When Fryn met Edward: Two rare syndromes in a single patient
We report a case of Fryns Syndrome who was incidentally found to have Edward Syndrome as well. Such an extremely rare combination is yet to be reported in medical literature.Also with updated genetic studies, better diagnostics and treatment options coming up in future, there are chances to improve the survivability of these babies. It is prudent to document all such cases to aid in better understanding of the disease process.
The surgical strategy for congenital diaphragmatic hernia (CDH) repair remains debated and mainly depends on the training and preference of the surgeon. Our aim was to evaluate the occurrence and nature of surgical reinterventions within the first year of life, following repair through thoracotomy as compared to laparotomy.
CONCLUSION: Morgagni hernias are amenable to minimally invasive repair with this simple technique. With large defects, synthetic patches should be used. Recurrences are rare, and morbidity is low. PMID: 31926567 [PubMed - in process]
We describe a newborn with fetal aberrant ductus venosus with progressive right ventricular dilation prompting early delivery. Post-natally, the baby had frequent episodes of cyanosis. Echocardiogram during distress showed compression of the inferior vena cava and of the right heart, with significant right to left shunt through patent foramen ovale. Infant underwent successful laparoscopic diaphragmatic hernia repair with resolution in her episodes of cyanosis.
Recurrence of congenital diaphragmatic hernia (CDH) was retrospectively evaluated after correction with or without a patch in an institution where tension-free repair is advocated.
CONCLUSIONS: AKI is common in CDH cases treated in our centre and is associated with adverse outcomes. Potentially modifiable risk factors include nephrotoxic medication exposure. Prevention and early recognition of contributory factors for AKI may improve outcomes in CDH. PMID: 31639793 [PubMed - as supplied by publisher]
We reported the surgical repair of bilateral CDH in a male infant aged 6 weeks. The patient had herniation of the liver on the right side with HPF in association with anomalous drainage of the right pulmonary vein into the hepatic vein and ventricular septal defect. We approached both sides thoracoscopically; however, the right side required a subcostal incision. We separated the liver from the right lower lung lobe laterally using electrocautery, and the defect was closed with a mesh. The medial portion was left intact to avoid injury of the anomalous pulmonary venous drainage. After 2 weeks, the patients had a surgi...
Publication date: Available online 9 September 2019Source: Journal of Pediatric Surgery Case ReportsAuthor(s): Amanda Muñoz, Victoria Pepper, Faraz A. Khan, Andrei RadulescuAbstractAnterior diaphragmatic hernias through the foramen of Morgagni are rare accounting for about 3% of surgically treated diaphragmatic hernias. Current management of Morgagni type diaphragmatic hernias involves surgical intervention, which is traditionally done by open laparotomy, laparoscopy or thoracoscopic approach. Here we present the case of a two year old female with a Morgagni type congenital diaphragmatic hernia (CDH) found during wo...
Congenital diaphragmatic hernia (CDH) repair is technically challenging with different approaches for repair. The aim of this study was to compare outcomes between patch and flap repair.
ConclusionAlthough there was a trend towards an increased risk of the pectus deformity and scoliosis in patients repaired with muscle flap, it did not reach statistical significance. There is a correlation between musculoskeletal deformities and the severity of the CDH.
Abstract RATIONALE Congenital diaphragmatic hernia (CDH) is an anomaly with a high morbidity and mortality. Cardiac dysfunction may be an important and under-recognized contributor to CDH pathophysiology and determinant of disease severity. OBJECTIVES To investigate the association between early, postnatal ventricular dysfunction and outcome among infants with CDH. METHODS Multicenter, prospectively-collected data in the Congenital Diaphragmatic Hernia Study Group (CDHSG) registry, abstracted between 2015 and 2018, were evaluated. Ventricular function on early echocardiograms, obtained within the first 48 hours of...