Prenatal diagnosis of closed gastroschisis: What to expect in the most severe form of gastroschisis? Case report and literature review.

Closed gastroschisis (CG) and vanishing gastroschisis (VG) are the most severe forms of evolution of the malformation. In this case, a fetus presented with gastroschisis at 13 weeks, and the gastroschisis was not visualized at 22 weeks of gestation. Distal ileum, cecum, and ascending colon atresia were diagnosed at surgery, and the child is fully enteral-fed at 5 months of age. In a literature review of 43 cases of prenatal diagnosis of CS-VG, intrauterine death occurred in 4.5% of cases. 79.6% of the cases underwent surgical treatment. Of these cases, 20% of cases died due to complications of treatment, 26% were still under treatment and 54% are alive with enteral feeding. In 77% of the cases, the closure of the umbilical ring occurred after 26 weeks of gestation and the absence of prenatal diagnosis of CG-VG during pregnancy has a risk of 71% of death.

Choledochal cyst theories going pear-shaped? Evolution of choledochal cyst during intrauterine life in a case evaluated using magnetic resonance imaging and postnatal outcomes.

A choledochal cyst is a rare abdominal malformation and was first reported almost three centuries before. There are few theories describing the evolution of the cyst through the lifespan of affected patients until diagnosis and prompt treatment; however, there is no image documentation of the evolution of the malformation. In this report, we demonstrate the evolution of a type I choledochal cyst in a fetus from the 24th to the 37th week of gestation using magnetic resonance imaging and perinatal outcomes with correlation with pathophysiological mechanisms.

Corporeal sparing dismembered clitoroplasty: an alternative technique for feminizing genitoplasty.

PURPOSE: Management for clitoral enlargement remains controversial. New understanding of clitoral function stimulated a search for more conservative surgical approaches, such as recession or partial resection. However, these techniques risk decreasing clitoral sensation or causing painful erections. Moreover, irreversibility continues to be the principal problem that fuels patient, surgeon and societal anxiety in the management of this challenging developmental issue. We describe a new technique, corporeal sparing dismembered clitoroplasty, that dismembers the corporeal bodies and preserves all clitoral structures. MATERIALS AND METHODS: After obtaining full informed consent and institutional review board approval 8 consecutive patients with clitoral enlargement underwent corporeal sparing dismembered clitoroplasty. Five girls had congenital adrenal hyperplasia (Prader IV and V in 4 and 1, respectively), 1 had ovotesticular disorder of sexual differentiation and 2 had partial androgen insensitivity syndrome. One pubertal girl was tested with warm, cold and pain clitoral stimulation before and after surgery. For the clitoroplasty technique the glans and its neurovascular bundles are dissected from the corpora. The isolated corpus is then completely divided starting at the bifurcation. Each separated hemicorpus is rotated inferior and lateral, to be placed inside the labial scrotal folds. The glans is reduced by superficial excision of its epithelium and fixed to the pubic attachments. Labia minora are constructed with preputial Byars flaps. Labioplasty and vaginoplasty are then routinely performed. RESULTS: Eight patients 6 months to 13 years old underwent this procedure. Followup was 6 to 12 months. All patients recovered well from surgery without early complications. The initial cosmetic result was good in all girls. The hemicorpora were easily palpated inside their labia majora pouches, which retained the desired cosmetic appearance following feminizing genitoplasty. All glans clitoris were preserved. The teenaged patient does not report painful erections. She has maintained clitoral sensation and is satisfied with the cosmetic result. CONCLUSIONS: Conservative reconfiguration of the female genitalia without removing genital structures is feasible in girls with clitoral enlargement. The cosmetic appearance of the genitalia is acceptable, at least to the surgeon and parents, in that the enlarged clitoris is hidden. The physiological consequences of the current operation and any surgery in the future to reverse it are unknown. With these aspects in mind we believe that corporeal sparing dismembered clitoroplasty should be incorporated into the armamentarium of surgeons involved in the treatment of clitoral enlargement and presented as an option for feminizing genitoplasty.