Giant Isolated Omphalocele: Role of Prenatal Diagnosis in Prognostic Asessment and Perinatal Management.

Omphalocele is a congenital malformation of the abdominal wall consisting of a protrusion of the abdominal contents at the base of the umbilical cord. It has a high association with genetic and structural defects; however, if the latter is ruled out, its prognosis improves significantly. Prenatal diagnosis has a key role in this condition as omphalocele can be diagnosed by ultrasound in the first trimester scan, enabling a coordinated approach strategy to achieve the best perinatal results. We present a case report of a pregnant patient with a fetus having a giant omphalocele in which prenatal diagnosis played a decisive role, allowing the coordination of a multidisciplinary team, which was crucial in the immediate care of the newborn.

Sentinel node disection in the treatment of early stages of vulvar cancer.

OBJECTIVES: To assess the results of sentinel lymph node (SLN) detection in the initial stages of vulvar cancer and the recurrences that may appear. STUDY DESIGN: 76 patients with vulvar carcinoma, Stage I and II. Between 2000 and 2010, identification of the SLN was performed with a perilesional injection of Tc99m and vital dye. Ninety sentinel lymph nodes were found. They were removed separately, and lymphadenectomy was performed depending on the involved areas. Vulvar tumour was also removed. RESULTS: 76 patients were included in the study; 20 (22.22%) out of 90 SLNs presented metastases and 70 (77.77%) did not. There were no false negatives, and the sensitivity and negative predictive value reached 100%. Thirty-six months after treatment, one patient presented recurrence with a negative SLN, and two with positive SLNs. CONCLUSION: Biopsy of the SLN is a reasonable alternative to lymphadenectomy in patients with vulvar cancer Stage I and II.

Prenatal diagnosis of right aortic arch: associated findings, pregnancy outcome, and clinical significance of vascular rings.

OBJECTIVES: To analyze the characteristics and clinical implications of right aortic arch (RAA) detected in fetal life. METHODS: Retrospective study of all cases of RAA diagnosed prenatally in high-risk patients who underwent fetal echocardiography between 2000 and 2007. RESULTS: There were 48 RAA; 18 had vascular ring (Group 1), including 15 RAA with aberrant left subclavian artery and 3 double aortic arch, and 30 had not vascular ring (Group 2), all RAA with mirror-image branching. The prenatal diagnosis was confirmed in 94%. In Group 1 most fetuses had normal heart (89%), and none had 22q11 deletion. There were 16 live births and all infants but one are asymptomatic (mean follow-up of 31 months). In Group 2 almost all fetuses had congenital heart defects (CHDs) (97%), and five were chromosomally abnormal (17%), including four 22q11 deleted. The 1-year survival rate was 69%, which was significantly higher in Group 1 (89% vs 57%, p < 0.05). CONCLUSIONS: RAA can be accurately diagnosed by fetal echocardiography. The outlook for these patients largely depends on the presence of associated defects, mainly CHDs, and their severity. If isolated, aortic arch anomalies are asymptomatic vascular variants in most cases. This information is important to assist parental counseling in pregnancy.