Laparoscopic Uterine Preservation and Vaginal Reconstruction in a 13-Year-Old Girl with Uterus Didelphys, Bilateral Vesicovaginal Fistulas, and Transverse Vaginal Septum.

BACKGROUND: Complex female genital tract malformations account for 1.2% of all female genitourinary malformations. Although exceedingly rare, they can cause severe gynecologic symptoms in young women and lead to fertility problems. CASE: We present the case of a 13-year-old girl with primary amenorrhea referred for cyclic abdominal lower pain and menouria. Detailed diagnostics revealed uterus didelphys, transverse vaginal septum, and bilateral vesicovaginal fistulas. Laparoscopic left hemi-hysterectomy and salpingectomy were performed. The vesicovaginal fistula on the right side was excised, and the proximal vagina was anastomosed with the distal dimple. Since the operation, the patient has been pain-free and menstruating regularly from the right uterus. SUMMARY AND CONCLUSION: Preservation of the uterus should be considered in any case of complex female genital tract malformation and, as successful laparoscopic treatment advocates, a minimally invasive approach is feasible.

Steroid metabolomic signature of liver disease in nonsyndromic childhood obesity.

OBJECTIVE: Analysis of steroids by gas chromatography-mass spectrometry (GC-MS) defines a subject's steroidal fingerprint. Here, we compare the steroidal fingerprints of obese children with or without liver disease to identify the 'steroid metabolomic signature' of childhood nonalcoholic fatty liver disease. METHODS: Urinary samples of 85 children aged 8.5-18.0 years with BMI >97% were quantified for 31 steroid metabolites by GC-MS. The fingerprints of 21 children with liver disease (L1) as assessed by sonographic steatosis (L1L), elevated alanine aminotransferases (L1A) or both (L1AL), were compared to 64 children without markers of liver disease (L0). The steroidal signature of the liver disease was generated as the difference in profiles of L1 against L0 groups. RESULTS: L1 comparing to L0 presented higher fasting triglycerides (P = 0.004), insulin (P = 0.002), INS/GLU (P = 0.003), HOMA-IR (P = 0.002), GGTP (P = 0.006), AST/SGOT (P = 0.002), postprandial glucose (P = 0.001) and insulin (P = 0.011). L1AL showed highest level of T-cholesterol and triglycerides (P = 0.029; P = 0.044). Fasting insulin, postprandial glucose, INS/GLU and HOMA-IR were highest in L1L and L1AL (P = 0.001; P = 0.017; P = 0.001; P = 0.001). The liver disease steroidal signature was marked by lower DHEA and its metabolites, higher glucocorticoids (mostly tetrahydrocortisone) and lower mineralocorticoid metabolites than L0. L1 patients showed higher 5α-reductase and 21-hydroxylase activity (the highest in L1A and L1AL) and lower activity of 11ßHSD1 than L0 (P = 0.041, P = 0.009, P = 0.019). CONCLUSIONS: The 'steroid metabolomic signature' of liver disease in childhood obesity provides a new approach to the diagnosis and further understanding of its metabolic consequences. It reflects the derangements of steroid metabolism in NAFLD that includes enhanced glucocorticoids and deranged androgens and mineralocorticoids.

How does nonsyndromic craniosynostosis affect on bone width of nasal cavity in children? - Computed tomography study.

Craniosynostosis is caused by premature fusion of one or more cranial sutures, restricting skull, brain and face growth. Nonsyndromic craniosynostosis could disturb the proportions of face. Although morphometric diameters of nasal cavity in healthy children are already known, they have not been established yet in children with nonsyndromic craniosynostosis. The aim our study was to check whether diameters of bone structures of nasal cavity in children with nonsyndromic craniosynostosis measured in CT are within normal range. 249 children aged 0-36 months (96 with clinical diagnosis of nonsyndromic craniosynostosis and 153 in control group) were included into the study. The following diameters were measured on head CT scans: anterior bony width (ABW), bony choanal aperture width (BCAW), right and left posterior bony width (between bone sidewall and nasal cavity septum-RPBW and LPBW). The study group has been divided into 4 categories, depending on child's age. The dimensions measured between bone structures of nasal cavity were statistically significantly lower in comparison to the control group. They did not depend on the sex for ABW, nor on age in groups 7-12 months and < 2 years for BCAW, RPBW and LPBW. The measured dimensions increased with age. In children with nonsyndromic craniosynostosis the diameter of pyriform aperture and bony choanal aperture were lower than in controls, what may be described as fronto-orbital anomalies. Morphometric measurements of anthropometric indicators on CT scans could be used as standards in the clinical identification of craniosynostosis type and may help in planning surgical procedures, particularly in the facial skeleton in children.

Intervertebral disc calcification in children: Case description and review of relevant literature.

BACKGROUND: Intervertebral disc calcification is a rare condition in children; in most cases, it is asymptomatic and therefore not diagnosed. CASE REPORTS: In our study, we present a case of idiopathic intervertebral disc calcification within the cervical segment, at the level of C2/C3 and C4/C5 vertebrae in a 5-year-old girl with torticollis. Basic neurological examination supplemented by X-ray examination was performed, showing calcification within the cervical segment at the level of C2/C3 and C4/C5 vertebrae. CONCLUSIONS: In order to complement the diagnostics, a CT scan of the cervical spine was performed; the scan confirmed the diagnosis and revealed additional calcification of the anterior longitudinal ligament at the level of C4/C5 vertebrae.