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Rare Congenital Genitourinary Anomalies
... 4,5 Genital abnormalities have been described in 4% of cases of popliteal pterygium syndrome, namely cryptorchidism, bifid scrotum, hypoplastic or absence of scrotum, and hypoplastic or aplastic labia majora. 6 In addition, inguinal hernias are occasionally present. The most common genital anomaly in boys is bifid scrotum and hypoplastic or aplastic labia majora in girls. ...
... The most common genital anomaly in boys is bifid scrotum and hypoplastic or aplastic labia majora in girls. 6 In severe cases, genital abnormalities may result in fertility disturbances. ...
Introduction:
Popliteal pterygium syndrome (PPS) is a rare autosomal-dominant condition that causes fixed flexion deformity of the knee. The popliteal webbing and shortening of the surrounding soft tissue could limit the functionality of the affected limb unless it is surgically corrected. We reported a case of PPS in a pediatric patient encountered in our hospital.
Case:
A 10-month-old boy came with a congenital abnormally flexed left knee with bilateral undescended testis and syndactyly of the left foot. The left popliteal pterygium extending from the buttock to the calcaneus was observed, with an associated fixed flexion contracture of the knee and equine position of the ankle. Normal vascular anatomy was seen in the angiographic CT scan; therefore, multiple Z-plasty and fibrotic band excision were performed. The sciatic trunk was exposed on the popliteal level, and the fascicular segment was excised from the distal stump and sutured to the proximal stump under the microscope to extend the sciatic nerve for approximately 7 cm. No postoperative complications were reported. Multiple tendons and soft tissue reconstruction were performed when the patient was 2-year-old to correct the adductus and equine deformity of the left foot.
Discussion:
Surgical correction for popliteal pterygium demands staged techniques to deal with the shortened structure. In our case, multiple Z-plasty were performed, and the fibrotic band was excised until its base with meticulous consideration of the underlying neurovascular bundle. Fascicular shifting technique for sciatic nerve lengthening can be considered in unilateral popliteal pterygium with difficulty extending the knee due to shortened sciatic nerve. The unfavorable outcome of nerve conduction disturbance resulting from the procedure may be multifactorial. Still, the existing foot deformity, including a certain degree of pes equinovarus could be treated by multiple soft tissue reconstructions and adequate rehabilitation to achieve the desired outcome.
Conclusion:
Multiple soft tissue procedures resulted in acceptable functional outcomes. However, the nerve grafting procedure is still a challenging task. Further study is required to explore the technique in optimizing the nerve grafting procedure for popliteal pterygium.
... In this review, four patients were included in the post sphincteric (patient 1, 2, 3, 5) and one patient (patient 4) pre sphincteric category as classified by Skoog and Belman (1989) The anus is open and normal. Previous literature reported that there was not a single aphallia with absent urethral meatus and imperforate anus could be survived (Fahmy, 2016). ...
... Androgen receptors were examined to rule out Androgen Insensitivity Syndrome (AIS) (Mendoza and Motos, 2013) Embryologically, sex development goes through two stages, namely sex determination and sex differentiation. The determination stage is when the fetus is still in a bipotential gonadal condition and the differentiation stage, occurs under the influence of factors produced by gonads (Sekido and Lovell-Badge, 2009;Fahmy, 2016. Sexual differentiation begins during the third month of intrauterine life when the external genitalia is established in the ectodermal cloacal fossa (Fig. 1). ...
... In humans, the penile and clitoral prepuces are undifferentiated in early fetal development, emerging from an ambisexual genital tubercle that is capable either of penile or clitoral development regardless of genotype (Baskin et al., 2018). Even at birth --and thereafter --the clitoral and penile prepuces may remain effectively indistinguishable in people with certain intersex traits or differences of sex development (Hodson et al., 2019;Fahmy, 2015;Pippi Salle et al., 2007). The prepuce is an integrated feature of the external genitalia, having evolved to function in concert with other genital structures; for example, it forms the anatomical covering of the glans penis or clitoris, thereby internalizing each and 'decreasing external irritation and contamination' (Cold & Taylor, 1999, p. 34). ...
