Marcela Leal da Cruz

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.

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.

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.

A 4-Year Prospective Urological Assessment of In Utero Myelomeningocele Repair—Does Gestational Age at Birth Have a Role in Later Neurogenic Bladder Pattern?

Purpose: Premature delivery is a major complication of in utero myelomeningocele repair. The prematurity rate in MOMS (Management of Myelomeningocele Study) was 79%, with a mean gestational age at birth of 34 weeks. We speculated that prematurity could also influence urological outcome in patients operated on prenatally for myelomeningocele. Materials and Methods: Beginning in November 2011, we prospectively followed a population of patients with myelomeningocele who had undergone in utero repair. Results: We compared patients based on gestational age at birth, ie younger than 34 weeks (group 1, 42 patients) and 34 weeks or older (group 2, 37 patients). Mean gestational age at birth was 28.3 weeks (range 25 to 33) in group 1 and 35.2 weeks (34 to 38) in group 2. Of the cases 47.5% in group 1 were classified as high risk, 35% as incontinent, 10% as hypocontractile and 7.5% as normal. By comparison, 54.5% of cases in group 2 were classified as high risk, 33.3% as incontinent and 12.1% as normal. Differences between the groups were not statistically significant. Mean followup was 27.9 months in group 1 and 24.3 months in group 2. Conclusions: Our results show that gestational age at birth has little impact on bladder pattern. These data reinforce the need to follow this population closely.

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.

Y-type urethral duplication with rectal implantation of the urethra: Which is the best approach?

INTRODUCTION Y-type urethral duplication describes the condition in which a functional urethra is implanted in the rectum, and there is also a dysplastic topic urethra which produces mostly urinary dribbling. These patients are at risk of urinary tract complications and UTI. We aimed to present the surgical steps of a case treated by ASTRA approach in which we separated the urethra from the rectum and created a perineal urethrostomy. MATERIAL AND METHODS We treated a 5-month-old boy with Y-type (IIA-2) urethral duplication, in whom the orthotopic urethra was patent just in the penile segment. The patient had urinary flow per anus and minimal dribbling through the orthotopic urethra. We performed a combined cystoscopy with retrograde urethrogram and managed to catheterize the dysplastic urethra with a guide-wire that showed ectopic implantation in the prostatic urethra, below the bladder neck. We performed an ASTRA procedure to separate the urethra from the rectum. The urethral stump was further mobilized to the perineum and anastomosed to a perineal skin flap to create a stoma and minimize the risk of stricture. RESULTS The patient was followed at 2 month-intervals, and at 6 months follow-up had an excellent outcome. CONCLUSION The ASTRA approach proved to be an excellent alternative for Y-type urethral duplication with functional urethra implanted in the rectum. We believe that further efforts to reconstruct the urethra should be avoided, with a better and simpler option being to create a definitive perineal urethrostomy.

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.

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.