Aphallia: a review to standardize management.

Congenital aphallia is a rare anomaly with little supporting literature and controversial management. The aim of this review is to assess the most recent literature with a focus on staged management of these cases. We performed a PubMed search of all English literature in the past 10 years using the term aphallia. Twenty-three articles were identified of which six were excluded. A further three papers meeting our criteria were found in the references to papers initially identified. We found that management can be staged in three phases: short, intermediate and long-term. We conclude that optimal short-term management centers on resuscitation and urinary diversion as necessary, intermediate-term management entails urethrorectal fistula division, urethrostomy and neophallus creation and long-term management results in successful neophalloplasty, urethroplasty, prosthetic implant and continued protection of the upper urinary tracts with a Mitrofanoff. All this within a multidisciplinary team ensuring shared decision-making with the patient and their family.

An unanswered question in pediatric urology: the post pubertal persistence of prepubertal congenital penile curvature correction by tunical plication.

OBJECTIVE: The aim of this study is to analyze post pubertal results of pre pubertal tunica albuginea plication with non-absorbable sutures in the correction of CPC. MATERIALS AND METHODS: The files of patients who underwent tunica albuginea plication without incision (dorsal/lateral) were retrospectively reviewed. Patients younger than 13 years of age at the time of operation and older than 14 years of age in November 2015 were included. Patients with a penile curvature of less than 30 degrees & more than 45 degrees and penile/urethral anomalies were excluded. All of the patients underwent surgery followed by circumcision. RESULTS: The mean age of patients at the time of the operation was 9.7 years (range, 6-13 years). The mean degree of ventral penile curvature measured during the operation was 39 degrees while it was 41 degrees in the lateral curvatures. All of the patients were curvature-free at the end of the operation. At the time of the follow-up examination, the mean age was 16.7 years (range, 14-25 years). Six patients had a straight (0-10 degrees) penis during erection and seven patients had recurrent penile curvatures ranging from 30 to 50 degrees. CONCLUSION: Pre pubertal tunica albuginea plication of congenital penile curvature (30-45 degrees) with non-absorbable sutures performed without incision is a minimal invasive method especially when performed during circumcision. However, recurrence might be observed in half of the patients after puberty.

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.

Imperforate anus with rectopenile fistula: a case report and systematic review of the literature.

BACKGROUND: Although anorectal malformations (ARMs) are frequently encountered, rare variants difficult to classify have been reported. METHODS: This study describes a patient with ARM and rectopenile fistula. The literature was reviewed systematically to assess the anatomical characteristics, clinical presentations and operations of this rare type of ARM. RESULTS: Eight patients were reported in the six included articles. In three patients, the fistula extended from the rectum to the anterior urethra without communication with the skin. In one patient, the fistula, located deep in corpus spongiosum, opened to the ventral aspect of the penis without communication with the urethra. In the remaining four patients, the fistula extended from the rectum to the cutaneous orifice in the ventral aspect of penis, with communication or a short common channel with the urethra. CONCLUSIONS: Imperforate anus with fistula extending into the penis is a rare variant of anorectal malformation. Unawareness of this lesion resulted in a delay of correct diagnosis and appropriate management. A thorough examination, including colonourethrography and fistulography, should be performed in all patients with a fistula opening in the ventral aspect of the penis.

Split median raphe: case series and brief literature review.

We describe three cases of split median raphe of the penis (SMR) from our hospital newborn records from 2004 to 2013. One case was associated with median raphe cyst, one with skin hypochromia, and one with a scar-like aspect of the region of interest. SMR is thought to be the result of defective fusion of ectodermal tissue in the urethra and scrotum area or of defective growth of the perineal mesoderm around the urethra during gestation. Although SMR associated with other major penile congenital defects (epispadias, hypospadias, penile torsion, bifid scrotum, chordee) is common, isolated SMR is probably an underdiagnosed (although not rare) malformative condition. Recognizing SMR in a newborn may be of educational value to neonatologists because it leads to the search for and exclusion of the above-mentioned pathologic conditions.

