Background: Berry syndrome is a challenging disease for surgeons to make early diagnosis and successful surgical correction in the neonatal period. Here, we summarized the clinical features of three neonates with berry syndrome in our center to optimize the therapeutic effect in the future.
Methods: From January 2014 to December 2019, three neonates with berry syndrome underwent one-stage surgical repair in our center. We mainly used two different surgical techniques to repair it, and collected clinical data retrospectively from hospitalization history, outpatient records, and telephone follow-up.
Results: The age at operation was 28, 8, and 8 days and the body weight was 3.65, 3.86, and 3.0 kg, respectively. The morphology of the interrupted aortic arch (IAA) was type A in two patients and type B in one patient. The aortopulmonary window (APW) morphology was type IIa, III, and IIb, respectively. The phenotype of the IAA type B combined with APW type III in our second patient was reported for the first time so far. All patients survived and were followed up to date. The second patient using intra-aortic baffle experienced twice reoperation for right pulmonary artery (RPA) stenosis. All patients grew well so far.
Conclusion: Once diagnosed in the neonatal period, berry syndrome can be safely corrected by one-stage surgical repair in experienced cardiac centers. Considering the variability of the location where the RPA arises from the posterior wall of the aorta, it is difficult to find the best surgical method for each patient.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8825814 | PMC |
| http://dx.doi.org/10.3389/fcvm.2021.790303 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Surgical treatment experience of seven cases of Berry syndrome.
J Cardiothorac Surg
December 2024
Beijing Children's Hospital Capital Medical University Beijing, Beijing, China.
Objective: Berry syndrome is a group of rare congenital cardiac malformations including aortopulmonary window (APW), aortic origin of the right pulmonary artery (AORPA), interruption of the aortic arch (IAA), patent ductus arteriosus (PDA) (supplying the descending aorta) and intact ventricular septum. This paper will analyze the clinical data of 7 patients with Berry syndrome who underwent surgical treatment in our institution and discuss the one-stage surgical correction of Berry syndrome in combination with the literature.
Methods: From January 2013 to July 2024, a total of 7 children with Berry syndrome were admitted to the Cardiac Surgery Department of Beijing Children's Hospital.
Cervical spine considerations in Goldenhar syndrome: a clinical perspective.
Childs Nerv Syst
December 2024
NJ Craniofacial Center, Morristown, NJ, 07960, USA.
Background: Goldenhar syndrome is a clinically heterogeneous disorder defined by a rare combination of congenital anomalies-an eye abnormality, in addition to two of the following three: ear anomalies, mandibular malformations, and vertebral defects. Notably, children with Goldenhar syndrome present with a high incidence of cervical spine malformations.
Clinical Case: In this report, we present an unusual case of a 15-year-old child with Goldenhar syndrome, who additionally presents with some clinical features of VACTERL syndrome.
Identifying Pain Subtypes in Patients With Craniofacial Lesions of Fibrous Dysplasia/McCune-Albright Syndrome.
J Oral Maxillofac Surg
December 2024
Assistant Professor, Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Harvard Medical School, Boston, MA; Assistant Professor, Department of Psychiatry, McLean Hospital, Harvard Medical School, Belmont, MA. Electronic address:
Background: Fibrous dysplasia/McCune-Albright syndrome (FD/MAS) is a genetic disorder, marked by bone lesions, often affecting the craniofacial skeleton. Pain is a prevalent yet heterogeneous symptom reported by patients with craniofacial FD. Effective treatments are currently lacking, posing a significant clinical challenge to patient care.
View Article and Find Full Text PDFThe First Compound Heterozygosity for Two Different α-Thalassemia Determinants Causes Hb Bart's Hydrops Fetalis in a Chinese Family.
Hemoglobin
December 2024
Department of Clinical Laboratory, Yulin Women and Children Health Care Hospital, Yulin, Guangxi Zhuang Autonomous Region, China.
In southern China, α-thalassemia is the most prevalent hereditary monogenic disorder, and deletion variants are the predominant form. Conventional thalassemia diagnosis techniques are numerous, however they are all limited in their ability to detect rare deletions. Here, we discuss a family who sought genetic counseling during their fourth pregnancy after experiencing Hb Bart's hydrops fetalis in two of their previous pregnancies.
View Article and Find Full Text PDFSource-sink manipulations through shading, crop load and water deficit affect plant morphogenesis and carbon sink priorities leading to contrasted plant carbon status in grapevine.
Ann Bot
December 2024
Université de Montpellier, INRAE, UMR LEPSE, 2 Place Viala 34060 Montpellier, France.
Backgrounds And Aims: Shading, water deficit, and crop load shape plant development in a very plastic way. They directly influence the plant's carbon supply and demand to and from the different organs via metabolic, hydraulic and hormonal mechanisms. However, how the multiple environmental factors combine through these mechanisms and how they interplay with carbon status, vegetative and reproductive development and carbon assimilation of the plant needs to be investigated in the context of current climatic and technological constraints.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!