SURGICAL MANAGEMENT OF FEMALE GENITAL ANOMALIES, DISORDERS OF SEXUAL DEVELOPMENT, UROGENITAL SINUS, AND CLOACAL ANOMALIES

2010 ◽  
pp. 476-499 ◽  
Author(s):  
Elizabeth B. Yerkes ◽  
Richard C. Rink
2015 ◽  
Vol 22 (04) ◽  
pp. 401-407
Author(s):  
Naima Rasool

Objectives: To evaluate the etiology, clinical presentations, investigationsand surgical management of patients presenting with atypical genitalia in Pediatric surgerydepartment. Disorders of Sexual development are one of the most complex congenitaldisorders encountered by the treating physicians. In recent years, the diagnostic ability andsurgical techniques of gender reconstructions have improved remarkably. The spectrumincludes chromosomal, genotype and phenotype abnormalities. The birth of a baby withatypical genitalia poses diagnostic and treatment challenges. This article focuses on etiology,clinical presentations, investigations and surgical management of patients presentingwith ambiguous genitalia. Study Design: A descriptive, prospective case series. Setting:Department of Pediatric Surgery, Military Hospital, Rawalpindi, Pakistan. Period: October 2010to June 2014. Methods: All patients with atypical genitalia, who underwent any kind of genitalreconstruction surgery were included in the study. Results: A total of 28 patients were operatedupon during the study period. Age ranged from 11months to 12 years. Patients were dividedinto two groups on the basis of their karyotyping. Seventeen patients had 46 XY and 11 had 46XX. No mosaic pattern was seen. Male genital reconstructive surgery was done in 16 patientswhile 12 underwent female genital reconstructive surgery. Conclusions: Disorders of sexualdevelopment present as a complex spectrum of psychosocial, sexual, endocrine and surgicalimplications, thus management demands a multidisciplinary team approach. Appropriatelytimed surgical intervention after thorough work up and detailed counseling, produce excellentcosmetic and functional results.


2021 ◽  
pp. 63-68
Author(s):  
A.R. Potsiurko ◽  
◽  
I.Ya. Hrymak ◽  
N.V. Helner ◽  
B.I. Hyzha ◽  
...  

Purpose – to analyze own results of surgical treatment of serious forms of disorders of sexual development (DSD) in children after feminizing surgeries. To define practical protocol guides on the choice of the type of surgical correction. Materials and methods. Over the past 5 years surgeons of Lviv regional paediatric clinical hospital «OHMATDYT» have examined 12 children with DSD, signs of hermaphroditism (intersex). After complex examination the following states were diagnosed: androgenital syndrome in 5 girls (referred for further treatment to Kyiv paediatric medical centres); true hermaphroditism – 1 child; mixed gonadal dysgenesis (MCG) – 3 children; female pseudohermaphroditism (without determining genesis) – 1 child; partial testicular feminization syndrome, Morris syndrome (male pseudohermaphroditism) – 2 children. In connection with marked masculinization of external genitalia two children with MCG and the girl with pseudohermaphroditism underwent feminizing surgery, namely genital zone reconstruction, mobilization and excision of urogenital sinus walls, excision of genital cavernous bodies (in children with MCG) with clitoroplasty, vulvoplasty with labioplasty and vaginoplasty. Children with Morris syndrome are being prepared to feminizing surgery. Results. Each child had a personal examination plan. Verification of a child’s state lasted from several weeks to 2–3 months. Children who underwent feminizing surgery did not have male structures and had relatively well-developed female structures. More «natural», less traumatizing correction was preformed for each specific case. In all children who underwent surgery vagina opened into urogenital sinus lower than external urethral sphincter, so there was no need to form front vagina wall (back and side walls were formed). Out of all children treated in our hospital gender was legally changed to the opposite. Conclusions. The birth of a child with DSD is a most challenging problem for parents, doctors, psychologists and social workers. Such states require complex examination. Children with severe hypospadias must undergo genetic examination. Statistic data and our own experience show higher frequency of feminizing surgeries in such cases. The most difficult part of such surgeries proves to be the excision of genital cavernous bodies with clitoroplasty and clitoris translocation; the most responsible part in functional sense is vaginoplasty. Surgical treatment is not the final stage of treatment for such patients. Children with DSD require constant further dynamic checkups by gynaecologists, urologists, oncologists, endocrinologists, psychologists with relevant therapy correction. The research was carried out in accordance with the principles of the Helsinki declaration. The study protocol was approved by the Local ethics committee of all participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: disorders of sexual development (DSD), hermaphroditism, feminizing correction.


Neonatology ◽  
2017 ◽  
pp. 1-25
Author(s):  
Antonio Balsamo ◽  
Paolo Ghirri ◽  
Silvano Bertelloni ◽  
Rosa T. Scaramuzzo ◽  
Franco D’Alberton ◽  
...  

Author(s):  
Timothy F. Murphy

People with intersex conditions have male-typical and female-typical traits, to varying degrees in genetics and anatomy. Because of these variations, people with intersex conditions or disorders of sexual development can face difficulties related to their identities and relationships. As a matter of ethics, clinicians should exhibit humane behavior toward patients of all ages as they explore the meaning of their intersex condition for their health and well-being. Clinicians can also help families understand the meaning of that condition for their children and themselves. Clinicians will also have responsibilities toward child, adolescent, and adult patients bearing on the psychological effects of body modifications taken to normalize their sexual appearance. In addition, clinicians will have the responsibility to address the effects of body modifications hoped for by the patient. Observance of confidentiality is key in relationships with intersex patients.


1991 ◽  
Vol 1 (3) ◽  
pp. 141-143 ◽  
Author(s):  
M. A. Steller ◽  
J. T. Soper ◽  
C. A. Szpak ◽  
J. T. Lanman ◽  
D. L. Clark-Pearson

Gonadal dysgerminomas developed in two girls, aged 12 and 15 years. Both were initially treated with conservative unilateral gonadectomy. Forty-six, XY gonadal dysgenesis was not suspected in either patient due to the normal appearance of the contralateral gonads and internal female genital organs. One died of a second germ cell malignancy which developed in the contralateral ovary 9½ years later. The diagnosis of 46, XY gonadal dysgenesis was established by karyotype in both patients. Although conservative surgical management is desirable for nulliparous women with unilateral dysgerminomas, the presence of 46, XY gonadal dysgenesis should be suspected in all premenarchal girls with ovarian germ cell malignancies. If karyotyping reveals the presence of an Y chromosome, bilateral gonadectomy is indicated because of the risk that another neoplasm may develop in the contralateral ovary.


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