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Cloaca
Published in Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg, Operative Pediatric Surgery, 2020
With a unified approach that includes the participation of a team of dedicated surgeons (pediatric surgeon, pediatric urologist, pediatric orthopedic surgeon, and neurosurgeon), most patients born with cloacal exstrophy can have a pull-through and are kept clean with bowel management, and dry with intermittent catheterization, allowing for a better quality of life (Figure 44.22).
Variation of sex differentiation
Published in Joseph S. Sanfilippo, Eduardo Lara-Torre, Veronica Gomez-Lobo, Sanfilippo's Textbook of Pediatric and Adolescent GynecologySecond Edition, 2019
Anne-Marie Amies Oelschlager, Margarett Shnorhavorian
Cell migration during fetal development may be impaired and result in differences in the development of the external genitalia or internal reproductive structures. These conditions are also associated with multiple other anomalies.12 For example, genitourinary anomalies are common with VACTERL or VATER association, which represents vertebral anomalies, anorectal malformation, cardiovascular anomalies, tracheoesophageal fistula, esophageal atresia, renal anomalies, and limb anomalies. In 46,XX individuals, Mayer-Rokitansky-Küster-Hauser syndrome, which occurs in 1:4000–1:5000 females, is associated with renal, spine, and ear anomalies.13 Cloacal anomalies, including cloacal exstrophy or bladder exstrophy, are less common. Typically, these patients will have normal ovarian function but often have underdevelopment or anomalies of the clitorophallus, labia, vagina, or uteri.14
Exstrophy-epispadias complex
Published in J Kellogg Parsons, E James Wright, The Brady Urology Manual, 2019
Cloacal exstrophy is often associated with: MyelomeningoceleShort gut syndromeTethered cordHip dislocationClubfoot.
Fetal Tethered Spinal Cord: Diagnostic Features and Its Association with Congenital Anomalies
Published in Fetal and Pediatric Pathology, 2023
Xiaomei Yang, Shiyu Sun, Yizheng Ji, Yasong Xu, Li Sun, Qichang Wu
Fetal pathological examination demonstrated low-lying conus medullaris in all 26 cases. Among them, 22 cases had prenatal ultrasound results indicating low-lying conus medullaris. According to the results of the pathological examination, four cases (4/26, 15.4%) were diagnosed with solitary TSC, and 22 cases (22/26, 84.6%) were non-solitary TSC. Among the 22 cases of non-solitary TSC, nine cases (9/26, 34.6%) were combined with neural tube malformations, and 13 cases (13/26, 50.0%) were combined with multisystem congenital malformations. In the nine neural tube malformation combined cases, four cases were combined with spina bifida occulta, four cases were combined with spina bifida aperta, and one case was combined with severe hydrocephalus. Among the 13 TSC cases with combined multisystem congenital malformations, two cases were diagnosed with combined kidney development abnormalities, four cases were diagnosed with vertebral defects, anal anomalies, cardiac defects, trachea-oesophageal fistula, renal anomalies, and limb anomalies (VACTERL) syndrome, one case was diagnosed with cloacal exstrophy (OEIS syndrome), and six cases were diagnosed with fetal chromosomal karyotype abnormality (Table 1).
Stability and Change in Sexual Orientation and Genital Arousal over Time
Published in The Journal of Sex Research, 2023
Dragos C. Gruia, Luke Holmes, Jaime Raines, Erlend Slettevold, Tuesday M. Watts-Overall, Gerulf Rieger
One hypothesis is that sexual orientation is a stable trait, which is possibly determined during early development and does not change later in life (Bogaert & Skorska, 2020; Breedlove, 2017; Farr et al., 2014). Support for this notion comes from case studies of males who had their genitals surgically removed due to a rare medical condition called cloacal exstrophy, and who were surgically re-assigned and raised as females. Despite these profound changes, the vast majority of these individuals later identified as males with attraction to females (Diamond et al., 2011; Meyer-Bahlburg, 2005; Reiner, 2004; Reiner & Gearhart, 2004; Zucker, 1999). These results suggest that at the very least, male sexual orientation is not malleable even in the face of severe social and physical manipulations. Moreover, conversion therapies, which make deliberate efforts to change male and female sexual orientation in adulthood, show little to no evidence that change is possible (Drescher et al., 2016; Haldeman, 1994). One author supported the effectiveness of reparative therapies (Spitzer, 2003); however, this has been criticized, as it was impossible to judge whether the reports of those undergoing therapy were truthful (Armelli et al., 2012; Drescher & Kenneth, 2013). Research into gender behavior also supports the idea that sexual orientation is a stable trait that forms early, since childhood gender nonconformity (femininity in males and masculinity in females) is a robust predictor of a non-heterosexual sexual orientation in adulthood (Bailey et al., 2016; Bailey & Zucker, 1995; Watts et al., 2018; Xu et al., 2021).
A case of penile duplication with neonatal teratoma and bladder neck incompetence
Published in Scandinavian Journal of Urology, 2021
Martin Skott, Ulrik Korsgaard, Yazan F. Rawashdeh
In the current case, pseudodiphallia was present, with a rudimentary atrophic penis with only one well-developed corpus cavernosum and one less developed corpus spongiosum and lack of urethral tissue and hence, no connection to the bladder. The patient voided through the orthotopic penis which had two corpora cavernosae and a corpus spongiosum with normal erectile function. Cystourethroscopy of the orthotopic penis revealed a normal distal urethra, but a short posterior urethra and a wide-open bladder neck. The latter has been described before in patients with penile duplication [9]. Some of these cases, moreover have other characteristics of exstrophy-epispadias complex [8], including variants of covered exstrophy, pseudoextrophy and cloacal exstrophy with concomitant penile duplication [13–14]. In the present case, no other characteristics of exstrophy-epispadias complex were noticed.