ABSTRACT
Posterior urethral valve (PUV) is the most common congenital cause of bladder outflow obstruction in male neonates. We report a preterm neonate with PUV who presented as nonimmune fetal hydrops with intestinal obstruction in the antenatal period. The mother of our patient is a 33-year-old woman who started her prenatal care at our hospital at 30 weeks' gestation. Her sonogram done at 32 weeks in our hospital revealed fetal hydrops. It showed polyhydramnios, mild pyelectasis of right kidney, normal left kidney, and fetal ascites. Amniocentesis revealed bile stained amniotic fluid. Ultrasound during the procedure showed dilated fetal bowel loops with increased echoes. Following delivery at 32 weeks postnatal exam showed ascites with absence of skin edema, pleural, or pericardial effusion. The abdominal sonogram showed distended urinary bladder and bilateral hydroureteronephrosis. Bladder catheterization was done which relieved the bladder outlet obstruction. Voiding cystourethrogram was done later which confirmed PUV and bilateral grade 5 vesicoureteral reflux. The formation of urinary ascites in PUV serves as a pop-off mechanism to relieve the intravesical and intrarenal pressure. When this happens by mechanisms other than bladder rupture, it can lead on to transient intestinal obstruction and hepatic synthetic defects.
ABSTRACT
BACKGROUND: Newborn screening (NBS) program is a simple and inexpensive method for early detection and treatment of over forty conditions as of 2005. Most cases of congenital hypothyroidism (CH) are sporadic and occurs in 1 in 3500 live births. Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive disorders that occurs in 1 in 1600 live births. The detection of CH is determined by either low T4 and/or elevated TSH and the detection of CAH is determined by elevated 17OH progesterone on Guthrie filter paper. Infants who meet the above criteria on NBS for either condition must undergo confirmatory testing. OBJECTIVE: Is the incidence of CH and CAH at FHMC, a community hospital in northern Queens, New York serving a multiethnic population different from theincidence of CH and CAH in New York State (NYS)? DESIGN/METHODS: We reviewed records of the infants recalled and confirmed to have CH and CAH for 2000, 2001 and 2004 and compared with reported incidence of the same conditions in NYS. RESULTS: The total number of live births in NYS was 760,112 and in FHMC 6348 for the three years studied, accounting for 0.7 to 0.8of total NYS live births. The population served by FHMC included 43White, 35Asian, 16Hispanic, 3Afro-American and 3other. A total of 5 patients were identified to have CH in the three years studied, accounting for an incidence of 0.12 (p > 0.05). Three patients were identified to have CAH in 2004 or an incidence of 1.4 (p < 0.05). [Table 1]. CONCLUSIONS: The incidence rate of CH was similar to that of NYS. However, the incidence rate of CAH at FHMC was greater than that reported by NYS. This difference in incidence rate for CAH may be related to the ethnic composition of the population that FHMC serves. Pediatricians and pediatric endocrinologists must be cognizant of the signs and symptoms of CAH in certain populations known to have higher incidence for this condition. Further follow up of incidence rate for CAH at FHMC is indicated since CAH was added to the NBS in 2003.
