A New Surgical Technique for the Treatment of Congenital Concealed Penis Based on Anatomical Finding.

PURPOSE: Surgery is the first line treatment for congenital concealed penis but penile retraction is inevitable in some cases. We investigate the anatomical and histological characteristics of penile fasciae and describe a new technique for the correction of concealed penis. MATERIALS AND METHODS: The anatomical structures of penile fasciae were observed in 10 adult cadaveric penises. Penile tissue samples were stained with hematoxylin-eosin, Masson's trichrome and Weigert's resorcin-fuchsin, respectively. From January 2017 to May 2019, 78 patients with congenital concealed penis were treated with the new surgical technique. Median patient age was 14 years (range 8 to 18). RESULTS: Dartos fascia had sublayers. The superficial layer was a well vascularized tissue composed of nonpolar collagen fibers intermixed with nerves and vessels. The deep layer was composed of a transverse arrangement of collagen fibers and elastic fibers, and there were fewer venules and nerve fibers. Based on this finding we performed anatomical resection of the deep layer of dartos fascia to correct concealed penis. During the operation dartos fascia was separated into 2 layers and a complete circular resection of the deep layer was made at the base of the penis. Mean followup was 14 months. All patients and their parents were satisfied with the outcomes. None of the patients underwent postoperative penile retraction. CONCLUSIONS: The anatomical resection of the deep layer of dartos fascia for correcting concealed penis is technically easy, safe and effective. It provides a good cosmetic appearance and functional outcomes.

A Rare Case of Partial Unilateral Corpus Cavernosum Agenesis With Concomitant Multiple Genitourinary Anomalies.

Congenital penile agenesis is a rare condition with an incidence of 1 in 30 million, while other congenital malformations of the cavernous bodies are much less common. In a few cases in the literature, it has been reported that the reason for consulting a physician with these conditions is the insufficient erection. As a first reported case, we present a 16-year-old male patient with partial unilateral corpus cavernosum agenesis accompanying genitourinary anomalies.

Agenesia renal y orquiepididimitis: ¿en qué debemos pensar? / Renal agenesis and orchiepididymitis: what should we think about?

El síndrome de Zinner es una alteración en el desarrollo embriológico poco común, que consiste en la asociación de una dilatación quística de la vesícula seminal con agenesia renal ipsilateral. Presentamos el caso de un varón de 14 años diagnosticado de síndrome de Zinner a raíz de un cuadro de orquiepididimitis izquierda y hematuria. Entre los antecedentes personales del paciente destaca una agenesia renal izquierda con función renal conservada. Debido a la sospecha clínica se realizó una resonancia magnética (RM), en la que se objetivó uréter izquierdo dilatado y tortuoso, confirmándose su desembocadura en vesícula seminal izquierda. Clínicamente suele manifestarse con episodios recidivantes de epididimitis, trastornos miccionales o alteraciones eyaculatorias, pudiendo aparecer a cualquier edad, aunque lo más frecuente es durante la segunda y tercera décadas de la vida. La RM es la técnica diagnóstica de elección. El tratamiento depende directamente de la clínica. La cirugía suele reservarse para los pacientes sintomáticos o para aquellos quistes que se detectan a una edad temprana, para evitar posibles complicaciones

Zinner syndrome is a rare embryological development disorder. It is the association of a cystic dilation of the seminal vesicle with ipsilateral renal agenesis. The case is presented as a 14-year-old male diagnosed with Zinner syndrome as a consequence of an orchiepididymitis and hematuria episode. Among his medical history, a left renal agenesis is highlighted with preserved renal function. Due to the clinical suspicion, a MRI was performed in which it was observed a dilated and tortuous left ureter, being able to confirm its opening in the left seminal vesicle. Zinner syndrome is clinically presented with recurrent epididymitis episodes, voiding disorders or ejaculatory alterations, being able to appear at any age, although the most common is during the second and third decade of life. MRI is the diagnostic method of choice. Treatment relies on the clinic. Surgery is usually reserved for symptomatic patients or for those cysts that are detected at an early age, in order to avoid possible complications

Prenatal imaging of genital defects: clinical spectrum and predictive factors for severe forms.

