Ambiguous genitalia–A social dilemma in Bangladesh: A case report

Abstract

HighlightsPhysical examination: Enlarged clitoris/micropenis, Bifid scrotum/labioscrotal fold, Separate urethral and vaginal orifices.USG: A rudimentary uterus with no ovary, the rt. testis in the mid inguinal canal, and lt. testis in the deep inguinal ring.Inj. Testosterone was given for consecutive 3 months followed by multiple surgeries at different settings.1st & 2nd stage of surgery: Seperation and closure of vaginal orifice, correction of chordee, and urethral plate was shaped.3rd stage of surgery: Urethroplasty, left orchiopexy and scrotal reconstruction were performed.

“Waking up” the sleeping metaphor of normality in connection to intersex or DSD: a scoping review of medical literature

“…normal vagina, normal penis, normal ovaries, normal testes, normal labia, normal ovarian tubes, normal fallopian tubes, abnormality of scrotum). Only in some occasions did the authors explain in more detail why these organs were considered (ab)normal, namely in terms of appearance (Adrião et al, 2020 ; Barham et al, 2016 ; Chen et al, 2018 ; Chowdhury et al, 2018 ; Fadil Iturralde et al, 2020 ; Hernández et al, 2016 ; Nasir et al, 2019 ; Ochi et al, 2019 ; Özdemir et al, 2019 ; Patil et al, 2019 ; Roth et al, 2018 ; Schteingart et al, 2019 ; Weaver et al, 2019 ), size (Adrião et al, 2020 ; Marei et al, 2016 ; Matsumoto et al, 2017 ; Mondal et al, 2016 ; Rich et al, 2016 ; Schteingart et al, 2019 ), length (AbouZeid & Mohammad, 2020 ; Finlayson et al, 2019 ; Goncalves et al, 2019 ; Morandi et al, 2018 ; Raveenthiran, 2017 ; van Leeuwen et al, 2019 ), location (Finlayson et al, 2019 ), and function (Heo et al, 2018 ; Lepais et al, 2016 ; Poyrazoglu et al, 2017 ; Raveendran et al, 2019 ; Raveenthiran, 2017 ). Some examples include: normal looking genitalia, normal appearing uterus, normal penis size, abnormal enlargement of clitoris, vagina with normal length, normally descended testes, abnormal location of the urethral meatus, and normal testicular function.…”

“…The studies touched on some important DSD-specific themes such as: (delay in, correct, timely etc.) diagnosis (Ameyaw et al, 2019 ; Caputo et al, 2019 ; Fadil Iturralde et al, 2020 ; Mondal et al, 2016 ; Şimşek et al, 2016 ; Teasdale & Morton, 2017 ; Xu et al, 2018 ), medical decision-making (Hernández et al, 2016 ; Khare et al, 2017 ; Teasdale & Morton, 2017 ; Witchel, 2018 ), multi-disciplinary teams (Berberoğlu & Şıklar, 2018 ; Caputo et al, 2019 ; Chowdhury et al, 2018 ; Heo et al, 2018 ; Hernández et al, 2016 ; Kearsey & Hutson, 2017 ; Nasir et al, 2019 ; Özdemir et al, 2019 ; Şimşek et al, 2016 ; Witchel, 2018 ; Wu et al, 2017 ) follow-up care (Berberoğlu & Şıklar, 2018 ; Chouhan et al, 2017 ; Hertweck & Rothstein, 2019 ), genetic screening (Kornman et al, 2018 ; Wu et al, 2017 ), fertility and reproduction (Adrião et al, 2020 ; Greeley et al, 2017 ; Hertweck & Rothstein, 2019 ; Pattamshetty et al, 2020 ; Witchel, 2018 ), the risk for tumor development of gonads (Chen et al, 2018 ; Chouhan et al, 2017 ; Heo et al, 2018 ; Huang et al, 2019 ; Lepais et al, 2016 ; Matsumoto et al, 2017 ; Pan et al, 2019 ; Şimşek et al, 2016 ) and sexuality (Baskin, 2017 ; Mendonca et al, 2016 ; Patil et al, 2019 ).…”

“…The study of Lepais and colleagues criticized the new DSD classification system for not taking into account histological anomalies (Lepais et al, 2016). Five studies used a term derived from intersex: intersexuality (Carsote et al, 2016;Mazen, 2017), intersex variation (Rich et al, 2016), intersex condition (Raveendran et al, 2019) or intersex disorder (Chowdhury et al, 2018;Raveenthiran, 2017;Teklu et al, 2016), not in the sense of an identity to be proud of, but rather as a more outdated medical concept for physical sex ambiguities. One paper (Roen et al, 2018) expressed its critique on the term "disorder" by placing it between hyphens.…”

Genetic counseling in the context of Bangladesh: current scenario, challenges, and a framework for genetic service implementation

“…Down syndrome is the most frequently reported chromosomal disorder in Bangladesh [ 15 , 52 ]. Other reports include Patau syndrome [ 53 ], Turner syndrome [ 54 , 55 ], intersex disorders (absence of typical binary notions of male or female bodies, e.g., due to aneuploidy) [ 56 , 57 ], and Klinefelter syndrome [ 55 , 58 , 59 ]. Among rare conditions, previous research reported several cases of Marfan syndrome [ 60 ], scleroderma [ 61 , 62 ], multiple epiphyseal dysplasia [ 63 ], Cutis laxa [ 64 ], and Bart’s syndrome [ 65 ].…”

Sexual Ambiguity—A Social Predicament

“…There are different causes of ambiguous genitalia in newborn, but the most common causes in inherent females are CAH, maternal ingestion of substances with male hormone activity during gestation, and male hormones generating tumor in female fetus, whereas possible causes in males genome are Leydig cell aplasia, 5-alpha-reductase deficiency, androgen insensitivity syndrome, and maternal ingestion of substances with female hormone activity during pregnancy [4][5][6].…”