Standardized Classification of Infants With Robin Sequence Using MicroNAPS: The Impact of Syndromes and Comorbidities

Abstract

Background: MicroNAPS, a classification for infants with Robin sequence (RS) intended to guide treatment decisions, consists of 5 elements: micrognathia, nutrition, airway, palatal clefting, and syndromes/comorbidities. Scoring of the first 4 elements is well defined, but the assignment of the syndromes/comorbidities score (S score) introduces subjectivity by necessitating judgment regarding the clinical impact of the diagnosis. A database of comorbid diagnoses associated with RS and consensus-based S scores for each is needed for the MicroNAPS system to be reproducible.

Methods: Diagnoses known to be associated with RS were identified from existing literature, and preliminary severity grades were applied. A series of electronic surveys were distributed to RS experts seeking agreement or recommendation and explanation for updated scoring, according to the Delphi technique, until at least 75% consensus was achieved for each item. A previously published series of 100 infants was rescored according to the resulting grades, and changes from initial scoring were assessed.

Results: A total of 170 associated syndromes and comorbidities were identified. Complete responses were received from 20 of the 35 experts (57.1%) surveyed. Consensus on S scores was achieved for all but 3 diagnoses (98.2%) after 4 rounds of surveys. Rescoring of the published series found 25 (25%) changes in S scores resulting in 5 modifications to R stages.

Conclusions: We have compiled a comprehensive list of syndromes and comorbid diagnoses associated with RS and achieved expert consensus regarding the impact on early treatment decision-making for most. This list, assigned S scores, and a complete MicroNAPS calculator are publicly available at www.prscalculator.com.

Fig. 1.

Flowchart of the study process.