Prenatal ultrasound diagnosis of fetal chest wall cystic lymphangioma: An Italian case series

Fetal lymphangioma is a rare congenital malformation of lymphatic system that involve the skin and the subcutaneous tissue. The vast majority of the lymphangioma occurs in the neck. More rarely lymphangiomas may occur in the axillary region, including chest wall. Our consecutive case series study included three cases of fetal chest wall cystic lymphangiomas. In our cohort, fetal chest wall cystic lymphangiomas were the 18.8% of the all cases of lymphangiomas of axillary region. In all the three cases no other fetal abnormalities were evaluated, and the chest wall cystic lymphangiomas were unilateral, honeycombed in appearance, with multiple echo-free area of varying size in the mass, with no color flow on Doppler sonography, and with a trend to increase during the gestation. The incidence of chromosomal abnormalities was 33.3%, with one case out of the three being trisomy 21.The literature review revealed only seven cases of fetal chest wall cystic lymphangiomas. The cases were not associated with other abnormalities, nor with abnormal karyotype and only one case of fetal death was reported. Three women delivered vaginally. In summary, fetal chest wall cystic lymphangioma is a very rare malformations with only seven cases reported in the literature. This malformation is usually not associated with abnormal karyotype or other abnormalities and the neonatal outcome is favorable after surgical removal. Spontaneous vaginal delivery may be a safe approach for delivery women with fetal chest wall cystic lymphangioma. CONDENSATION: Fetal chest wall cystic lymphangioma is a very rare malformations with only seven cases reported in the literature. This malformation is usually not associated with abnormal karyotype or other abnormalities and the neonatal outcome is favorable after surgical removal. Spontaneous vaginal delivery may be a safe approach for delivery women with fetal chest wall cystic lymphangioma

Step-Up Approach for Sodium Butyrate Treatment in Children With Congenital Chloride Diarrhea

Objectives: Oral salt substitutive therapy is pivotal for the survival of patients with congenital chloride diarrhea (CLD), however this therapy is unable to influence the symptoms severity. Butyrate has been proposed to limit diarrhea severity in CLD. Unfortunately, the optimal dose schedule is still largely undefined. In addition, butyrate seems not to be well-tolerated by all patients, with some subjects reporting diarrhea worsening. We investigated the efficacy of a step-up therapeutic approach with sodium butyrate in patients who experienced a diarrhea worsening or an absent improvement after the direct administration of 100 mg/kg/day of sodium butyrate. Methods: The efficacy of a step-up therapeutic approach starting from 50 mg/Kg/day with a subsequent 25 mg/kg/day weekly increase up to 100 mg/kg/day of oral sodium butyrate was investigated in previously three unresponsive CLD children. Results: The step-up therapeutic approach resulted effective in limiting diarrhea severity in all our three previously unresponsive CLD patients. Conclusions: Our results suggest the efficacy of the step-up therapeutic approach in CLD children

The Vermian-Crest Angle: A New Method to Assess Fetal Vermis Position within the Posterior Fossa Using 3-Dimensional Multiplanar Sonography.

BACKGROUND Normal morphometry of the vermis and its relation to the posterior fossa (PF) rule out most major anomalies of the cerebellum. However, accurate categorization of the position and size of the fetal vermis remains a challenge. OBJECTIVE Our aim was to test a new method to assess the position and size of fetal vermis on 3-dimensional ultrasound (3D-US). METHODS We measured the vermian-crest angle (VCA) in normal fetuses using multiplanar 3D-US. We also assessed the diameters (superoinferior, anteroposterior, and horizontal) and volume of the vermis. The Spearman rank test and linear and polynomial regression analyses were used for statistical purposes. RESULTS We included 126 fetuses. Mean ± SD gestational age (GA) was 26.3 ± 4.6 (range 17-35.5) weeks. Mean ± SD superoinferior, anteroposterior, and horizontal diameters were 16.2 ± 4.9, 11.2 ± 3.6, and 5.6 ± 1.6 mm, respectively. Median (range) vermian volume was 0.50 (0.05-2.9) cm3. The VCA was 64.49° ± 11.45. We found no correlation between GA and VCA (r = 0.15; p = 0.13), a linear correlation between GA and vermian diameters, and a quadratic correlation between GA and vermian volume. CONCLUSIONS We provide a new method to assess vermian position and size within the PF using 3D-US. The combined information may be of value for screening purposes, particularly to differentiate between the various pathological situations encountered within the PF