Radial forearm free flap phalloplasty for penile inadequacy in patients with exstrophy.

PURPOSE: Male patients with bladder and cloacal exstrophy are born with demure genitalia and often desire phalloplasty during late adolescence or early adulthood. Radial forearm free flap phalloplasty was used successfully for congenital aphallia in a few small series. We present a series of patients treated with phalloplasty using a radial forearm free flap after the repair of bladder and cloacal exstrophy. MATERIALS AND METHODS: We reviewed the records of 10 patients who underwent radial forearm free flap phalloplasty between 2007 and 2012. Indications for phalloplasty were classic bladder exstrophy in 8 cases and cloacal exstrophy in 2. Nine patients underwent prior urinary diversion and 1 underwent urethroplasty at phalloplasty. In each case we reviewed the details of prior exstrophy repair, flap size, donor and recipient vessels, complications, need for subsequent surgeries and self-reported sensation. RESULTS: The graft survived in all 10 patients. Short-term complications requiring surgical intervention developed in 2 patients. Five patients subsequently underwent placement of a penile prosthesis and 2 devices were removed secondary to erosion. All 10 patients reported protective sensation and erogenous sensation with ability to achieve orgasm at last followup. CONCLUSIONS: Radial forearm free flap phalloplasty results in a sensate, cosmetic neophallus. No patient had long-term complications related to phalloplasty but complications related to penile prostheses continue to be a challenging aspect of phalloplasty. The long-term results of the forearm free flap are encouraging in this series of patients with bladder and cloacal exstrophy who desired phalloplasty.

Superficial tunica albuginea excision, using geometric principles, for the correction of congenital penile curvature.

UNLABELLED: Study Type--Therapy (practise pattern survey) Level of Evidence 3b. What's known on the subject? and What does the study add? Congenital penile deviation has become a relatively frequent finding due to a greater awareness of the problem among patients and physicians. Since the first surgical correction for congenital penile curvature was performed, many modifications have been implemented to overcome the disadvantages of the standard procedure and to improve functional results. Among the possible side effects of the original technique are postoperative erectile dysfunction, the development of painful nodules at the suture sites ('dog ears'), alteration of cutaneous sensibility and significant penile shortening. This study presents a novel approach for the correction of congenital penile curvature. We modified the conventional Nesbit technique by applying superficial tunica albuginea excisions, according to the geometric principles of the Egydio technique. OBJECTIVE: • To report our experience with a new technique for the correction of congenital penile curvature based on geometric principles. PATIENTS AND METHODS: • Between January 2006 and March 2011, 211 men with congenital penile curvature underwent our modified Nesbit technique. • The technique consists of an objectivation of the degree of curvature and distribution of the bending force by multiple, small, superficial, elliptical excisions of the tunica albuginea. RESULTS: • The overall success rate was 99.1%. • A residual curvature of less than 20° was reported in 5% (n = 11) of the cases; none of these patients opted for further surgical correction. • Residual curvature of up to 30° was observed in 0.9% (n = 2); these patients underwent a reoperation. • Acquiring or regaining the ability to perform sexual intercourse brought major relief and high rates of satisfaction and self-esteem. • No recurrence of a ventral curvature occurred. CONCLUSIONS: • Our modified Nesbit technique, consisting of superficial tunica albuginea excision according to the geometric principles of the Egydio technique, leads to rapid and excellent results due to an objectivation of the curvature. • It is a safe and valid alternative for the treatment of congenital ventral or ventro-lateral penile deviation.

Congenital penile drop: defective suspensory ligament versus severe proximal curvature, a case series.

