Ex-utero Intrapartum Treatment (EXIT) Procedure for Giant Fetal Epignathus.

BACKGROUND: Large fetal oropharyngeal tumors are rare, and have the potential to cause airway obstruction during birth. CASE CHARACTERISTICS: A 35-year-old woman with antenatally diagnosed large heterogenous mass in fetal neck displacing trachea and filling up the orophanygeal space. INTERVENTION: The infant was delivered at 31 weeks of gestation by ex-utero intrapartum therapy procedure to secure the airway. OUTCOME: Tumor was resected successfully on day 8 of life. Histopathology revealed mixed teratoma. MESSAGE: Ex-utero intrapartum therapy for fetuses with severe upper airway compromise may prove life-saving.

Nonimmune hydrops fetalis in a children's hospital: a six-year series.

INTRODUCTION: We aimed to study the profile of nonimmune hydrops fetalis (NIHF) in the local population and identify its outcomes and causes. METHODS: We carried out a retrospective review of the medical records in KK Women's and Children's hospital, a single tertiary referral centre, for pregnancies with an antenatal diagnosis of NIHF in the six-year period from 1 January 2005 to 31 December 2010. RESULTS: A total of 29 cases of NIHF were identified; 19 (66%) cases underwent karyotype evaluation, 17 (59%) underwent intrauterine infection screening, and all underwent antenatal thalassaemia screening. The median gestational age at diagnosis was 27 (range 12-37) weeks, median gestational age at birth was 33 (range 27-37) weeks, and median birth weight of live births was 2,480 (range 1,230-3,900) g. The aetiologies for NIHF were identified in 20 (69%) cases, which included cardiac anomalies (n = 5), haematological problems (n = 4), congenital tumours (n = 4), genetic/metabolic disorders (n = 4) and cystic hygromas (n = 3). The cause of NIHF was not identified in the remaining 9 (31%) cases. There were 19 live births - 8 (42%) survived and 11 (58%) died in the neonatal period - and one stillbirth. Nine women opted for medical termination of pregnancy following the diagnosis of NIHF. CONCLUSION: It is important to thoroughly investigate all cases of NIHF and identify its causes in order to provide appropriate antenatal and postnatal counselling. In our series, almost one-third of NIHF cases had no identified aetiology. The neonatal mortality rate was approximately 58%.

Borderline viability--neonatal outcomes of infants in Singapore over a period of 18 years (1990 - 2007).

INTRODUCTION: This study assesses the trends and predictors of mortality and morbidity in infants of gestational age (GA) <27 weeks from 1990 to 2007. MATERIALS AND METHODS: This is a retrospective cross-sectional cohort study of infant deliveries between 1990 and 2007 in the largest perinatal centre in Singapore. This is a study of infants born at <27 weeks in 2 Epochs (Epoch 1 (E1):1990 to 1998, Epoch 2 (E2):1999 to 2007) using logistic regression models to identify factors associated with mortality and composite morbidity. The main outcomes that were measured were the trends and predictors of mortality and morbidity. RESULTS: Four hundred and eight out of 615 (66.3%) live born infants at 22 to 26 weeks survived to discharge. Survival improved with increasing GA from 22% (13/59) at 23 weeks to 87% (192/221) at 26 weeks (P <0.01). Survival rates were not different between E1 and E2, (61.5% vs 68.8%). In logistic regression analysis, higher survival was independently associated with increasing GA and birthweight, while airleaks, severe intraventricular haemorrhage (IVH) and necrotizing enterocolitis (NEC) contributed to increased mortality. Rates of major neonatal morbidities were bronchopulmonary dysplasia (BPD) (45%), sepsis (35%), severe retinopathy of prematurity (ROP) (31%), severe IVH/ periventricular leucomalacie (PVL) (19%) and NEC (10%). Although composite morbidity comprising any of the above was not significantly different between the 2 Epochs (75% vs 73%) a decreasing trend was seen with increasing GA (P <0.001). Composite morbidity/ mortality was significantly lower at 26 weeks (58%) compared to earlier gestations (P <0.001, OR 0.37, 95% CI, 0.28 to 0.48) and independently associated with decreasing GA and birth weight, male sex, hypotension, presence of patent ductus arteriosus (PDA) and airleaks. CONCLUSION: Increasing survival and decreasing composite morbidity was seen with each increasing week in gestation with marked improvement seen at 26 weeks. Current data enables perinatal care decisions and parental counselling.

High prevalence of hearing loss in Down syndrome at first year of life.

INTRODUCTION: Infants with Down syndrome (DS) are at higher risk of hearing loss (HL). Normal hearing at one year of age plays an important part in language development. An audit was conducted to determine the impact of the newborn hearing screening program on the incidence, type and timing of diagnosis of HL during first year of life. MATERIALS AND METHODS: Infants with DS were scheduled for Universal Newborn Hearing Screening (UNHS) within 4 weeks of life. If they passed, they had a high-risk screen at 3 to 6 months. They were referred to the otolaryngology department if they did not pass the UNHS or the high-risk screen. Information was obtained from the computerised data tracking system and case notes. Infants born from April 2002 to January 2005 and referred to the DS clinic of our hospital were analysed. RESULTS: Thirty-seven (82.2%) of 45 infants underwent UNHS, of which 12 (32.4%) infants did not pass. Of remaining 33 infants, 27 had high-risk screen done of which 14 (51.8%) did not pass. Twenty-eight infants were referred to the ear, nose, throat (ENT) clinic: 12 from UNHS, 14 from high-risk screens and 2 from the DS clinic. Eleven (39.2%) defaulted follow-up. Fourteen (82.3%) of 17 infants who attended the ENT Clinic had HL. Twelve (85.7%) were conductive, and 2 (14.2%) mixed. Nine (64.2%) had mild-moderate HL and 3 (21%) had severe HL. The mean age of diagnosis was 6.6 ± 3.3 months. All were treated medically, plus surgically if indicated. By 12 months of age, the hearing had normalised in 4 (28.6%) infants and remained the same in 3 (21.4%). Five (35.7%) defaulted follow-up. Thirty-five out of 45 (77.8%) underwent complete hearing screen in the first year of life (UNHS & High-risk screen). Six out of 45 (13.3%) had incomplete screening. Fourteen out of 41 (34.1%) had HL of varying degrees. Four out of 45 (8.8%) did not have any audiological assessment in first year of life. CONCLUSION: The incidence of HL in the first year of life was high (34.1%). Eighty-five percent were conductive with 64.2% in mild-moderate range. One third of infants hearing normalized after treatment, one third remained unaltered and one third of infants did not attend follow-up. An aggressive approach involving early screening after birth and continued surveillance and early referral to appropriate agencies are essential for establishing timely diagnosis and treatment. Measures to reduce the high default rate during long-term follow-up are needed. Parent education and integrated multidisciplinary follow-up clinic may be useful.