Pulmonary hypertension occurring with diazoxide use in a preterm infant with hypoglycemia

Pharmacologic modulation to open the KATP channels with diazoxide is useful in treating hyperinsulinemia. Diazoxide is being used more often in neonates with hyperinsulinemic hypoglycemia. This report highlights a case of severe pulmonary hypertension (PH) with re-opening of ductus arteriosus in an extremely premature infant after the use of diazoxide. The rapid onset of PH with respiratory failure was completely reversible. This case emphasizes the need for extreme caution with use of diazoxide in the premature infant population, especially those with chronic lung disease of prematurity. In addition, the use of diazoxide should be limited to the persistent form of congenital hyperinsulinism, after adequate work up has been completed to evaluate for other causes of hypoglycemia. It is postulated that development of PH could be related to KATP agonsim.University of ArizonaOpen access journal.This item from the UA Faculty Publications collection is made available by the University of Arizona with support from the University of Arizona Libraries. If you have questions, please contact us at [email protected]

Fatal Disseminated Herpes Simplex in a very premature neonate

Herpes Simplex Virus infections (HSV) are ubiquitous. The neonatal HSV infection (NHSV) is rare. The incidence is estimated globally at only 10.3 per 100,000 births, but it can cause devastating disease in premature infants. Both HSV-1 and HSV-2 can be the etiologic agents in this type of vertically transmittted NHSV infection. Here we describe the pathological findings from a complete autopsy of a very low birth weight infant who succumbed to the infection despite early institution of antiviral treatment. We urge more awareness of this disease with continued surveillance; every effort should be taken to make an early diagnosis and thus prevent this devastating disease

Fatal Disseminated Herpes Simplex in a very premature neonate

Herpes Simplex Virus infections (HSV) are ubiquitous. The neonatal HSV infection (NHSV) is rare. The incidence is estimated globally at only 10.3 per 100,000 births, but it can cause devastating disease in premature infants. Both HSV-1 and HSV-2 can be the etiologic agents in this type of vertically transmittted NHSV infection. Here we describe the pathological findings from a complete autopsy of a very low birth weight infant who succumbed to the infection despite early institution of antiviral treatment. We urge more awareness of this disease with continued surveillance; every effort should be taken to make an early diagnosis and thus prevent this devastating disease

Pseudomonas aeruginosa necrotizing bronchopneumonia

Extremely low birth weight (ELBW) infants are at particularly high risk for infection due to an immature immune system, invasive procedures such as endotracheal intubation, intravascular catheterization, and other factors. Neonatal infections in this population are associated with a high mortality, poor growth, and neurodevelopmental outcomes. Pseudomonas aeruginosa (P. aeruginosa) infection is an uncommon but potentially devastating cause of pneumonia and sepsis in the ELBW population. P. aeruginosa is an important cause of healthcare-associated infections (HAI) or nosocomial infections. P. aeruginosa can perceive unfavorable environmental changes and orchestrate adaptations by developing plasmid-mediated and adaptive resistance to antibiotics. We describe an ELBW infant born at 26 weeks’ gestation who succumbed at 13 days of life to P. aeruginosa infection. Some of the factors related to the pathogenesis and multidrug resistance are described

Tracheal Stenosis and Congenital Heart Disease in Trisomy 21

Tracheal rings (TR) are rare, congenital cartilaginous defect of the upper airway and are usually due to complete or near complete circumferential cartilaginous tracheal rings, with variable degrees of tracheal stenosis (TS) and shortening. Chromosomal anomalies like trisomy 21 are characteristically associated with a wide range of upper airway anomalies including TS and congenital heart disease (CHD). However, the overall prevalence of severe forms of TS is rare and reported in 1.2% of all CHD patients. Herein, we present a rare association of severe TS due to complete tracheal rings in a trisomy 21 patient with CHD and the challenges in the management.Open access journalThis item from the UA Faculty Publications collection is made available by the University of Arizona with support from the University of Arizona Libraries. If you have questions, please contact us at [email protected]

