Akshaya Vachharajani

Cushing syndrome from percutaneous absorption of 1% hydrocortisone ointment in Netherton syndrome.

Abstract:  Netherton syndrome is a congenital skin disease associated with decreased skin barrier function and increased percutaneous absorption. We report an 11‐year‐old boy with Netherton syndrome who developed Cushing syndrome after application of 1% hydrocortisone ointment to his entire body for more than 1 year. This presentation illustrates that even low‐potency steroid ointments should be used with caution in Netherton syndrome and warns about the use of long‐term topical medications with potential systemic side effects when used in large quantities in any chronic skin disease.

Predictors of the need for extracorporeal membrane oxygenation and survival in congenital diaphragmatic hernia: a center's 10‐year experience

To determine the prenatal factors associated with the need for extracorporeal membrane oxygenation (ECMO) and neonatal survival in congenital diaphragmatic hernia (CDH).

Neonatal Outcomes of Pierre Robin Sequence: An Institutional Experience

Objective: The aim of this study was to characterize the outcomes of one institute’s experience regarding upper airway and feeding management in Pierre Robin sequence (PRS) neonates. Study Design: A retrospective review of 33 neonates was performed. Average daily weight gain for all patients and average length of stay for each group was calculated and the results were compared using Student’s t test. Result: Average daily weight gain was 16.5 g in the mandibular distraction osteogenesis (MDO) group (MDO; N = 12) and 5.6 g in the nonsurgical intervention (NSI) group (N = 18; P = .043). Average length of stay was longer in the MDO group (P = .01). In all, 67% of MDO patients were discharged with total PO (per os) feeds compared with 22% of NSI patients. Conclusion: Neonates with PRS who do not require surgical airway intervention are more likely to require assisted feeding, have slower weight gain, and a shorter average hospital stay compared to neonates undergoing mandibular MDO.

Short-Term Outcomes of Late Preterms: An Institutional Experience

This article describes the short-term outcomes of late preterm neonates born between 34 and 36 (6/7) weeks gestational age (GA) in a 4-year period. A total of 1381/ 20554 (6.7%) births were between 34 and 36 weeks of GA and were predominantly Caucasian (87%). In all, 697/1381 (51%) were admitted to nursery whereas 684/1381 (49%) remained with their mothers. Of the babies born at 34, 35, and 36 weeks GA, 97%, 53%, and 32%, respectively, required admission and of these 30%, 33%, and 23%, respectively, required respiratory support. Air leaks developed in <4% infants. Median length of stay and age at full enteral feeds were 11, 6, and 4 days and 10, 6, and 3 days for 34, 35, and 36 weeks GA, respectively. It is concluded that late preterms have significant morbidity in the neonatal period.

Cerebral arteriovenous fistula to pulmonary artery onyx embolization

TE Herman, MJ Siegel, A Vachharajani, P Masand and D Cross Department of Radiology, Mallinckrodt Institute of Radiology, St Louis Children’s Hospital, Washington University, St Louis, MO, USA; Division of Neonatology, Department of Pediatrics, St Louis Children’s Hospital, Children’s Place, Washington University School of Medicine, St Louis, MO, USA and Division Interventional Neuroradiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine, St Louis, MO, USA

Prevalence of upper urinary tract anomalies in hospitalized premature infants with urinary tract infection

Objective:The 2011 American Academy of Pediatrics (AAP) guidelines address imaging after initial febrile urinary tract infection (UTI) in infants >2 months of age. We sought to determine the frequency of upper urinary tract anomalies (hydronephrosis and vesicoureteral reflux (VUR)) in hospitalized premature infants with UTI.Study design:We retrospectively reviewed the electronic medical records of neonatal intensive care unit (NICU) admissions at a tertiary care children’s hospital between 1 January 2006 and 31 December 2010. We queried the records for UTI, renal ultrasound (US) and voiding cystourethrogram (VCUG).Result:We identified 3518 unique admissions. UTI occurred in 118 infants (3%). Sixty-nine (60%) had a normal US. Renal dilation was predominantly renal pelvic dilation (12%) and isolated caliectasis (22%). VUR was identified in 15 (14%) infants evaluated with a VCUG. VUR was identified in nine (12%) infants without and in seven (16%) with an abnormality on US. Reflux was identified in 7% of male and 38% of female infants with a UTI.Conclusion:Anatomic abnormalities of the upper urinary tract are uncommon in premature infants with a UTI that occurs during neonatal hospitalization. In concordance with the AAP guidelines, a VCUG may not be required in all NICU infants under age 2 months after a single UTI.

