Neophalloplasty in boys with aphallia: A systematic review.

OBJECTIVE: Aphallia is a rare penile congenital abnormality. The aim of this systematic review was to assess all available literature on neophalloplasty in infancy with an interest in technical alternatives and clinical outcome. MATERIALS AND METHODS: We performed a Pubmed search using the terms: neophalloplasty, neophallus, and phalloplasty, and selected articles that presented technical alternatives for penile construction in cases of penile dysgenesis, especially aphallia in children and/or adolescents. RESULTS: A total of 319 articles were identified in the initial search. Among the different techniques presented in 19 papers collected, we categorized reconstructive procedures into two groups: microsurgical and non-microsurgical techniques. Among the microsurgical techniques, procedures such as the osteocutaneous fibular flap (n = 18), the myocutaneous latissimus dorsi flap (n = 24), and the radial forearm free flap (n = 293) were reported. Among the non-microsurgical techniques, we found a wider range of technical options (6 in total). Most were based on groin, abdominal, and scrotal flaps. Long-term reports on the functionability of the neophallus and psychological aspects of the patients are lacking. CONCLUSION: In assessing recent literature, it seems rational that neophalloplasty should be regarded as the preferred strategy for boys born with aphallia, in opposition to female gender assignment.

Primary congenital bladder diverticula: Where does the ureter drain?

BACKGROUND: Primary congenital bladder diverticulum (PCBD) is related to a deficient detrusor layer allowing out-pouching of the bladder mucosa through the inadequate muscularis wall. We aimed to review our experience with symptomatic PCBD in order to correlate clinical findings with anatomical aspects and to present late outcome. MATERIALS AND METHODS: We reviewed all patients operated in our institution since 2004. We evaluated the charts for complaints, radiological exams, method of treatment, complications and length of follow-up. RESULTS: We treated 10 cases (11 renal units - [RU]), predominantly males (9/10), mean age at surgery of 5.3 years. All patients had significant urological complaints presenting either with antenatal hydronephrosis (4) or febrile urinary tract infection (5) and urinary retention in one. The ureter was found implanted inside the diverticulum in 8/11 RU. An extravesical psoas-hitch ureteroneocystostomy and diverticulum resection was performed in 10/11 cases, whereas 1 case was treated intravesically based on surgeon's preference without performing cystoscopy. Mean follow-up was 34.1 months (1-120) without complications. CONCLUSIONS: PCBD is an uncommon diagnosis and has a high probability of drainage inside the diverticulum (72.7%). We recommend the extravesical approach associated with diverticulectomy and ureteroneocystostomy as the preferred technique to treat this abnormality.

Penile prosthesis implantation in a patient with congenital aphallia treated using the De Castro technique 10 years previously. Is it feasible?

OBJECTIVE: Aphallia is a rare congenital abnormality with an incidence of 1 in 30 million births. In this video, we demonstrate implantation of a penile prosthesis in a neophallus performed 10 years previously in a patient aged 21. METHODS: Through a midline perineal incision aiming to reach the inferior surface of the pubic arch, we created a 16-cm tunnel for prosthesis insertion into the neophallus. We dressed the prosthesis with a polypropylene mesh to give stability to the component and avoid its extrusion. We anchored the lateral mesh to the inferior aspect of the pubic arch with 2.0 vicryl sutures in both sides. RESULTS: The patient had an excellent initial outcome, without any complaints of pain or other inflammatory findings. CONCLUSION: We acknowledge limited clinical experience with this technique. Further psychological evaluation will confirm if patients can have pleasant sexual experiences.

Neophalloplasty to congenital aphallia using two transverse skin-flaps: an alternative approach.

OBJECTIVE: Penile agenesis is a rare congenital abnormality with an incidence of 1 in 30 million births. We want to demonstrate in this video a new technique of neophalloplasty based on two transverse skin flaps from lower abdominal wall. METHODS: The patient was placed into a prone position; the urethral meatus was found inside the rectum, 2 cm from the anal border. An Anterior Sagittal Transrectal Approach (ASTRA) approach was performed; the urethra was isolated and repositioned as a perineal urethrostomy. We created the neophallus based on two flaps, each measuring 7 × 3 cm. After incision, flaps were rotated 90° and sutured to each other creating the neophallus. We also created a Mitrofanoff channel to secure bladder emptying in cases of urethrostomy stricture. RESULTS: The patient had an uneventful immediate postoperative evolution, but later showed some degree of penile retraction. He voids preferably through the urethrostomy and his mother uses the Mitrofanoff once a day. The current follow-up is 18 months. CONCLUSION: We acknowledge limited clinical experience with this technique. However, the same principle has been used to create catheterizable urinary channels, with favorable results and longer follow-up. The use of transverse skin flaps might be an interesting alternative for neophalloplasty in aphallia.

Total urogenital mobilization by CAH: a step-by-step illustration of the technique.

INTRODUCTION: Congenital adrenal hyperplasia (CAH) resulting from deficient 21-hydroxylase activity is an autosomal recessive disorder with an incidence of 1:5000-25,000 in Caucasian populations. Despite various techniques to treat CAH, total urogenital mobilization (TUM) has gained popularity. This technique has low morbidity, and can be performed by the perineal route with the patient in the dorsal lithotomy position without the need to separate the urethra from the vagina. We aim to demonstrate in this video the TUM technique step by step. METHODS: A 9-month-old child, born with ambiguous genitalia (Prader 3) with a 46XX karyotype and CAH diagnosis presented to our facility. Endoscopy showed a 2-cm common channel. RESULTS AND DISCUSSION: The patient was treated as described in the video and she had an uneventful clinical outcome with complete healing. TUM is an excellent alternative for treating CAH.

Complete female epispadia: the case for perineal approach.

INTRODUCTION: Complete female epispadia (CFE) is a rare congenital anomaly occurring in 1 of 500,000 live births. The goals of CFE management include achieving continence, while protecting the kidneys, and creating functional, cosmetic external genitalia. PATIENT AND METHODS: We demonstrate in this video the steps of the surgery and present midterm follow-up of a 6-year-old child with CFE. RESULTS AND DISCUSSION: The patient had an excellent cosmetic result, and reported continence of 3 h period with mild leakage. The perineal infrapubic approach offers the possibility of restoring cosmesis and providing resistance in one surgery precluding the need for abdominal bladder neck surgery.