Primary Repair of Aortopulmonary Window with an Interrupted Aortic Arch in a Very Low-Birth-Weight Premature Neonate

Posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, and sternal malformations' (PHACES) syndrome comprises various defects that require a concrete therapeutic plan. Herein, we report a case of a very low birth weight infant with PHACES syndrome presenting complete sternal cleft, interrupted aortic arch with ventricular septal defect, and absence of communicating cerebral arteries. Due to the infant's very low birth weight, we planned staged surgery for this complex disease. First, bilateral pulmonary artery banding was performed to improve unstable hemodynamics. Then, after sufficient body weight was obtained, aortic arch and intracardiac repair was done. However, when the tracheal tube was removed 2 weeks after surgery, the patient could not breathe well due to paradoxical chest movement related to complete sternal cleft. Consequently, to improve chest wall compliance, the upper sternum was constructed with dislocated ribs. After these treatments, the patient gained adequate weight and was in stable condition. Staged surgical treatment of sternal cleft and congenital heart defects is a potential therapeutic option for PHACES syndrome, particularly in very low birth weight infants with complex cardiovascular disease.

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Hybrid therapy for interrupted aortic arch with aortopulmonary window in a low birth weight infant

Catheterization and Cardiovascular Interventions ◽

10.1002/ccd.23160 ◽

2011 ◽

Vol 78 (6) ◽

pp. 945-947 ◽

Cited By ~ 5

Author(s):

Ming-Chih Lin ◽

Chung-Chi Wang ◽

Yun-Ching Fu

Keyword(s):

Birth Weight ◽

Low Birth Weight ◽

Aortic Arch ◽

Interrupted Aortic Arch ◽

Aortopulmonary Window ◽

Hybrid Therapy ◽

Low Birth Weight Infant

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Successful Treatment of a Rare Case of Neonatal Ebstein Anomaly in a Very Low-Birth-Weight Premature Neonate

Journal of Cardiac Surgery ◽

10.1111/jocs.12363 ◽

2014 ◽

Vol 29 (5) ◽

pp. 709-711

Author(s):

Keisuke Nakanishi ◽

Shiori Kawasaki ◽

Ken Takahashi ◽

Toshiaki Shimizu ◽

Atsushi Amano

Keyword(s):

Birth Weight ◽

Low Birth Weight ◽

Successful Treatment ◽

Rare Case ◽

Very Low Birth Weight ◽

Premature Neonate ◽

Ebstein Anomaly

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Outcome of primary repair in extremely and very low-birth-weight infants with esophageal atresia/distal tracheoesophageal fistula

Journal of Pediatric Surgery ◽

10.1016/j.jpedsurg.2017.05.011 ◽

2017 ◽

Vol 52 (10) ◽

pp. 1567-1570 ◽

Cited By ~ 8

Author(s):

Andreas Schmidt ◽

Florian Obermayr ◽

Justus Lieber ◽

Christian Gille ◽

Frank Fideler ◽

...

Keyword(s):

Birth Weight ◽

Low Birth Weight ◽

Esophageal Atresia ◽

Tracheoesophageal Fistula ◽

Very Low Birth Weight ◽

Primary Repair ◽

Low Birth Weight Infants

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Intraventricular Hemorrhage in the Premature Neonate

Neonatal Network The Journal of Neonatal Nursing ◽

10.1891/0730-0832.24.3.55 ◽

2005 ◽

Vol 24 (3) ◽

pp. 55-71 ◽

Cited By ~ 17

Author(s):

Rebecca Owens

Keyword(s):

Birth Weight ◽

Low Birth Weight ◽

Preterm Infant ◽

Gestational Age ◽

Intraventricular Hemorrhage ◽

Very Low Birth Weight ◽

Premature Neonate ◽

Vlbw Infants ◽

Severe Hemorrhage ◽

Common Variety

THE MOST COMMON VARIETY of neonatal intracranial hemorrhage is an intraventricular hemorrhage (IVH).1 Although much research has been conducted concerning the etiology and prevention of IVH in the preterm infant, the problem continues to plague very low birth weight (VLBW) infants (<1,500 grams) in particular. The risk of IVH correlates inversely with gestational age. The incidence of hemorrhage in the infant <28 weeks gestation is three times that of infants 28–31 weeks gestational age. The incidence of severe hemorrhage in infants <28 weeks gestational age is twice that of infants 28–31 weeks gestational age.2

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