Internal Hernia Masquerading As Necrotizing Enterocolitis

In extremely preterm infants, acute abdominal emergencies are fortunately less common with improving care. Spontaneous intestinal perforation and necrotizing enterocolitis are conditions where emergency surgery is most often needed. Conservative medical management and placement of temporary drain are often used in the initial management. Internal hernia (IH) is an uncommon cause of bowel obstruction in neonates, is difficult to diagnose and unfortunately are found only at autopsy. The presentation in preterm infants, distinction between these conditions, and the need for early diagnosis of IH are discussed.Open Access Journal.UA Open Access Publishing Fund.This item from the UA Faculty Publications collection is made available by the University of Arizona with support from the University of Arizona Libraries. If you have questions, please contact us at [email protected]

Pneumomediastinum Mimicking Congenital Pulmonary Airway Malformation

Pneumomediastinum is the collection of free air in the mediastinum. Its incidence is higher in preterm infants and those on positive airway pressure support or on mechanical ventilation. But it has decreased dramatically after the introduction of surfactant and synchronized, non-invasive mechanical ventilation. Underlying cystic lesions could also increase the risk of pneumomediastinum and other air leak syndromes. Most cases resolve spontaneously but rare hemodynamic compromise may require ultrasound-guided intervention.Open access journalThis item from the UA Faculty Publications collection is made available by the University of Arizona with support from the University of Arizona Libraries. If you have questions, please contact us at [email protected]

Complete and Incomplete Pentalogy of Cantrell

Pentalogy of Cantrell (PC) is a malformation characterized by defects in the ventral abdominal wall, lower sternum, diaphragmatic pericardium, anterior diaphragm associated with omphalocele, thoraco-abdominal ectopia cordis, diaphragmatic hernia, and intracardiac abnormalities. PC is stratified as complete or incomplete and we present both the complete and incomplete forms.Open access journalThis item from the UA Faculty Publications collection is made available by the University of Arizona with support from the University of Arizona Libraries. If you have questions, please contact us at [email protected]

Managing Congenital Lobar Overinflation Associated with Congenital Heart Disease

The incidence of congenital lobar overinflation (CLO) is reported at 1 in 20,000-30,000 live births and represents 10% of all congenital lung malformations. The occurrence of concomitant congenital heart disease (CHD) and CLO ranges from 12% to 20%. There are diverging views in the management as to whether early lobectomy or repair of the cardiac defect, with the assumption that respiratory symptomatology would gradually resolve, or a combined lung and cardiac repair would be the ideal first step in the management. In concomitant CLO and CHD, the surgical decision has to be individualized. Prior to surgical intervention a thorough evaluation may be needed with contrast computed tomography (CT) or magnetic resonance imaging (MRI), bronchoscopy, and if needed cardiac catheterization. CLO improves with management of many left to right shunts and in those with anomalous vessels, but early lobectomy or combined approach may be considered in those symptomatic patients with more complex CHD.Open access journalThis item from the UA Faculty Publications collection is made available by the University of Arizona with support from the University of Arizona Libraries. If you have questions, please contact us at [email protected]

Spontaneous resolution of choledochal cyst

Choledochal cysts (CCs) are rare, congenital cystic dilations of the hepatobiliary tree that require surgical resection to avoid complications such as increased risk of malignancy. A 25‑week gestational age male infant developed acholic stools, elevated alkaline phosphatase, and ultrasound findings consistent with CC Todani Type IVA. Surgery was deferred due to the patient’s low weight. The patient’s symptoms and radiographic findings subsequently resolved spontaneously. CCs have not been previously reported in extremely preterm infants. There are rare reported cases of spontaneously resolving hepatic cysts, all containing key differences from our patient. In patients in whom immediate surgery is not feasible, conservative management with close follow‑up and serial ultrasound examinations would appear to be a reasonable course of action.Open Access Article.This item from the UA Faculty Publications collection is made available by the University of Arizona with support from the University of Arizona Libraries. If you have questions, please contact us at [email protected]