INTRODUCTION: Penile drop is a condition where the erect penis is pointing downward. It can either be congenital or posttraumatic. Congenital cases can be attributed to either a defective penile suspensory ligament (PSL) or severe congenital curvature, with the point of maximum curvature proximal, at the base of the penis, concealed within the pubic fat (proximal penile curvature [PPC]). It is important to diagnose the underlying abnormality and choose the surgical approach accordingly, considering that surgical correction of PSL defects results in shortening that can be avoided in PPC cases. AIM: This work describes a method for discriminating PSL defects from PPC and their surgical management. MAIN OUTCOME MEASURES: Accuracy of preoperative diagnosis and results of surgery in terms of erection angle and penile length. METHODS: This is a retrospective study of the management of 12 male patients with congenital penile drop. Diagnostic measures were the pubic gap sign: palpation of a gap between the pubis and the penis, the straightening sign: patients with PSL defects can correct the angle of erection while contrary to PPC cases, Sexual Health Inventory for Men (SHIM), and intraoperative trial rotation of the corpora cavernosa that will correct PPC cases and point them out. Accordingly, PSL cases were managed by anchoring the base of the penis to the pubis, while PPC cases were managed by corporal rotation. RESULTS: Penile drop was fully corrected with shortening in PSL group and without in the PPC group. The straightening sign, SHIM, and trial rotation were in agreement contrary to the pubic gap sign. CONCLUSION: Discriminating PSL defects from PPC is possible by the combination between "straightening sign" and SHIM results. PSL cases should be managed by anchoring sutures despite the inevitable shortening. PPC cases can be spared the shortening by corporal rotation.

Congenital penile curvature: update and management.

Congenital penile curvature results from disproportionate development of the tunica albuginea of the corporal bodies and is not associated with urethral malformation. Patients usually present after reaching puberty as the curvature becomes more apparent with erections, and severe curvature can make intercourse difficult or impossible, at which point surgical repair is recommended. Excellent outcomes can be expected with surgical intervention. The three most commonly used repair techniques are the original Nesbit procedure, modified Nesbit procedure, and plication. Nesbit and modified Nesbit techniques require that an incision is made in the tunica albuginea while plication techniques utilize plicating sutures without an incision. While Nesbit and modified Nesbit techniques are more complex operations, these generally result in less recurrences and more satisfactory outcomes as opposed to the quicker and simpler plication technique.

Congenital Lymphedema of the Foreskin in a 3-Year-Old Boy: A Case Report and Literature Review.

Congenital lymphedema of the external genitalia is a rare, disfiguring disorder. We describe here a case of a 3-year-old male with primary foreskin lymphedema persisting since birth. A compact, heterogenous swelling of the foreskin's distal third was observed, inhibiting preputial retraction (phimosis). Right lower extremity lymphedema was also observed in this case, while no further abnormalities were found. Surgery was performed, maintaining the foreskin, producing an excellent result with no recurrence at 10-month follow up.

Congenital penile masses.

A 2-month-old boy was referred to our department for evaluation of a double mass on the ventral aspect of the penile shaft. There were no associated signs or symptoms of urologic dysfunction. The patient underwent circumcision with complete resection of the masses under general anesthesia.

Minimally invasive technique for the concealed penis lead to longer penile length.

OBJECTIVES: The aim of this study is to report a simple and minimally invasive surgical technique for congenital concealed penis repair. METHODS: Described technique includes two approaches based on common principle, which could be selected according to the degree of penis buried. Through the minimally invasive incision at the base of penis, the repair is performed combination of tacking penile base to prepubic fascia and anchoring subcutaneous penile skin to base of penile shaft. The outcome of the operation is evaluated by improvement of penile length and satisfaction of penile appearance. RESULTS: A statistically significant difference of the penile length between preoperative and postoperative was 2.6 cm for procedure I, and 2.7 cm for procedure II (P < 0.001). There were 58 parents (93.5%) satisfied with the result. No serious intraoperative or perioperative complications. Edema and swelling on the penis are common, but improves with time. All patients are followed for at least 5 months, no retractions occur in any case. CONCLUSIONS: The described technique is simple and minimally invasive. Excellent cosmetic results are achieved with low complication rate and high parent satisfaction. In our experience, there were no additional procedures required to perform deglove or reorganization of the penile skin.

Current trends in the surgical treatment of congenital penile curvature.