... This striking comparison -between female and male so-called 'circumcision' within this Muslim community -forms a backbone of Shweder's analysis (see Table 1 for further comparison). (Baskin et al., 2018;Bossio et al., 2016;Cold & Taylor, 1999;Fahmy, 2015Fahmy, , 2020Hodson et al., 2019;Kigozi et al., 2009;Myers & Earp, 2020;O'Connell et al., 2008;Pippi Salle et al., 2007;Purpura et al., 2018;Sorrells et al., 2007;Werker et al., 1998) (Adler, 2012;Askola, 2011;Boyle et al., 2000;Brigman, 1984;Davis, 2001;Earp, 2020a;Geisheker, 2013;Mason, 2001;Merkel & Putzke, 2013;Price, 1997;Somerville, 2004;Svoboda et al., 2016Svoboda et al., , 2019 If non-consensual, medically unnecessary FC is legally considered to be physical assault and battery (which parents are not entitled to authorize for their children) (see, e.g., United States of America vs. Jumana Nagarwala et al., 2018). ...
Defenders of male circumcision increasingly argue that female ‘circumcision’ (cutting of the clitoral hood or labia) should be legally allowed in Western liberal democracies even when non-consensual. In his target article, Richard Shweder (2022) gives perhaps the most persuasive articulation of this argument to have so far appeared in the literature. In my own work, I argue that no person should be subjected to medically unnecessary genital cutting of any kind without their own informed consent, regardless of the sex characteristics with which they were born or the religious or cultural background of their parents. Professor Shweder and I agree that Western law and policy on child genital cutting is currently beset with cultural, religious and sex-based double standards. We disagree about what should be done about this. In this commentary, I argue that ‘legalising’ childhood FGC so as to bring it into line with current treatment of childhood MGC is not an acceptable solution to these problems. Instead, all medically unnecessary genital cutting of non-consenting persons should be opposed on moral and legal grounds and discouraged by all appropriate means.
... Hasta el 50% de los pacientes con quiste de Gartner y displasia renal también puede presentar obstrucción ipsilateral del conducto de Müller. (7,8,14,15) PRESENTACIÓN DEL CASO Paciente femenina de 44 años de edad, con antecedentes obstétricos de 3 embarazos, 2 partos y un aborto 1 espontáneo. Acudió a la institución hospitalaria por presentar, desde hacía alrededor de dos años, dolor en hemiabdomen inferior, más hacia la fosa ilíaca izquierda y al contacto sexual; se aliviaba con analgésicos y se asociaba a hiperpolimenorrea. ...
... Difiere de este reporte, la ausencia de síntomas a pesar de su tamaño. (1,15) Molina Escudero R, et al. (7) muestran una serie de cuatro reportes con quistes de Gartner, todos de localización vaginal: 2 de las pacientes lo tenían en la pared lateral izquierda, una tercera en la pared anterior y la cuarta en la pared lateral derecha. Carrera Gutiérrez J, et al. (8) presentan un caso con un quiste de Gärtner gigante, en la cara posterosuperior de la vagina de aproximadamente de 10 cm de diámetro; asociado a útero polimiomatoso. ...
ABSTRAC Gartner’s duct cyst, mostly little, benign and asymptomatic, are vestiges of the Wolffian mesonephric duct representing 11 % of the vaginal cysts; this location is the most frequently reported and published one up to date. The authors presented the case of a patient who underwent a surgery in the Military Hospital “Dr. Mario Muñoz Monroy¨ with a cyst in the posterior side of the uterine isthmus.
... Over the last few decades, due to the rise of public interest in male infertility, during inguinal surgery surgeons payed more attention to epididymal abnormalities [1,2]. As reported by many authors, the incidence of epididymal abnormalities in boys with undescended testis [3,4] and the significance of testicular location are the point of interest: the higher the arrest of testicular descent, the more grossly abnormal is the associated ductal system [5]. Regarding the incidence of abnomalities, a comparison between the group of patients with undescended testis and group having widely patent processus vaginalis, has been drawn [3,5]. ...
... Embryologically, the testis and epididymis have two origins: gonads and the upper part of epididymis derive from the genital ridge, but distal part of the epididymis and vas deferens from the mesonephric duct [4,6]. The point of union is important for the continuity of ductal system. ...
Introduction. Congenital anomalies of the epididymis, detected either during orchiopexy or routine herniectomy, as well as their importance in male fertility, have been described in many papers in clinical surgery. Clinical Considerations. The aim of this study was to report various types of epididymal congenital abnormalities registered in the clinical practice of pediatric surgery, to describe their basic characteristics and to classify them into three groups: (I) fusional anomalies of the epididymis and testis; (II) anatomic forms of anomalies; (III) congenital epididymal cysts. Conclusion. The clinical experience of pediatric surgeons indicates that during these surgical procedures, it is necessary to carefully examine the epididymis to detect anomalies, and it is of utmost importance to inform the patient and/or his parents. It is also recommended to follow the patients up to the age of fertility.
