Dorsal chordee is very rare. In many normal males mild degrees of curvature can exist. Causes of dorsal chordee can be diverse but urethral duplication as its etiology is even rarer. We report a case of six year old boy who presented with severe dorsal chordee with associated double urethra and cleft of the glans dorsally without hypospadia.

V45 - Urethral duplication as cause of incontinence in a 5-year-old female: Treatment of a rare congenital condition

V45 - Urethral duplication as cause of incontinence in a 5-year-old female: Treatment of a rare congenital condition

Findings in physical examination of the external genitalia in pediatric age − different is not always pathological - Part II (female)

Introduction: Findings in the physical examination of the external genitalia in children are often a source of concern for parents and caregivers, not only for the emotional significance that people unconsciously attribute to these structures (partly due to their reproductive function), but also for the physical and psychological impact in the child. Due to the child’s close monitoring and periodic surveillance, the family physician has a key role in the identification and initial guidance of these cases. Objectives: To review the evidence about main variations and anomalies of the external male genitalia in pediatric age regarding diagnosis and clinical approach in primary health care. Results: In most cases, anomalies in the external male genitalia represent variants of normal and/or do not significantly affect function, thus only requiring clinical surveillance and no intervention. However, some cases − as epispadias, hypospadias, and urethral duplication − require surgical intervention and early action is crucial for the success of implemented measures. Conclusion: Physical examination is a key aspect of child assessment. Although changes in physical examination of the external male genitalia mostly represent normality variants, clinicians should be able to recognize pathological changes and properly refer those cases without overloading health services or causing unnecessary anxiety to children and caregivers.

Findings in physical examination of the external genitalia in pediatric age − different is not always pathological − Part I (male)

Introduction: Findings in the physical examination of the external genitalia in children are often a source of concern for parents and caregivers, not only for the emotional significance that people unconsciously attribute to these structures (partly due to their reproductive function), but also for the physical and psychological impact in the child. Due to the child’s close monitoring and periodic surveillance, the family physician has a key role in the identification and initial guidance of these cases. Objectives: To review the evidence about main variations and anomalies of the external male genitalia in pediatric age regarding diagnosis and clinical approach in primary health care. Results: In most cases, anomalies in the external male genitalia represent variants of normal and/or do not significantly affect function, thus only requiring clinical surveillance and no intervention. However, some cases − as epispadias, hypospadias, and urethral duplication − require surgical intervention and early action is crucial for the success of implemented measures. Conclusion: Physical examination is a key aspect of child assessment. Although changes in physical examination of the external male genitalia mostly represent normality variants, clinicians should be able to recognize pathological changes and properly refer those cases without overloading health services or causing unnecessary anxiety to children and caregivers.