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Tarryn Gabler, Robyn Charlton, Jerome Loveland, Ellen Mapunda
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...
April 20, 2018: Pediatric Surgery International
Venkatachalam Raveenthiran
Sex assignment in 46XY genetic male children with congenital inadequacy of the penis (CIP) is controversial. Traditionally, children with penile length less than 2 cm at birth are considered unsuitable to be raised as males. They are typically re-assigned to female-sex and feminizing genitoplasty is usually done in infancy. However, the concept of cerebral androgen imprinting has caused paradigm shift in the philosophy of sex re-assignment. Masculinization of the brain, rather than length of the penis, is the modern criterion of sex re-assignment in CIP...
September 2017: Indian Journal of Pediatrics
A Macedo, M I S Silva, J A Pompermaier, S L Ottoni, R de Castro, M Leal da Cruz
INTRODUCTION: Severe genital abnormalities such as urogenital sinus and urethral duplication with ectopic urethra in the rectum represent a major challenge in reconstructive urology. OBJECTIVE: We aimed to review our cases presenting with functional ectopic urethra implanted in the rectum that were treated through an ASTRA approach. METHODS: We reviewed the medical records of all patients who had undergone an ASTRA approach from 2005-2016...
December 2017: Journal of Pediatric Urology
Rajeev Chaudhry, Katherine M Theisen, Pankaj P Dangle, Francis X Schneck
Phallic reconstruction is an important part of management for congenital aphallia. Scrotal flap phalloplasty has been described in pediatric patients to create the appearance of a nonfunctioning phallus. We describe a modified scrotal flap technique with the addition of an acellular dermal matrix patch (AlloDerm) to provide additional girth and support to the phallus. The postoperative cosmetic outcome has been satisfactory and there is no documented complication over a 12-month follow-up. AlloDerm dermal matrix can be a safe addition to phallic reconstruction and its overall application needs to be further studied...
April 12, 2017: Urology
Ariella A Friedman, Paul F Zelkovic, Edward F Reda, Israel Franco, Lane S Palmer
INTRODUCTION: Aphallia is exceedingly rare (1/30 million births). Previous reports have provided limited detail on associated urinary tract findings. OBJECTIVE: We reviewed urinary tract anomalies in two boys with aphallia (patients 1 and 2) and a girl with urinary tract dysplasia, a similar external appearance and lack of corporal tissue (patient 3), also consistent with aphallia. CASE REPORTS (FIGURE): Patients 1 and 2 both had a 46XY karyotype, bilateral descended testes in well-formed scrotums, and posterior skin tags containing rudimentary urethras...
August 2016: Journal of Pediatric Urology
Fereshteh Talebpour Amiri, Davood Nasiry Zarrin Ghabaee, Ramezan Ali Naeimi, Seyed Javad Seyedi, Seyed Abdollah Mousavi
BACKGROUND: Aphallia or penile agenesis is a rare malformation accompanying with no phallus. This anomaly is extremely rare with abnormality of urogenital system and psychological consequences. Its outbreak is estimated 1 out of 10-30 million births. CASE: Reviewing 3 cases of male external genitalia agenesis, which associated with multiple anomalies of musculoskeletal, cardiovascular and genitourinary system. CONCLUSION: Aphallia has psychosocial consequences and a guarded prognosis...
April 2016: International Journal of Reproductive Biomedicine (Yazd, Iran)
Pierre D E Mouriquand, Daniela Brindusa Gorduza, Claire-Lise Gay, Heino F L Meyer-Bahlburg, Linda Baker, Laurence S Baskin, Claire Bouvattier, Luis H Braga, Anthony C Caldamone, Lise Duranteau, Alaa El Ghoneimi, Terry W Hensle, Piet Hoebeke, Martin Kaefer, Nicolas Kalfa, Thomas F Kolon, Gianantonio Manzoni, Pierre-Yves Mure, Agneta Nordenskjöld, J L Pippi Salle, Dix Phillip Poppas, Philip G Ransley, Richard C Rink, Romao Rodrigo, Léon Sann, Justine Schober, Hisham Sibai, Amy Wisniewski, Katja P Wolffenbuttel, Peter Lee
Ten years after the consensus meeting on disorders of sex development (DSD), genital surgery continues to raise questions and criticisms concerning its indications, its technical aspects, timing and evaluation. This standpoint details each distinct situation and its possible management in 5 main groups of DSD patients with atypical genitalia: the 46,XX DSD group (congenital adrenal hyperplasia); the heterogeneous 46,XY DSD group (gonadal dysgenesis, disorders of steroidogenesis, target tissues impairments …); gonosomic mosaicisms (45,X/46,XY patients); ovo-testicular DSD; and "non-hormonal/non chromosomal" DSD...
June 2016: Journal of Pediatric Urology
Diego Estevam Gomes Oliveira, Marcela Leal da Cruz, Riberto Liguori, Gilmar Garrone, Bruno Leslie, Sérgio Leite Ottoni, Geórgia Rubiane Souza, Valdemar Ortiz, Roberto de Castro, Antonio Macedo
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...
February 2016: Journal of Pediatric Urology
W G Reiner
No abstract text is available yet for this article.
