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We sought to examine the relative importance of surgical lesion complexity versus the presence of genetic/syndromic/extracardiac anomalies (GSAs) in determining survival, morbidity or need for reinterventions following repair for aortic arch hypoplasia.
Methods:
A single-centre, retrospective cohort study of infants undergoing biventricular aortic arch repair via sternotomy from 2010 to 2021 was conducted. Survival analysis was performed using Kaplan–Meier methods, with additional Bayesian survival modelling for subgroups. Composite morbidity comprised respiratory, renal, neurologic, or sepsis-related complications.
Results:
Of 83 included infants, n = 13/83 (15.7%) had complex repairs; 27/83 (32.5%) were GSA+. Operative mortality was significantly higher in GSA+ versus GSA− patients (18.5% vs. 1.8%; p = 0.01), though not for complex versus non-complex repairs. Overall 10-year Kaplan–Meier survival was 86.7%. Bayesian modelling suggested equivalent post-discharge attrition in non-complex/GSA+ and complex/GSA− patients, with the poorest outcomes in complex/GSA+ patients; non-complex/GSA− patients had 100% survival. GSA+ patients exhibited higher composite morbidity (44.4% vs. 7.1% in GSA− p < 0.001), with their mode of death seemingly related to a high incidence of respiratory and neurological morbidity, notably in Dandy–Walker syndrome. The 10-year freedom from arch reinterventions was 87.7%; neither complexity, GSA status, nor post-repair peak arch velocity predicted the need for arch reinterventions.
Conclusions:
Whilst anatomic complexity may have been somewhat neutralised as a risk factor for operative mortality, in contrast to GSA+ status, there is further post-discharge attrition attributable to complexity or GSA+ status, with additive risk effects. Morbidity directly related to certain syndromes underlies some of this risk. Non-anatomic substrates represent a persistent limitation to outcomes of surgical aortic arch repair in infants.
Edited by
Helen Liapis, Ludwig Maximilian University, Nephrology Center, Munich, Adjunct Professor and Washington University St Louis, Department of Pathology and Immunology, Retired Professor
We present the sequence of events leading to the permanent human kidney. Nephrogenesis begins around 22 days after fertilisation and completes around the 34–36th week of gestation. There are three pairs of kidneys in human development: the pronephros, mesonephros and metanephros, arising sequentially from intermediate mesoderm on the dorsal body wall. The first two pairs involute and are resorbed during fetal life, but they are essential precursors to the metanephros and normal adult kidneys do not develop if they are disrupted. Human metanephric kidney development begins around day 28 post conception when the ureteric bud arises as an outpouching of the distal mesonephric duct/Wolffian duct. Glomeruli form from 8–9 weeks and nephrogenesis continues in the outer rim of the cortex until 34 weeks. Perturbation of early pathways can lead to a range of phenotypes including renal agenesis, dysplasia and duplex kidneys, as well as malpositioning defects such as pelvic or horseshoe kidneys if they fail to ascend to their normal 12th thoracic to 3rd lumbar vertebral site during development.
To review the clinical characteristics, prevalence and outcomes of chronic rhinosinusitis patients with a hypoplastic maxillary sinus who underwent functional endoscopic sinus surgery.
Methods
A retrospective review was performed for the 814 consecutive, elective functional endoscopic sinus surgery procedures performed at an academic centre from 2010 to 2020, to identify patients with a hypoplastic maxillary sinus.
Result
A total of 56 hypoplastic maxillary sinus cases were detected. Maxillary sinus hypoplasia presented unilaterally in 20 cases and bilaterally in 18 cases. Of the maxillary sinus hypoplasia cases, 38 were type I, 17 were type II and 1 was type III. The average Lund–McKay score was 8.6. No major post-operative complications were reported. Four patients had minor complications and one had persistent post-nasal drip.
Conclusion
Functional endoscopic sinus surgery is a safe and effective procedure for improving the clinical condition of patients with a hypoplastic maxillary sinus; however, careful pre-operative radiological evaluation, identification of intra-operative endoscopic landmarks and use of additional techniques may be essential to achieve satisfactory results and avoid possible serious complications.
A rare case of incomplete tracheal duplication with severe unilateral lung hypoplasia is presented. Photo-documentation of the gross post-mortem specimens is presented so that the anatomical aspects of this unusual anomaly can be recognized and appreciated. Clinical information is presented in the hope that successful premorbid identification of potential complications of this anomaly could be made by future physicians. To our knowledge, this is the first reported case of pathologically-confirmed duplication of the trachea.
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