Defining Tip-Frenulum Length for Ankyloglossia and Its Impact on Breastfeeding: A Prospective Cohort Study.

OBJECTIVE: The purpose of this study was to investigate the normal lingual frenulum anatomy in newborns and to evaluate tip-frenulum distance as an objective diagnostic tool for identifying newborns at risk for anterior and posterior tongue tie and breastfeeding difficulty. MATERIALS AND METHODS: The distance from the tongue tip to the insertion of the lingual frenulum was measured in a group of 100 healthy newborns to establish normative data. The presence of a visible or palpable cord was noted. Inter-rater reliability was assessed. Breastfeeding surveys including a maternal pain scale and Infant Breastfeeding Assessment Tool (IBFAT) were administered on days of life 2 and 14 to determine whether these findings predict breastfeeding difficulty. RESULTS: Mean tip-frenulum length was 9.07 mm. Intraclass correlation coefficient between observers for tip-frenulum length was 0.82. A visible cord was identified in 21 subjects (21%). A palpable cord was identified in 59 subjects (59%). Unweighted κ coefficients for inter-rater reliability of visible and palpable cords were 0.91 and 0.47, respectively. Visible cord and shorter tip-frenulum distance were independently predictive of higher maternal pain scores. A positive correlation was identified between tip-frenulum length and IBFAT scores for mothers with two or more previous breastfed children. CONCLUSIONS: Tongue tip-frenulum length correlated with maternal nipple pain, and was useful as an objective tool for identifying newborns at risk for ankyloglossia. Maternal breastfeeding experience appears to be an important factor in the link between tongue anatomy and breastfeeding difficulty. The presence of a palpable cord was variable across examiners, and should be interpreted with caution when evaluating newborns for posterior tongue tie.

Development and validation of a quality of life instrument for patients with laryngeal cleft.

OBJECTIVE: To develop and validate a novel pediatric health-related quality of life (HR-QoL) instrument for patients with laryngeal cleft and their families. METHODS: We surveyed primary caregivers of patients that underwent endoscopic repair of Type I or II laryngeal cleft. The proposed HR-QoL instrument consists of 40 items representing four domains, assessing the patient's physical symptoms, the patient's and family's social functioning, and the family's emotions regarding the patient's illness pre- and post-operatively. Confirmatory factor analysis was employed to assess construct validity, dimensionality, and optimal simple structure. RESULTS: Of 78 eligible participants reached by phone, 40 (51%) of them completed the questionnaire. Confirmatory factor analysis suggested that all four measured constructs were well supported by the measured items in comparison to a unidimensional model. All factor loadings and factor correlations were significant and factor correlations ranged between 0.723 and 0.879. Indices of test-retest reliability and internal consistency reliability were well above recommended standards. There was a significant correlation between current instrument and PedsQL™ score. The overall QoL score significantly improved from 112.3 (±28.1) before surgery to 158.0 (±28.5) after surgery (mean difference 45.7; 95% CI: 37.3, 54.1; p < 0.001). CONCLUSION: Our proposed pediatric HR-QoL instrument is a valid tool for measuring quality of life in patients with laryngeal cleft and their families. This instrument can provide insight into the effects of medical and surgical therapy and guide pre- and post-operative management of laryngeal cleft.

Neurologic Evaluation in Children With Laryngeal Cleft.

Importance: Referral to a neurologist and imaging play important roles in the management of laryngeal cleft. Swallowing involves a complex series of neuromuscular interactions, and aspiration can result from anatomical causes (eg, laryngeal cleft), neuromuscular disorders, or some combination thereof. To date, no protocols or guidelines exist to identify which patients with laryngeal cleft should undergo neuroimaging studies and/or consultation with a neurologist. Objective: To establish guidelines for neurologic evaluation and imaging techniques to identify or rule out neuromuscular dysfunction in children with laryngeal cleft. Design: Retrospective review of the medical records of 242 patients who were diagnosed with laryngeal cleft at a tertiary children's hospital between March 1, 1998, and July 6, 2015. Based on this review, an algorithm to guide management of laryngeal cleft is proposed. Main Outcomes and Measures: Data extracted from patient medical records included the type of laryngeal cleft, details of neurologic referral, results of neuroimaging studies, and objective swallow study outcomes. Results: Of the 242 patients, 142 were male and 100 were female. Mean age at the time of data analysis was 8.7 years (range, 10 months to 25 years), and there were 164 type I clefts, 64 type II, 13 type III, and 1 type IV. In all, 86 patients (35.5%) were referred to a neurologist; among these, 33 (38.4%) had examination findings indicative of neuromuscular dysfunction or dyscoordination (eg, hypotonia, spasticity, or weakness). Abnormal findings were identified in 32 of 50 patients (64.0%) who underwent brain imaging. Neurosurgical intervention was necessary in 3 patients diagnosed with Chiari malformation and in 1 patient with an intraventricular tumor detected on neuroimaging. Conclusions and Relevance: A substantial proportion of patients with laryngeal cleft have coexistent neuromuscular dysfunction as a likely contributing factor to dysphagia and aspiration. Collaboration with a neurologist and appropriate neuroimaging may provide diagnostic and prognostic information in this subset of patients. At times, imaging will identify critical congenital malformations that require surgical treatment.