IMPORTANCE Detecting elevated intracranial pressure in children with subacute conditions, such as craniosynostosis or tumor, may enable timely intervention and prevent neurocognitive impairment, but conventional techniques are invasive and often equivocal. Elevated intracranial pressure leads to structural changes in the peripapillary retina. Spectral-domain (SD) optical coherence tomography (OCT) can noninvasively quantify retinal layers to a micron-level resolution.OBJECTIVE To evaluate whether retinal measurements from OCT can serve as an effective surrogate for invasive intracranial pressure measurement. DESIGN, SETTING, AND PARTICIPANTSThis cross-sectional study included patients undergoing procedures at the Children's Hospital of Philadelphia from September 2014 to June 2015. Three groups of patients (n = 79) were prospectively enrolled from the Craniofacial Surgery clinic including patients with craniosynostosis (n = 40). The positive control cohort consisted of patients with hydrocephalus and suspected intracranial hypertension (n = 5), and the negative control cohort consisted of otherwise healthy patients undergoing a minor procedure (n = 34).MAIN OUTCOMES AND MEASURES Spectral-domain OCT was performed preoperatively in all cohorts. Children with cranial pathology, but not negative control patients, underwent direct intraoperative intracranial pressure measurement. The primary outcome was the association between peripapillary retinal OCT parameters and directly measured elevated intracranial pressure. RESULTSThe mean (SD) age was 34.6 (45.2) months in the craniosynostosis cohort (33% female), 48.9 (83.8) months in the hydrocephalus and suspected intracranial hypertension cohort (60% female), and 59.7 (64.4) months in the healthy cohort (47% female). Intracranial pressure correlated with maximal retinal nerve fiber layer thickness (r = 0.60, P Յ .001), maximal retinal thickness (r = 0.53, P Յ .001), and maximal anterior retinal projection (r = 0.53, P = .003). Using cut points derived from the negative control patients, OCT parameters yielded 89% sensitivity (95% CI, 69%-97%) and 62% specificity (95% CI, 41%-79%) for detecting elevated intracranial pressure. The SD-OCT measures had high intereye agreement (intraclass correlation, 0.83-0.93) and high intragrader and intergrader agreement (intraclass correlation Ն0.94). Conventional clinical signs had low sensitivity (11%-42%) for detecting intracranial hypertension.CONCLUSIONS AND RELEVANCE Noninvasive quantitative measures of the peripapillary retinal structure by SD-OCT were correlated with invasively measured intracranial pressure. Optical coherence tomographic parameters showed promise as surrogate, noninvasive measures of intracranial pressure, outperforming other conventional clinical measures. Spectral-domain OCT of the peripapillary region has the potential to advance current treatment paradigms for elevated intracranial pressure in children.
Goals of a successful cleft palate repair include separation of the oral and nasal components without fistula, achieving sufficient velar length, and creating functional transverse orientation of the levator muscle sling. A number of techniques have been described to achieve these goals, but they all have the following technical details in common: elevation of oral mucosal flaps based on the greater palatine arteries, tension free nasal lining mobilization, and functional intervelar muscle dissection. After palate repair, speech evaluation needs to be performed by an objective interdisciplinary team following a standardized protocol. Identification of velopharyngeal insufficiency secondary to an incompetent nasopharyngeal port will necessitate secondary speech surgery. These secondary techniques include pharyngeal flaps, soft palate lengthening, or pharyngeal sphincters, which should be tailored to optimize speech, while minimizing the risk of obstructive sleep apnea.
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