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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
Meenakshi Bothra, Vandana Jain
Aphallia or complete absence of penis is a rare disorder, often associated with other congenital anomalies of the genitourinary and gastrointestinal systems. This disorder is difficult to manage and has a very significant psychosocial impact on the child as well as the family. Individualized assessment and formulation of management plan is important.
2012: Journal of Pediatric Endocrinology & Metabolism: JPEM
Eric Z Massanyi, Angela Gupta, Sameer Goel, John P Gearhart, Arthur L Burnett, Trinity J Bivalacqua, Richard J Redett
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...
October 2013: Journal of Urology
Minu Bajpai
All the currently known reconstructions for aphallia are carried out around puberty. We describe a novel technique as a temporizing procedure which would see the patient through childhood. This would not cause any hindrance while undertaking any of the other established procedures later on after puberty.
October 2012: Journal of Indian Association of Pediatric Surgeons
Rachel Bluebond-Langner, Richard J Redett
The most common indications for phalloplasty in children include aphallia, micropenis/severe penile inadequacy, ambiguous genitalia, phallic inadequacy associated with epispadias/bladder exstrophy and female to male gender reassignment in adolescents. There are many surgical options for phalloplasty; both local pedicled tissue as well as free tissue transfer. The advantages of local tissue include a more concealed donor site, less complex operation and potentially faster recovery. However, pedicled options are generally less sensate, making placement of a penile prosthesis more risky and many children with bladder exstrophy have been previously operated upon making the blood supply for local pedicled flaps less reliable...
August 2011: Seminars in Plastic Surgery
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