Purpose: Large congenital diaphragmatic hernias (CDH) can be repaired with either a muscle flap or prosthetic patch. The purpose of this study was to assess the frequency and severity of scoliosis, chest wall, and abdominal wall deformities following these repairs.
Methods: Neonates who underwent CDH repair (1989-2012) were retrospectively reviewed. We then validated our retrospective review by comparing results of a focused radiologic evaluation and clinical examination of patients with large defects seen in prospective follow-up clinic. Tests for association were made using Fisher's exact test.
Results: 236 patients survived at least 1year. Of these patients, 30 had a muscle flap, and 13 had a patch repair. Retrospectively, we identified pectus in 9% of primary repairs, 47% of flap repairs, and 54% of patch repairs. We identified scoliosis in 7% of primary repairs, 13% of flap repairs, and 15% of patch repairs. Prospectively, 75% of flap patients and 67% of patch patients had pectus and 13% of flap patients and 33% of patch patients had scoliosis. There was no significant difference between flap and patch patients.
Conclusions: Scoliosis and pectus deformity were common in children with large CDH. The operative technique did not appear to affect the incidence of subsequent skeletal deformity.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1016/j.jpedsurg.2014.01.018 | DOI Listing |
Epilepsy Res
January 2025
Fuller Graduate School of Psychology, Travis Research Institute, Pasadena, CA 91101, United States; International Research Consortium for the Corpus Callosum and Cerebral Connectivity (IRC5), Pasadena, CA 91106, United States; California Institute of Technology, Division of Humanities and Social Sciences, Pasadena, CA 91125, United States. Electronic address:
Background And Aims: For young children with intractable epilepsy caused by congenital abnormalities or acquired cortical lesions, pediatric hemispherectomy surgery (pHS) may offer the only path to seizure remediation. Although some sensory and motor outcomes of pHS are highly predictable, the long-term cognitive and functional sequelae of pHS are far more variable. With the aim of identifying potential post-pHS intervention targets, the current study examined daily executive functioning and self-awareness in adults with pHS and broadly intact cognitive outcomes (indicated by average or above performance on intelligence tests).
View Article and Find Full Text PDFTher Adv Respir Dis
January 2025
Division of Pulmonary and Sleep Medicine, Department of Pediatrics, University of Washington School of Medicine, Seattle Children's Hospital, 4800 Sand Point Way NE, OC 7.730, Seattle, WA 98105, USA.
Background: Joubert syndrome (JS) is an autosomal recessive disorder with a distinctive mid-hindbrain malformation known as the "molar tooth sign" which involves the breathing control center and its connections with other structures. Literature has reported significant respiratory abnormalities which included hyperpnea interspersed with apneic episodes during wakefulness. Larger-scale studies looking at polysomnographic findings or subjective reports of sleep problems in this population have not yet been published.
View Article and Find Full Text PDFMol Genet Genomic Med
January 2025
Diagnostics and Therapeutics of Intractable Diseases, Intractable Disease Research Center, Graduate School of Medicine, Juntendo University, Tokyo, Japan.
Background: Sengers syndrome is an autosomal recessive mitochondrial DNA depletion syndrome characterized by hypertrophic cardiomyopathy, congenital cataracts, skeletal myopathy, exercise intolerance, and lactic acidosis. Dysfunction of acylglycerol kinase (AGK) is responsible for the disease, and several AGK gene variants have been reported.
Methods: We employed a comprehensive genomic analysis approach, including whole-genome sequencing and RNA sequencing, combined with various bioinformatics tools.
An atrial septal defect (ASD) is a common congenital heart anomaly that results in irregular blood flow between the systemic and pulmonary circulations due to an opening in the atrial septum. Ostium secondum ASD accounts for a large proportion of these defects and often goes unnoticed during childhood and adolescence. Pulmonary hypertension (PH), affecting a significant number of patients with ostium secondum ASD, is associated with functional limitations, heart failure, and tachyarrhythmias.
View Article and Find Full Text PDFBackground: Cardiovascular diseases are the primary cause of nonobstetric morbidity and mortality in pregnant women worldwide. Pakistan's high maternal and neonatal mortality rates underscore the need for effective screening protocols to detect cardiovascular diseases during pregnancy.
Objectives: The objective of this study was to assess the prevalence and factors associated with structural heart disease among pregnant women without active cardiorespiratory symptoms (no symptoms or symptoms attributed to pregnancy) attending routine antenatal appointments.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!