Neonatal marfan syndrome with hiatus hernia and intrathoracic stomach.

CASE A 3040 gram infant was born at 37 weeks to an 18 year old gravida 1, para 0 mother. The pregnancy was complicated by maternal Marfan syndrome and by abnormal maternal fetal sonogram. The mother had characteristic features of Marfan syndrome with a marked scoliosis, typical facial appearance, mitral and tricuspid valve prolapse and a slightly dilated aortic root, tall stature and arachnodactyly. She was being treated for Marfan syndrome and dilated aortic root with atenolol, which was changed to propranolol during the pregnancy. Her father (the patient’s maternal grandfather) also has Marfan syndrome and had an aortic valve replacement. The maternal grandfather’s sister also had Marfan syndrome and died at age 53 with a ‘heart problem’. Fetal maternal sonograms had suggested the presence of a left congenital diaphragmatic hernia. At delivery the child had Apgars of 2, 6 and 7 at 1, 5 and 10 min, respectively. The child was mildly dysmorphic with a long face and a somewhat ‘senile’ appearance, micrognathia, crumpled ears, and arachnodactyly. A cardiac sonogram demonstrated only a patent foramen ovale. He was intubated for 3 days with mild respiratory distress. An initial plain radiograph of the chest and abdomen (Figure 1) demonstrated what appeared to be a large hiatus hernia with intrathoracic stomach. After the patient was extubated, an upper gastrointestinal series (Figure 2) was performed demonstrating an nonobstructed intrathoracic stomach with organoaxial volvulus. Subsequently on the 5th day of life the child had repair of the hiatus hernia with intrathoracic stomach with a Nissen fundoplication and gastrostomy.

Massive congenital orbital teratoma

A 3150 g infant girl was born at 38 weeks gestation to an 18-yearold gravida 2 para 0 mother. An intrauterine sonogram and subsequent fetal magnetic resonance imaging (MRI) (Figure 1) demonstrated a large orbital mass. At birth a 5 cm exophytic right orbital mass was present. The infant had a postnatal orbital computed tomography (CT) and MRI (Figure 2). In preparation for resection, an angiogram was performed that demonstrated the mass had arterial supply from the middle meningeal artery and right ophthalmic artery (Figure 3). The branches to the right middle meningeal artery were embolized with a butylcyanoacylate/ ethiodol solution. The next day the tumor was resected.

Orbital lymphangioma with non-contiguous cerebral arteriovenous malformation, manifesting with thrombocytopenia (Kasabach-Merritt syndrome) and intracerebral hemorrhage

This study describes the first reported case in a preterm infant of an orbital lymphangioma with non‐contiguous cerebral arteriovenous malformation, manifesting with thrombocytopenia (Kasabach‐Merritt syndrome) and intracerebral hemorrhage.

Comparison of Short-Term Outcomes of Late Preterm Singletons and Multiple Births: An Institutional Experience:

We compare 4 short-term outcomes—namely admission to special care nursery (SCN), length of stay (LOS), age at full feeds (AFF) and respiratory morbidity/need for ventilation—in 1015 late preterm singletons and 366 twins and triplets born at our institution over a 4-year period. Birth weight (BW) and gestational age (GA) rather than plurality of birth determined need for admission to SCN, LOS, AFF, and need for respiratory support. When matched for GA, compared to singletons, twins and triplets needed less admission to SCN and respiratory support at 36 weeks, whereas at 34 weeks, they had longer LOS and took longer to get to full feeds. We conclude that the outcomes of interest are affected by GA and BW rather than plurality.