Staged Repair of Complete Sternal Cleft and Interrupted Aortic Arch Associated With PHACES Syndrome in a Very Low Birth Weight Infant
| العنوان: | Staged Repair of Complete Sternal Cleft and Interrupted Aortic Arch Associated With PHACES Syndrome in a Very Low Birth Weight Infant |
|---|---|
| المؤلفون: | Shunji Uchita, Toshiaki Yamauchi, Hideki Mori, Takashi Higaki, Sonoko Fujii, Hironori Izutani, Yujiro Kawanishi, Asami Tozawa, Fumiaki Shikata, Toru Okamura, Ai Kojima, Masaaki Ohta |
| المصدر: | International Surgery. 101:313-317 |
| بيانات النشر: | International College of Surgeons, 2016. |
| سنة النشر: | 2016 |
| مصطلحات موضوعية: | medicine.medical_specialty, business.industry, Interrupted aortic arch, 030204 cardiovascular system & hematology, medicine.disease, Surgery, Eye abnormality, 03 medical and health sciences, Low birth weight, 0302 clinical medicine, Posterior fossa malformations, 030228 respiratory system, medicine, Cardiac defects, PHACES Syndrome, Staged repair, medicine.symptom, business, Sternal cleft |
| الوصف: | 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. |
| تدمد: | 2520-2456 0020-8868 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_________::ed1659c7661151dda330e5253c11727e https://doi.org/10.9738/intsurg-d-15-00228.1 |
| حقوق: | OPEN |
| رقم الأكسشن: | edsair.doi...........ed1659c7661151dda330e5253c11727e |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 25202456 00208868 |
|---|