OBJECTIVES: To report the clinical spectrum of genital defects diagnosed before birth, identify predictive factors for severe phenotypes at birth, and determine the rate of associated malformations. PATIENTS AND METHODS: A retrospective study (2008-2017) of 4580 fetuses, identified prenatally with abnormalities evaluated by our Reference Center for Fetal Medicine, included cases with fetal sonographic findings of abnormal genitalia or uncertainty of fetal sex determination. Familial, prenatal and postnatal data were collected via a standardised questionnaire. RESULTS: In all, 61 fetuses were included. The positive predictive value (PPV) of the prenatal diagnosis of genital defects was 90.1%. Most cases were 46,XY-undervirilized boys, 42 cases (68.8%), which included 29 with mid-penile or posterior hypospadias, nine with anterior hypospadias, and epispadias, micropenis, scrotal transposition, and buried penis (one each). In all, 46,XX-virilized girls were identified in seven cases (11.5%), which included four with congenital adrenal hyperplasia, two with isolated clitoromegaly, and one with ovotestis. Other defects included prune belly syndrome and persistent cloaca (six cases). Early detection during the second trimester (58.1% vs 18.8%, P = 0.03), intra-uterine growth restriction (IUGR) (45.2% vs 9.1%, P = 0.06), and curvature of the penis (38.7% vs 0%, P = 0.02), were more frequently related to severe defects in male newborns. Associated malformations (14 cases, 22.9%) and genetic defects (six) were frequent in undervirilized boys. CONCLUSION: Prenatal imaging of genital defects leads to a wide range of phenotypes at birth. Its PPV is high and extra-urinary malformations are frequent. Early diagnosis during the second trimester, associated IUGR, and curvature of the genital tubercle, should raise suspicion of a severe phenotype and may justify delivery near a multidisciplinary disorders/differences of sex development team.

[Congenital seminal vesicle cysts in children].

The article reports two cases of seminal vesicle cysts with ipsilateral renal agenesis in children. The congenital nature of this condition, characteristic features of the clinical manifestation of seminal vesicle cysts makes it difficult to recognize this rare genitourinary disease. Using current methods of diagnosis and surgical treatment result in successful outcomes.

Minimally Invasive Approach for Treatment of Seminal Vesicle Cyst Associated with Ipsilateral Renal Agenesis.

OBJECTIVES: We reviewed the clinical presentation and approach for the treatment of symptomatic seminal vesicle cyst associated with ipsilateral renal agenesis while presenting our experience with a series of 5 patients treated with minimally invasive surgery for Zinner syndrome. MATERIALS AND METHODS: Between the years 2008 and 2016, we operated on 5 patients who presented with symptomatic seminal vesicle cyst and ipsilateral renal agenesis. Patients' charts and medical records were reviewed and compared with past published minimally invasive series. RESULTS: Four patients were treated in a laparoscopic approach and one was treated with robotic-assisted seminal vesicle cyst excision. In all cases, the cystic complex was drained and excised with marsupialization of the remaining cyst walls to prevent cystic recurrence. The mean operating time was 3:47 h and mean hospitalization time was 7 days (4-14). The mean follow-up period was 3.2 years (range 1.7-4.8 years). All patients reported a resolution of symptoms during postoperative follow-up. CONCLUSIONS: Seminal vesicle cyst with ipsilateral renal agenesis should be suspected in young male patients presenting with pelvic cystic masses. Treatment is reserved for symptomatic patients and the preferred approach is minimally invasive surgery. This approach is feasible and effective while providing advantages both for the patient and the surgeon.

Median raphe cyst: report of two cases.

Median raphe cysts are rare congenital lesions ofthe male genitalia that occur as a result of alteredembryologic development. We report two such casesof median raphe cysts in the pediatric age group. Inaddition, we review the literature.

Efficacy and Safety of Continuous Subcutaneous Infusion of Recombinant Human Gonadotropins for Congenital Micropenis during Early Infancy
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BACKGROUND: Early postnatal administration of gonadotropins to infants with congenital hypogonadotropic hypogonadism (CHH) can mimic minipuberty, thereby increasing penile growth. We assessed the effects of gonadotropin infusion on stretched penile length (SPL) and hormone levels in infants with congenital micropenis. METHODS: Single-center study including 6 males with micropenis in case of isolated CHH (n = 4), panhypopituitarism (n = 1), and partial androgen insensitivity syndrome (PAIS; n = 1). Patients were evaluated at baseline, monthly and at the end of the study through a clinical examination (SPL, testicular position and size), serum hormone assays (testosterone, luteinizing hormone, follicle-stimulating hormone, inhibin B, anti-Müllerian hormone [AMH]), and ultrasound of penis/testes. RESULTS: In CHH, significant increases occurred in serum testosterone (from undetectable level to 3.5 ± 4.06 ng/mL [12.15 ± 14.09 nmol/L]), SPL (from 13.8 ± 4.5 to 42.6 ± 5 mm; p < 0.0001), inhibin B (from 94.8 ± 74.9 to 469.4 ± 282.5 pg/mL, p = 0.04), and AMH (from 49.6 ± 30.6 to 142 ± 76.5 ng/mL, p = 0.03). Micropenis was corrected in all patients, except one. On treatment, in the patient with PAIS, SPL was increased from 13 to 38 mm. CONCLUSIONS: Early gonadotropin infusion is a safe, well-tolerated and effective treatment. The effect in PAIS has not been reported previously. Long-term follow-up is needed to assess the impact, if any, on future fertility and reproduction.
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Preliminary report on a new surgical technique for the management of bothersome scrotomegaly in selected adolescent males.