... Diphallia is a rare congenital anomaly, which means two structurally and anatomically separate phalluses, discovered in 1609 by a Swiss doctor Johannes Jacob Wecker who found this condition while working on a cadaver [7]. Diphallia is most often associated with other congenital anomalies like hypospadias, ectopic scrotum, duplication of bladder, imperforate anus, duplication of colon, exstrophy of cloaca or bladder, ventral hernia and vertebral malformations [8]. ...
Introduction and importance:
Diphallia is extremely rare urological anomaly with reported incidence of 1 in 5-6 million live births. It can present as complete or incomplete diphallia. In most of the cases it is associated with complex urological, gastrointestinal or anorectal malformations.
Case presentation:
We report here case of a newborn who was presented to us at 1st day of life with diphallia and anorectal malformation. He had true diphallia with two separate uretheral orifices. Both phalluses were uncircumcised, phallus 1 was 2.5 cm in length while phallus 2 was 1.5 cm. Both phalluses had normal shaped glans with uretheral opening located at the normal place He had a single scrotum with two midline raphe and well-formed rouge. He was passing urine from both orifices. His ultrasonography of urological system showed two ureters and a single hemi bladder. He was admitted and operated upon and a sigmoid divided colostomy was constructed. Per-operatively congenital pouch colon (type 4) was identified. His post-operative recovery was uneventful. The patient was discharged on second post-operative day and called for follow up.
Clinical discussion:
Diphallia is a rare congenital anomaly, which means two structurally and anatomically separate phalluses. Complete Duplication in Diphallia presents the kind of Diphallia in which both the phalluses have two corpus cavernosum and only one corpus spongiosum. As diphallia presents with a spectrum of diseases; therefore, it requires a multidisciplinary approach. A case of Diphallia may well present with complex urogenital, gastrointestinal or anorectal malformations. As in our case the patient had Diphallia with anorectal malformation. Hence he was operated upon and a sigmoid colostomy was constructed.
Conclusion:
Diphallia is a very rare congenital anomaly which can occur in association with anorectal malformations. Management of such cases should be individualized depending upon the spectrum of disease.
... In humans, the penile and clitoral prepuces are undifferentiated in early fetal development, emerging from an ambisexual genital tubercle that is capable either of penile or clitoral development regardless of genotype (Baskin et al., 2018). Even at birth-and thereafter-the clitoral and penile prepuces may remain effectively indistinguishable in people with certain intersex traits or differences of sex development (Fahmy, 2015;Hodson et al., 2019;Pippi Salle et al., 2007). The prepuce is an integrated feature of the external genitalia, having evolved to function in concert with other genital structures; for example, it forms the anatomical covering of the glans penis or clitoris, thereby internalizing each and "decreasing external irritation and contamination" (Cold & Taylor, 1999, p. 34). ...
In recent years, there has been a resurgence in debates on the ethics of child genital cutting practices, both female and male, including within a Muslim context. Opponents of female genital cutting sometimes assert that the practice is not mentioned explicitly in the Qur’an as a way of implying that it does not have any religious standing within Islam. However, neither is male genital cutting mentioned explicitly in the Qur’an, and yet most people accept that it is a Muslim religious practice. Both practices, however, are mentioned in secondary sources of Islamic jurisprudence, with disagreement among religious authorities about the status or authenticity of some of these sources. This paper considers the religious status of both female and male genital cutting practices within Islam and employs a philosophical argument based on “peer disagreement” to ask whether either practice is necessary (i.e., religiously required) for a devout Muslim to endorse.
The diagnosis and treatment of male infertility, excluding assisted conception, are limited because of, but not limited to, poor understanding of sperm post‐testicular development and storage. Many may think that sperm dysfunction is only self‐contained in the sperm cell itself as a result of defective spermatogenesis. However, it can also be a consequence of inadequate epididymal maturation following disorders of the epididymis. Improper epididymal functions can disturb semen parameters and sperm DNA integrity, result in high leucocyte concentrations and high numbers of immature germ cells and debris or even cause idiopathic infertility. To date, the data are limited regarding critical markers of sperm maturation and studies that can identify such markers for diagnosis and managing epididymal dysfunction are scarce. Therefore, this article aims to draw attention to recognise a disturbed epididymal environment as a potential cause of male infertility.
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