Congenital penile curvature (CPC) is a relatively uncommon condition, characterized by congenital angulation of the erect penis. Surgical correction of CPC is the gold standard therapy with a variety of proposed surgical techniques and modifications. This review summarizes the contemporary literature on the surgical management of CPC, trying to address the current trends, as well as the advantages and disadvantages of available methods. We performed a non-systematic narrative and interpretative literature review until December 2018. We included articles with isolated CPC in human adults. We excluded original research articles regarding the surgical treatment of Peyronie's disease (PD). In total, we identified 34 studies including 2155 patients with CPC that met our inclusion criteria. Nine studies included patients with both CPC and PD, but reported sufficient data regarding the CPC cohort. Excisional corporoplasty and incisionless plication seemed to be the preferred surgical methods (10 and 12 studies, respectively), followed by incisional corporoplasty (5) and grafting (3). Four studies reported results of more than one method. Overall, patients with CPC can expect excellent outcomes with surgical repair and minimal side effects. No definite conclusions can be made regarding which technique is superior. The widely varied outcomes highlight the need for standardized outcomes measures in future research.

Estrogen-induced developmental disorders of the rat penis involve both estrogen receptor (ESR)- and androgen receptor (AR)-mediated pathways.

This study tested the hypothesis that the estrogen receptor (ESR) pathway, androgen receptor (AR) pathway, or both mediate estrogen-induced developmental penile disorders. Rat pups received diethylstilbestrol (DES), with or without the ESR antagonist ICI 182,780 (ICI) or the AR agonist dihydrotestosterone (DHT) or testosterone (T), from Postnatal Days 1 to 6. Testicular T concentration, penile morphology and morphometry, and/or fertility was determined at age 7, 28, or 150 days. DES treatment alone caused 90% reduction in the neonatal intratesticular T surge; this reduction was prevented by ICI coadministration, but not by DHT or T coadministration. Unlike the T surge, coadministration of ICI and coadministration of DHT or T mitigated penile deformities and loss of fertility. Generally, ICI, DHT, or T treatment alone did not alter penile morphology; however, fertility was 20% that of controls in ICI-treated rats vs. 70%-90% in DHT- or T-treated rats. The lower fertility in the rats treated with ICI alone could be due to altered sexual behavior, as these males did not deposit vaginal plugs. In conclusion, observations that both an ESR antagonist and AR agonists prevent penile deformities and infertility suggest that both pathways are involved in estrogen-induced penile disorders. Observations that coadministration of ICI, but not DHT or T, prevents the DES-induced reduction in the neonatal T surge suggest that, although ICI exerts its mitigating effect both at the level of Leydig cells and penile stromal cells, DHT and T do so only at the level of stromal cells.

Modern technique for penile torsion repair.

PURPOSE: Penile torsion is a congenital malformation that results in a rotational defect of the penile shaft. Various techniques have been described for correcting penile torsion, although there is no consensus on the best repair. We describe our experience using a dorsal dartos flap to correct penile torsion. MATERIALS AND METHODS: We retrospectively reviewed the records of all 25 patients who underwent repair of significant penile torsion using a dorsal dartos flap at our institution between 2004 and 2007. A total of 17 repairs were performed in association with chordee repair, 7 with hypospadias and 1 with bilateral inguinal hernias. Patient age at the time of repair ranged from 6 to 19 months (mean 9). Of the patients 15 had torsion of at least 90 degrees, 8 had torsion of 60 to 90 degrees and 2 had torsion that was not recorded in degrees. RESULTS: Mean followup was 4 months (range 1.5 to 19). Of the cases 16 demonstrated complete resolution of penile torsion, 7 had residual torsion less than 10 degrees and 2 had documented improvement that was not reported in degrees. No patient has undergone further repair for torsion. CONCLUSIONS: Penile torsion is a challenging congenital anomaly. The dorsal dartos flap is familiar to pediatric urologists and can be varied for use in repair of penile torsion. The procedure results in successful repair of the torsion, has few complications and can easily be performed concurrently in the setting of other operative repairs. This approach provides excellent short-term results.

[Quiste de prepucio deformante en un recién nacido]. / Deforming foreskin cyst in a newborn.

