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Severe Hypertrophic Cardiomyopathy Caused by a Protein Kinase Adenosine Monophosphate-Activated Non-catalytic Subunit Gamma 2 (PRKAG2) Mutation With Refractory Chylous Effusions in a Neonate: A Case Report and Literature Review.
Cureus
October 2024
Department of Pediatrics, Saitama Medical Center, Saitama Medical University, Kawagoe, JPN.
Protein kinase adenosine monophosphate-activated non-catalytic subunit gamma 2 (PRKAG2) cardiac syndrome is a rare genetic disorder characterized by hypertrophic cardiomyopathy and heart rhythm disturbances caused by mutations in the gene. Reports on PRKAG2 cardiac syndrome associated with refractory chylous effusion are extremely limited. Here, we present a neonatal case involving severe hypertrophic obstructive cardiomyopathy accompanied by chylous ascites and lymphatic malformations.
View Article and Find Full Text PDFA Data-Driven Approach to Inguinal Hernia Repairs in Infants and Children.
J Laparoendosc Adv Surg Tech A
December 2024
Division of Pediatric Surgery, Yale School of Medicine, New Haven, Connecticut, USA.
Laparoscopic inguinal hernia repair has become increasingly popular in children. The laparoscopic technique inherently assesses the contralateral processus vaginalis, reducing the risk of metachronous contralateral hernias. We hypothesized that primary laparoscopic repair would be associated with lower rates of subsequent hernia repair in the youngest patients, in whom metachronous contralateral hernias are most common.
View Article and Find Full Text PDFCaudal block, high flow oxygen insufflation and dexmedetomidine sedation for inguinal hernia surgery in infants-A prospective evaluation of an alternative anesthesia technique.
Paediatr Anaesth
November 2024
Department of Anaesthesia and Pain Medicine, Perth Children's Hospital, Perth, Western Australia, Australia.
Article Synopsis
- Inguinal hernia repair is the most common surgery for infants, and this study aimed to assess a new anesthetic technique combining caudal block, high-flow nasal oxygen, and dexmedetomidine sedation instead of general anesthesia.
- Conducted in Australia and New Zealand, the study involved 50 infants and found that the new technique successfully completed surgery in 82% of the cases without needing to switch to general anesthesia.
- The approach resulted in few intraoperative and postoperative complications, suggesting it could be a safer alternative for this type of surgery in infants.
Management of cloacal exstrophy: Experience from tertiary hospital, Tanzania. Case series.
Int J Surg Case Rep
November 2024
Department of Surgery, School of Medicine, Muhimbili University of Health and Allied Sciences, Dar es Salaam, Tanzania.
Introduction And Importance: Cloacal exstrophy (CE) is defined as a complex anomaly that affects the urogenital and intestinal tracts. It is the most serious form of anomaly that is described within the so-called exstrophy-epispadias complex. These malformations usually present a challenge in the management of particular conditions, as most of these forms require multiple surgeries, resulting in the use of multidisciplinary approaches, including reconstructive urologists, pediatric surgeons, orthopedic surgeons, endocrinologists, pediatricians, psychologists and nutritionists.
View Article and Find Full Text PDFInguinal hernias in children: Update on management guidelines.
J Paediatr Child Health
November 2024
Department of Paediatric Surgery, The Children's Hospital at Westmead, Sydney, New South Wales, Australia.
Inguinal hernia repair is one of the most performed procedures in children, but aspects of care remain controversial. The aim of this review was to provide a critical appraisal of recently published guidelines on the management of inguinal hernias in children, by the American Academy of Pediatrics (2023) and the European Pediatric Surgeons' Association Evidence and Guideline Committee (2022). This was achieved by delineating areas of controversy and reviewing the most relevant recent literature on these topics.
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