February 2016: Journal of Pediatric Urology
Deepak Sharma, Ravinder Singh, Sweta Shastri
Aphallia (absence of penis) is an extremely rare abnormality which has rarely been described in medical literature and can be part of the urorectal septum malformation sequence (URSMS). URSMS has hardly been reported in medical literature and includes the absence of perineal and anal openings in association with ambiguous genitalia and urogenital, colonic, and lumbosacral anomalies. This case report tells the importance of detailed examination of infants that are diagnosed with aphallia. We report a case of a newborn who was diagnosed as aphallia with the URSMS syndrome after birth...
2015: International Medical Case Reports Journal
Zafer Demirer, Bilal Firat Aip, Sami Uguz, Ali Guragac, Hasan Cem Irkilata
The penis as a component of external genitalia, takes part in fertility, urinary and psychosexual structure of males with its complex character. We report a case of penis agenesis with associated left renal agenesis, left superior segment ureteral agenesis, prostate agenesis, left ureterocele, right vesicoureteral reflux and high urethrorectal communication above the rectal sphincter. The patient refused any surgical intervention because of his religious beliefs.
2015: International Journal of Surgery Case Reports
Antonio Macedo, Marcela Leal da Cruz, Gustavo Marconi Caetano Martins, João Luiz Gomes Parizi, Diego Estevam Oliveira, Valdemar Ortiz
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...
October 2015: Journal of Pediatric Urology
Jhonson Joaquim Gouvea, Gilmar Garrone, Marcela Leal da Cruz, Gustavo Marconi Caetano Martins, João Luiz Gomes Parizi, Diego Estevam Oliveira, Valdemar Ortiz, Antonio Macedo
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...
October 2015: Journal of Pediatric Urology
Aditya Joshi, Jody Gross, James Vincent Thomalla
Congenital aphallia is an extremely rare disorder with <100 cases reported globally. We report on an 11-month-old infant with aphallia and associated syrinx formation. We also review the pathogenesis, associated malformations, and current treatment guidelines for penile agenesis.
August 2015: Urology
Saeid Aslanabadi, Sina Zarrintan, Habib Abdollahi, Reza Rikhtegar, Samad Beheshtirouy, Davoud Badebarin, Mohamed A Baky Fahmy
Aphallia or penile agenesis is an extremely rare congenital anomaly with an estimated incidence rate of 1 in 10 to 30 million births. We report a rare case of aphallia with right kidney hypoplasia and left kidney dysplasia in a 10-day old Iranian-Azeri male. The patient had creatinine rise and renal failure due to dysplastic left kidney and hypoplastic right kidney and expired on fifth day of admission. There were only six cases of renal malformation associated with aphallia in the literature review. Three of the cases were complicated by Potter sequence and one of them was accompanied by chronic renal failure...
April 2015: Archives of Iranian Medicine
Priya Bahe, Deepak Sharma, Anupam Bahe, Aakash Pandita
Complete penile agenesis (aphallia) is a very rare congenital anomaly. Around 80 cases have been reported to date. Diagnosis of this rare anomaly is made by clinical examination, but treatment options and parental counselling for gender assignment is difficult and challenging for the treating doctor. We report a case of an infant with complete penile agenesis with recto-urethral fistula, who was referred on day 6 of life. This baby developed obstructive uropathy which required vesicostomy. Surgical management consists of gender assignment after discussing in detail the pertinent situation with the parents and accepting their final decision...
January 2016: Tropical Doctor
Anju Goyal, Adrian Bianchi
Aphallia is best managed by early penile and urethral reconstruction. We describe parascrotal flap phallo-urethroplasty, with a 6-year follow-up and a psychological profile, that constructs a 'penis' of realistic shape, colour, and position, and having skin sensation, with an integral urethra that allows a free concentrated urinary stream and satisfies the psychological needs of family and child during childhood. The lack of corpora cavernosa only becomes significant at puberty.
August 2014: Journal of Pediatric Urology
Minu Bajpai
AIM: To describe a technique of phalloplasty that is devoid of donor site scarring and suitable for urethral inlay and penile prosthesis in subsequent stages in cases of aphallia. MATERIALS AND METHODS: Four patients with various disorders of sex development with 46 XY and severe penile deficiency, including one with complete androgen insensitivity syndrome who was initially raised as female, have been operated using a "Bird Wing" lower abdominal skin crease incision...
April 2013: Journal of Indian Association of Pediatric Surgeons
Vishwajeet Singh, Deepak Sharanappa Nagathan, Rahul Janak Sinha, Dheeraj Kumar Gupta
An 18-year-old man born with aphallia had undergone phallic reconstruction in childhood followed by total urethral reconstruction with a buccal mucosal graft and groin-based pedicle (Singapore) flap urethroplasty at 13 years of age. The patient presented with obstructive voiding symptoms lasting 6 months followed by acute urinary retention. The results of a voiding cystourethrogram showed a filling defect in the distal urethra that had been reconstructed by use of the skin flap. On urethroscopy, a 3 cm×2 cm sized tricholithobezoar was seen in the distal urethra...
May 2013: Korean Journal of Urology
J A García Andrade, J E Arboleda Bustán, L A Vaca Falconí, M J Flores Núñez, J I Ramírez Rivera
Aphallia had an incidence of 1/30.000.000 newborn. This is a rare genitourinary anomaly derived from a faulty development of the genital tubercles. It usually coexists with series of other anomalies which are incompatible with normal life. This article presents a description of a 2 years old patient.
July 2012: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
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