Penile cysts are uncommon lesions. In general, they are asymptomatic and do not interfere with sexual function. Most of them are present since birth, but usually they are only detectable in adolescence or adulthood. We report a clinical rare case of a newborns of 4 days of birth with a 3.5 cm diameter nodule on the prepuce (dorsal face), which appeared of birth. The cyst was excised by circumcision and a histopathologic study was performed. Histopathologic examination revealed a mucoid cyst by ectopic urethral mucoid of penis skins. No recurrence was observed at a 6 months follow-up after of surgery.

Los quistes del prepucio son lesiones muy raras. En general, son asintomáticos y no tienen repercusión en la función sexual. Muchos de ellos se presentan al nacimiento, pero usualmente suelen ser diagnosticados en la adolescencia y en el adulto. Reportamos el caso clínico de un paciente recién nacido, de 4 días de edad, con una masa de 3.5 cm de diámetro aproximadamente en la piel dorsal del prepucio, que se descubrió desde el momento de su nacimiento. El quiste fue resecado por completo y se realizó circuncisión para fines de estética. El examen histopatológico reveló un quiste mucoide por mucosa uretral ectópica en la piel del prepucio. No se observó recurrencia durante un año de seguimiento de su cirugía.

Median raphe cyst of the penis: a case report and review of the literature.

BACKGROUND: A defect in embryological development or closure of median raphe may lead to formation of cyst(s) anywhere in the midline from glans to anus. These cysts are referred to as median raphe cysts, an uncommonly encountered clinical condition. The cyst is generally solitary, with the penile shaft being the most common location, with average size of around 1 cm. The diagnosis is mostly clinical and confirmed histologically. We report a case of a patient with a rare histological variant of median raphe cyst and provide a focused review on presentation, histopathology, and management. CASE PRESENTATION: A 29-year-old unmarried Nepali man presented to our clinic with an asymptomatic, solitary, soft, translucent, nontender cystic lesion of about 1-cm diameter at the ventral aspect of glans penis, close to the meatus, that had been noticed at the age of 3 and was nonprogressive for the past 15 years. Ultrasonography demonstrated an isoechoic cystic lesion at the tip of the penis, separated from the urethra, and lying entirely within the mucosa without any evidence of solid component, septation, or vascularity. On the basis of clinical and ultrasonographic findings, a diagnosis of median raphe cyst of the penis was made. The cyst was excised with the patient under local anesthesia, and there was no evidence of recurrence in 2 years of follow-up. The histopathological examination with Hematoxylin and eosin staining showed the cyst wall was lined partly by ciliated pseudostratified columnar epithelium and partly by columnar epithelium with apical mucin. CONCLUSIONS: Median raphe cyst is an uncommon, mostly asymptomatic condition in young patients. The cyst may occur anywhere along the midline from glans to anus. The diagnosis is clinical with histological confirmation. Excision is the treatment of choice with minimal chance of recurrence.

Shaeer's corporal rotation for length-preserving correction of penile curvature: modifications and 3-year experience.

INTRODUCTION: Correction of penile curvature by corporal rotation enabled the correction of 90 degrees ventral curvature with neither shortening nor erectile dysfunction. However, some limitations were described, and only one case was reported upon. AIM: This work described a 3-year experience with corporeal rotation, the modifications addressing and eliminating its drawbacks and limitations, as well as the long-term follow-up of 22 patients. METHODS: Modified corporeal rotation was performed in 22 patients with various degrees of curvature. Degree of deviation, erect penile length, symmetry, and erectile function were evaluated and compared pre- and postoperatively. MAIN OUTCOME MEASURES: Correction of curvature, resultant sexual function, penile length, and girth. RESULTS: Full correction of curvature was achieved in 20 out of 22 patients, with no shortening, asymmetry, or erectile dysfunction. Residual curvature in two patients was no more than 10 degrees. CONCLUSIONS: Corporal rotation can restore straightness to the penis with no loss in phallic length, asymmetry, or erectile dysfunction. While a variety of surgical techniques are feasible for the correction of milder degrees of curvature, we believe that severe degrees should be spared the shortening and corrected by corporeal rotation.