Idiopathic scrotomegaly in adolescent boys can be a bothersome and distressing complaint. The challenge lies in accepting its potential impact on the patient's body image, offering a simple solution with minimal morbidity. Herein, we present a novel approach to reduction scrotoplasty, which adds to the limited published strategies available, and provides surgeons with a safe and effective procedure to correct this condition. Our report also calls attention to a potentially under-recognized medical problem in adolescents and young adults.

Laparoscopic removal of a congenital seminal vesicle cyst in Zinner's syndrome.

INTRODUCTION: Seminal vesicle cysts are rarely diagnosed, but symptomatic congenital seminal vesicle cysts are reported in the literature describing Zinner syndrome. CASE DESCRIPTION: We present the case of a 26-year-old patient admitted to the urology department because of abdominal pain. A left seminal vesicle cyst and left kidney agenesis were found on examination, and the patient qualified for laparoscopic removal of the left seminal vesicle cyst. The procedure was performed with transperitoneal access using 5 trocars. The peritoneum was incised between the bladder and the rectum to reveal the left seminal vesicle, which was resected from the surrounding tissue. A TachoSil (Takeda Pharmaceuticals, Zurich, Switzerland) hemostatic sponge was placed in the ledge after cutting the base of the seminal vesicle. DISCUSSION: The aim of this study was to evaluate usefulness of the laparoscopic technique to remove a cystic seminal vesicle. The procedure lasted 180 minutes, and the estimated blood loss was 50 mL. We did not record any intra- or postoperative complications. The patient was discharged from the hospital on the third postoperative day and remains symptom-free after 12 months. Histopathologic examination confirmed the presence of a seminal vesicle cyst, and renal agenesis and stenosis of the vas deferens suggests Zinner syndrome. Symptomatic seminal vesicle cysts are a good indication for the application of a laparoscopic technique and, in our opinion, this is better than the open technique because of the view into the surgical area. Therefore, it should be used as the method of choice in treating seminal vesicle defects.

Congenital hemiscrotal agenesis: report of a rare entity.

Congenital scrotal agenesis is the rarest anomaly of scrotal development disorder and is characterized by the absence of scrotal rugae in the perineum between the penis and anus. We report here a case of hemiscrotal agenesis in a 2-year-old boy. To the best of our knowledge, our patient is the second reported case of hemiscrotal agenesis in the English literature.

[Seminal vesicle cyst associated with ipsilateral renal dysplasia and vena cava malformation : a case report].

We report a rare case of seminal vesicle cyst associated with ipsilateral renal dysplasia and vena cava malformation. A 76-year-old man was hospitalized because of difficulty in urination. We diagnosed benign prostate hyperplasia with vesical diverticulum and administered medication that was found to improve urination. However, positron emission tomography-computed tomography showed a large mass in the pelvic region ; therefore, additional examinations were performed. Urethrocystography showed a filling defect in the bladder. Computed tomography revealed the absence of the right kidney and the presence of a double vena cava and a large seminal vesicle cyst on the same side. Magnetic resonance imaging showed a cystic ectopic ureter associated with the seminal vesicle cyst. Therefore, we diagnosed the patient with a seminal vesicle cyst associated with ipsilateral renal dysplasia and performed cyst puncture. The patient is currently free from urinary symptoms at 12 months after surgery.

Poliorquias. Revisión de la literatura española (1965 - 2012) / No disponible

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Reference values for fetal penile length and width from 22 to 36 gestational weeks.

