In our patient, HSV was confined to the larynx, without evidence of local or systemic disease. Localized HSV laryngitis has been attributed to suppressed cellmediated immunity from HIV, chemotherapy, and prolonged corticosteroid use. In contrast, our patient was likely susceptible to HSV infection from damaged laryngeal mucosa from prior radiation exposure. Vrabec et al. noted the importance of maintaining a high index of suspicion of HSV laryngitis in patients who fail extubation, as was evident in this patient. Our case demonstrates that HSV can present at an atypical anatomic site and cause extensive damage, compromising airway patency if left untreated.Case Report: Spinocerebellar Ataxia Type 7 (SCA-7) is a pathological condition stemming from CAG repeat expansions. The CAG expansions induces propagation of the ataxin-7 protein, with subsequent rapid progression of multi-organ tissue infiltration. An increasing number of pathological repeats may represent prognostic indicators for patients; with larger repeats correlating with worse prognosis. We report a 2 month old female with global hypotonia, intermittent hypoxemia, and a family history notable for walking difficulty and visual impairment across multiple generations, consistent with autosomal dominant inheritance. Echocardiogram revealed a Patent Ductus Arteriosus (PDA); Catheterization demonstrated Coarctation of Aorta, type B as well as elevated trans-pulmonary gradient, responsive to oxygen and inhaled Nitric Oxide. The patient underwent end to end aortic anastomoses and PDA resection via lateral thoracotomy. Morbidity included chronic anasarca secondary to capillary leak syndrome; respiratory failure and inability to wean from ventilatory support due to global hypotonia necessitating tracheostomy; chronic renal failure with azotemia; concentric left ventricular hypertrophy-cardiomyopathy in the absence of residual aortic gradient; and visual evoke potentials consistent with blindness. DNA testing revealed a normal Karyotype (46,XX) and 78 CAG repeats of ataxin-7 gene protein.Repeats between 35 to 300 are known to be associated with pathology, while normal alleles contain 4 to 34 CAG units. The patient died within 3 mo of surgical repair; autopsy revealed septic pneumonia and cardiomyopathy as a cause of death. The longitudinal course of SCA-7 is unknown, as are the correlation between the repeat expansion sequences, clinical signs and symptoms, cardiomyopathy and neuropathology. Case reports of infantile SCA-7 suggests life expectancy of less than one year. Coarctation of Aorta has never been described in conjunction with SCA-7, making the post-operative course challenging when encountering multiorgan manifestations seen with this rarely described syndrome.
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