Laparoscopic Management of Abdominal Pregnancy

Objective To illustrate the laparoscopic surgical management of a particular localization of extrauterine pregnancy misdiagnosed until 12 weeks gestational age, complicated by hemoperitoneum and abortion. Design Canadian Task Force III on the Periodic Health Examination's Levels of Evidence. Setting The prevalence of ectopic pregnancy among women presenting to an emergency department with first trimester bleeding, pain, or both ranges from 1% to 16% [1]. The most common localization of ectopic pregnancy is the fallopian tubes, whereas abdominal pregnancy accounts for at least 1% of extrauterine pregnancies. The reported incidence of abdominal pregnancy ranges from 1:10 000 to 1:30 000 pregnancies [2]. Abdominal pregnancy can be localized in the pelvic cul-de-sac, broad ligament, bowel, or pelvic sidewall. This rare type of ectopic pregnancy is often misdiagnosed until later in pregnancy, evolving in hemoperitoneum, abortion, embolism, or rarely, in diagnosed cases, live birth by cesarean section. In the literature, it is recommended that the placenta be left in situ in cases of abdominal pregnancy to avoid hemorrhage and organ injury, even though this approach may be associated with a higher rate of postoperative complications, such as infection, secondary bleeding, and cancer transformation [3]. We present a case of abdominal pregnancy in which the gestational sac was implanted in the broad ligament and resulted in hemoperitoneum at 12 weeks gestational age. Intervention In August 2010, a 35-year-old woman, gravida 3 para 1, presented at the Di Meglio ultrasound diagnostic center in Naples for a noninvasive prenatal ultrasound (bi-test) to confirm gestational age in what to that point had been considered a normal pregnancy at 12 weeks gestation. Ultrasound revealed an ectopic abdominal pregnancy with a live fetus located in the left parauterine side. A suspicious fluid level in the pouch Douglas was also detected, and so the woman was advised to go to an obstetric hospital for a medical evaluation of the clinical situation (starting hemoperitoneum). Later that same day, the woman presented at the Villa dei Platani Hospital in Avellino, where ultrasound confirmed increased fluid in the pouch of Douglas, along with initial signs of hemoperitoneum and loss of the fetal heartbeat. The woman was immediately transferred to the Malzoni Center for Advanced Endoscopic Gynecological Surgery in Avellino, where she underwent operative laparoscopy for removal of the abdominal pregnancy (surgeon, M.M.). Informed consent for the laparoscopic surgery was provided by the patient in accordance with local regulations. The patient also provided informed consent for the use of images and a video of the procedure. Institutional Review Board approval was not required. The procedure involved laparoscopic hemoperitoneum drainage (at least 500 mL of blood), left adnexectomy after transperitoneal identification of the left uretheral pathway, and complete removal of left broad ligament pregnancy abortion with consensual removal of the ectopic placenta. Conclusion The laparoscopic management of abdominal pregnancy and hemoperitoneum resulting from rupture of the gestational chamber and abortion was optimal. With this minimally invasive technique, it was possible to drain the hemoperitoneum completely and then proceed to total removal of the gestational chamber and the fetus. Thanks to the magnification of the image by laparoscopy, it was also possible to completely remove the placenta and the cotyledons from the peritoneal surface, thereby avoiding possible postoperative bleeding, infection, and sepsis resulting from retention of incomplete removal of the placenta. On the first postoperative day, the patient was in excellent clinical condition, with a marked reduction in circulating \uce\ub2-human chorionic gonadotropin. She was discharged on the second postoperative day and currently is in good health