CASE: A 9-year-old girl presented with 3 days of intermittent lower abdominal pain. Ultrasound revealed an ovarian mass, but laparotomy revealed an ischemic enlarged ovary and uterus rotated 180°. No reperfusion occurred after 60 minutes. A subtotal hysterectomy and right salpingoophorectomy were thus performed.
CONCLUSION: Uterine and adnexal torsion presents with symptoms similar to those of adnexal torsion. Delays in diagnosis and referral continue to be an issue, resulting in suboptimal outcomes. Uterine torsion, although exceedingly rare in childhood, appears to occur only in the setting of ovarian masses, which provide the impetus for the rotational force to the elongated cervix of the prepubertal uterus.
METHODS: A retrospective cohort study was conducted at the Royal Children's Hospital in Melbourne, Australia. Data were collected from medical records of patients presenting with dysmenorrhea and/or pelvic pain.
RESULTS: Of 154 patients, mean age of presentation was 15.7 years (SD = 2.2) and mean duration of pain was 14.9 months (SD = 10.8). Regular cycles were reported by 64.5%, and heavy menstrual bleeding (HMB) in 67.8%. Patients self-reporting HMB reported less pain on the day prior to menses than those not reporting HMB (P
DESIGN: Multi-center cross-sectional study.
SETTING: Two tertiary medical centers in Malaysia.
PARTICIPANTS: A total of 59 patients with CAH who were raised as females and more than 10 years old, and their parents.
METHODS: The CAH respondents completed the validated and translated Body Image Disturbance Questionnaires (BIDQ). All CAH respondents and their parents underwent semi-structured interviews to explore their views on FG.
MAIN OUTCOME MEASURES: Body image disturbance score and perspectives on FG.
RESULTS: The 59 CAH respondents consisted of 12 children, 29 adolescents, and 18 adults. The majority were of Malay ethnicity (64.4%) with classical CAH (98.3%) and had undergone FG (n = 55, 93.2%). For the BIDQ scores, the median score (interquartile range) for general body image was 1.29 (0.71), range 1.00-3.29, whereas the genital appearance score was 1.07 (0.39), range 1.00-4.29, revealing a greater concern for general body parts over genitalia. With regards to FG, it was perceived as necessary. Infancy and early childhood were perceived as the best timing for first FG by both respondents and parents, most preferring single-stage over 2-stage surgery.
CONCLUSIONS: General body appearance concerns were greater than for genital appearance, with more impact on the patients' lives. Contrary to much international opinion, feminizing surgery was perceived as necessary and appropriate by CAH respondents and their families, and should be offered in infancy or early childhood. Future qualitative studies are recommended.
METHODS: A cross-sectional study was conducted over 6 months in the two main tertiary centres for CAH patients in Malaysia. Participants including 59 female-raised CAH patients (mean age ± SD = 16.3 ± 4.2 years, range 10-28 years) compared to 57 age-matched female diabetic patients (mean age ± SD = 16.5 ± 3.4 years, range 10-26 years). Socio-demographic and medical profiles was obtained through semi-structured interviews. HRQOL of participants were evaluated utilising validated, Malay translated questionnaires which were age appropriate: Pediatric Quality of Life Inventory (PedsQL v4.0) scales for Child (8-12) and Adolescent (13-18) and Medical Outcome Survey 36-item Short Form version. These were then compared to the diabetic controls.
RESULTS: The CAH participants consisted of children (ages 10-12 years, n = 12), adolescents (ages 13-17 years, n = 29) and adults (≥ 18 years, n = 18). The majority were Malays (64.4%) and had salt-wasting CAH (67.8%). There were no significant differences between the total mean score of the HRQOL of the combined children and adolescents CAH group (total mean score ± SD = 81.6 ± 17.9, 95% CI = 75.6-87.6) when compared to age-matched diabetic patients (total mean score ± SD = 80.8 ± 11.0, 95% CI = 77.0-84.5, P = 0.81, effect size = 0.05); no significant difference between the adult CAH and diabetic controls in the physical [median score (IQR) CAH vs diabetics; 49.3 (11.4) vs. 50.2 (6.1), P = 0.60, effect size = 0.09] and the mental composite scores [median score (IQR) CAH vs. diabetics; 47.8 (14.1) vs. 50.0 (10.8), P = 0.93, effect size = 0.01].
CONCLUSIONS: The HRQOL of the Malaysian CAH cohort were comparable to the diabetic controls.