Naoyuki Nakanami

Prenatal Sonographic Findings and Biochemical Assessment of Amniotic Fluid in a Fetus With Congenital Chloride Diarrhea

(CCD) is a rare autosomal recessive disorder in which the transport of chloride in the distal ileum is disrupted.1 Prenatal sonographic features of CCD include multiple dilated bowel loops and polyhydramnios.1–4 We describe more specific prenatal sonographic findings that can be combined with a biochemical assessment of the amniotic fluid to correctly diagnose CCD prenatally. A 36-year-old Japanese woman, gravida 3, para 2, was referred to our hospital at 32 weeks’ gestation for evaluation of fetal dilated bowel loops. Her familial and medical histories were unremarkable. A sonographic examination showed a single male fetus with generalized dilatation of the bowel in both the peripheral and caudal parts of the abdomen. Peristalsis was evident. The bowel had a honeycomb appearance (Figure 1). Polyhydramnios (amniotic fluid index, 29.2) was present. The fetal urine production rate (3.42 mL/10 min) was normal (reference range, 1.5–5.5 mL/10 min).5 No other abnormalities were detected. Neither fetal ascites nor intraperitoneal calcification was noted. Fetal growth was appropriate for the gestational age, but the fetal abdominal circumference (31.8 cm) was greater than the mean ± 1.5 SD value (28.5cm).6 Oral glucose tolerance test and TORCH (toxoplasmosis, other infections, rubella, cytomegalovirus, and herpes simplex) serologic test results were negative. At 32 weeks’ gestation, amnioreduction was performed for maternal abdominal distension. Analysis of electrolytes in the amniotic fluid showed concentrations of sodium, potassium, and chloride of 129, 4.4, and 112 mmol/L, respectively (Table 1). The karyotype was normal male. The elevated chloride level (reference value, 107.4 ± 3.4 mmol/L)7 was suspicious for CCD. Serial follow-up scans showed no notable change except an increasing fetal abdominal circumference. The elevated chloride levels in the amniotic fluid persisted. At 36 weeks 1 day, the mother went into spontaneous labor, and an emergent cesarean delivery was performed for cephalopelvic disproportion. The neonatal birth weight was 2755 g; the Apgar scores were 9 and 9 at 1 and 5 minutes, respectively; and the umbilical artery pH was 7.39. After birth, the neonate had a distended abdomen and yellowish watery diarrhea without meconium passage. An abdominal radiograph immediately after birth showed no gaseous bowel dilatation (Figure 2A), indicating fluid-filled bowel loops. A barium enema on the first day of life showed no evidence of bowel obstruction or bowel malrotation. Abdominal radiographic

Acute Pancreatitis and Cholecystitis Associated with Postpartum HELLP Syndrome: A Case and Review

We report a case of preeclampsia associated with hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome and concomitant nonbiliary acute pancreatitis and cholecystitis in the first postpartum day. A thorough investigation ruled out known etiologies of both pancreatitis and cholecystitis. Following conservative treatment, the patients HELLP syndrome, pancreatitis, and cholecystitis resolved on the third postpartum day. Preeclampsia is associated with microvascular abnormalities that may involve the splanchnic circulation. These abnormalities may cause not only HELLP syndrome but also pancreatitis and cholecystitis. Recognizing that ischemia can damage not only the liver but also the pancreas and gallbladder, could result in improvements in the diagnosis and management of pancreatitis in patients with preeclampsia.

Transplacental digitalization for nonimmune hydrops fetalis caused by isolated noncompaction of the ventricular myocardium

Noncompaction of the ventricular myocardium (NCVM) is a rare form of cardiomyopathy with a poor prognosis, and it is characterized by prominent trabeculations and deep intertrabecular recesses. This disease is speculated to occur because of arrest of the normal myocardial development process during early embryogenesis. In symptomatic infants, cardiac transplantation may be the only current curative option. Although NCVM is a congenital disorder, there are only a few previous reports on its status during fetal life. Here we report a case of nonimmune hydrops fetalis caused by this rare disease; in this case, digoxin administration to the mother could improve the fetal hemodynamics, and evaluation of the circulatory state of the fetus by using the preload index of the inferior vena cava (PLI) was proven to be sensitive and useful.

Pleural fluid/serum immunoglobulin ratio is a diagnostic marker for congenital chylothorax in utero

Abstract Objective: To distinguish congenital chylothorax from other causes of hydrothorax in utero. Methods: Serum and pleural fluid samples from 8 fetuses with congenital chylothorax and 5 control fetuses with other causes of hydrothorax were tested for total protein, albumin, IgG, IgA, and IgM. Results: Fetuses with congenital chylothorax had higher levels of IgG in pleural fluid, but not the other four proteins, than control fetuses (P<0.05). There were no significant differences in serum proteins among fetuses. When we examined pleural fluid to serum ratios, the IgG ratio in fetuses with congenital chylothorax was significantly higher than that of control fetuses (P<0.05). The IgG ratio in chylothorax was greater than 0.6 regardless of lymphocyte count. Conclusion: Pleural fluid/serum IgG ratio may be a diagnostic marker for congenital chylothorax in utero.

Fetal neck capillary hemangioma associated with Kasabach-Merritt syndrome.

The most common fetal neck masses include teratomas and lymphangiomas. Goiters and hemangiomas are relatively rare. 1 Correct diagnosis of tumor type is important because these neck tumors differ in prognosis and treatment. The prenatal diagnosis of cavernous hemangiomas in the fetal neck has been reported previously. 2,3 The characteristic sonographic and magnetic resonance imaging (MRI) features of cavernous hemangiomas are increased or pulsatile flow signals in the mass with hyperintensity on T2-weighed images. However, the prenatal sonographic and MRI features of capillary hemangiomas are not well described. In this report, we describe a giant fetal neck capillary hemangioma associated with Kasabach-Merritt syndrome (KMS) and discuss the prenatal sonographic and MRI features of capillary hemangiomas.