Primum non nocere: lingual frenotomy for breastfeeding problems, not as innocent as generally accepted.

The frenotomy or surgical release of the lingual frenulum is performed with increasing frequency. Restricted tongue mobility, ankyloglossia, is the main indication for this procedure. This clinical diagnosis is often used as synonym for tongue-tie which is blamed for many feeding difficulties resulting in an increase in performed frenotomies. Until recently, little was known about the anatomical structure and normal variation of the tongue-tie. Different grading systems have been developed. Some are exclusively based on appearance of the tongue-tie; others also include functional elements. There is, however, no established relation between the tongue-tie score and the observed feeding problems or outcomes following frenotomy. Therefore, caution is warranted before submitting babies to this procedure.Conclusion: This narrative review aims to give an overview of current knowledge and concerns regarding the tongue-tie, which need to be considered before referral for a frenotomy. What is Known: • The presence of a tongue-tie is associated with a higher frequency of breastfeeding problems. • Hence, frenotomy is advocated and increasingly performed in infants with breastfeeding problems. Current tongue-tie classifications do not allow to predict breastfeeding problems. What is New: • New anatomy insights caution for possible complications resulting from this seemingly innocent practice of frenotomy. • Frenotomy should only be performed after multidisciplinary evaluation of feeding problems, following exclusion and remediation of other causative factors.

Confirmation of an ARID2 defect in SWI/SNF-related intellectual disability.

We present a 4-year-old girl with delayed neuromotor development, short stature of prenatal onset, and specific behavioral and craniofacial features harboring an intragenic deletion in the ARID2 gene. The phenotype confirmed the major features of the recently described ARID2-related intellectual disability syndrome. However, our patient showed overlapping features with Nicolaides-Baraitser syndrome and Coffin-Siris syndrome, providing further arguments to reclassify these disorders as "SWI/SNF-related intellectual disability syndromes."