OBJECTIVE: Current reference range values for fetal penile growth are based on length measurements. However, methodologies for measuring penile length differ among studies and from the standard technique used in children. We propose that the measurement of penile width may aid in its evaluation. The aim of the study was to create normograms for penile length and width. METHODS: A prospective cross-sectional design was used. One hundred male fetuses at 22 to 36 gestational weeks were included. On ultrasound examination, penile length was measured from tip to base, where the penis joins the scrotum. Penile width was measured at the widest point across the penis. Reference values for the 5th and 95th percentiles were calculated for each gestational week. RESULTS: There was a good correlation between gestational age and penile length (R(2) = 0.606) and width (R(2) = 0.683). The percentile of fetal weight estimation independently affected penile length and width. The interobserver correlation coefficient was 0.939 for length and 0.909 for width. CONCLUSION: Reference range values of fetal penile length and width are presented for 22 to 36 weeks of gestation. These values correlate to gestational age and estimated fetal weight percentile. These reference range measurements may help the evaluation of suspected micropenis.

Distancia anogenital en recién nacidos: un marcador sensible de disrupción hormonal intrauterina / Anogenital distance in newborns: A sensitive marker of prenatal hormonal disruption

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Prenatal environmental risk factors for genital malformations in a population of 1442 French male newborns: a nested case-control study.

BACKGROUND: Over the past decades, an increasing trend in male external genital malformations such as cryptorchidism and hypospadias has led to the suspicion that environmental chemicals are detrimental to male fetal sexual development. Several environmental pollutants, including organochlorine pesticides, polychlorinated biphenyls, bisphenol A, phthalates, dioxins and furans have estrogenic and anti-androgenic activity and are thus considered as endocrine-disrupting chemicals (EDCs). Since male sex differentiation is critically dependent on the normal production and action of androgens during fetal life, EDCs may be able to alter normal male sex differentiation. OBJECTIVE: The objective of this study was to determine the incidence of external genital malformations in a population of full-term newborn males in southern France. We also performed a case-control study to identify the risk factors for male external genital malformations, with a focus on parental occupational exposure to EDCs. METHODS: Over a 16-month period, 1615 full-term newborn males with a birth weight above 2500 g were registered on a level-1 maternity ward, and the same pediatrician systematically examined 1442 of them (89%) for cryptorchidism, hypospadias and micropenis. For every male newborn with genital malformation, we enrolled nearly two males matched for age, parity and term. All parents of the case and control newborns were interviewed about pregnancy aspects, personal characteristics, lifestyle and their occupational exposure to EDCs using a detailed questionnaire. RESULTS We report 39 cases of genital malformation (2.70%), with 18 cases of cryptorchidism (1.25%), 14 of hypospadias (0.97%), 5 of micropenis (0.35%) and 2 of 46,XY disorders of sexual differentiation (DSD; 0.14%). We observed a significant relationship between newborn cryptorchidism, hypospadias or micropenis and parental occupational exposure to pesticides [odds ratio (OR) = 4.41; 95% confidence interval (95% CI), 1.21-16.00]. Familial clustering for male external genital malformations (OR = 7.25; 95% CI, 0.70-74.30) and medications taken by mothers during pregnancy (OR = 5.87; 95% CI, 0.93-37.00) were associated with the risk of cryptorchidism, hypospadias and micropenis, although the association was not statistically significant. CONCLUSIONS: Although the causes of male genital malformation are multifactorial, our data support the hypothesis that prenatal contamination by pesticides may be a potential risk factor for newborn male external genital malformation and it should thus be routinely investigated in all undervirilized newborn males.

[Health assessment of newborns with congenital urinary malformation].

Improvement in diagnosis of prenatal congenital urinary malformations (CUM) resulted in increased detection of CUM cases among newborns. To facilitate medical care for CUM newborns, we have developed an objective method of CUM newborns' health assessment. We studied 40 case histories of newborns with prenatally detected CUM admitted to urological clinics (20 with diagnosis of poor health and 20 with moderate condition severity) and 40 CUM newborns examined outpatiently (moderate health hazard). The computer analysis of the available data has established 13 most informative diagnostic criteria: 4 sonographic criteria, 3 lab criteria and 6 physical exam criteria estimated by 4 points - from 0 to 4. The criteria were pooled to a table which was used as a scale to assess general health condition. After delivery, CUM newborn gets inpatient health assessment by a neonatologist using 13 criteria. According to the total score, the condition is assessed as satisfactory (0-5), moderately severe (6-11), severe ( > 12). Efficacy of such health assessment was proved in a population study of 312 prenatal CUM newborns. Grouping of such newborns helps design of programs of further examination and management.