Growth and prevalence of feeding difficulties in children with Robin sequence: a retrospective cohort study

Abstract

ObjectivesIn addition to breathing problems, patients with Robin sequence (RS) often encounter feeding difficulties (FD). Data regarding the occurrence of FD and possible influencing factors are scarce. The study aim was to elucidate these factors to improve treatment strategies.Material and methodsA retrospective comparative cohort study was conducted, consisting of 69 infants diagnosed with both RS and a cleft palate and 64 isolated cleft palate only (iCPO) infants. Data regarding FD, growth, and airway intervention were collected during the first 2 years of life. A systematic review of the literature was conducted to identify reported FD in RS patients.ResultsRS patients had more FD (91 %) than iCPO patients (72 %; p = 0.004). Also, nasogastric (NG)-tube feeding was necessary more frequently and for a longer period (both p < 0.001). Growth was lower in RS than iCPO infants (p = 0.008) and was not affected by the kind of airway management (conservative/surgical; p = 0.178), cleft palate grade (p = 0.308), or associated disorders (p = 0.785). By contrast, surgical intervention subtype did significantly affect growth. Mean reported FD for RS in the literature is 80 % (range = 47–100 %), and 55 % (range = 11–100 %) of infants need NG-tube feeding.ConclusionsFD is present in a large proportion of infants with RS, which indicates the need for early recognition and proper treatment to ensure optimal growth. Growth during the first 2 years of life is significantly lower in RS patients than iCPO patients, which indicates the need for careful attention and long-term follow-up.Clinical relevanceThis study indicates the need for early recognition and proper treatment of FD in RS to ensure optimal growth. In addition, growth needs careful attention and long-term follow-up.

Pierre Robin Sequence: Diagnostic Difficulties Faced while Differentiating Isolated and Syndromic Forms

“…Prone positioning, placing a nasopharyngeal airway, providing continuous positive airway pressure, and using a laryngeal mask airway are the common noninvasive techniques used to relieve the airway obstruction (1-3). Supplemental feeding using a nasogastric/orogastric tube (mild cases) and gastronomy tube (chronic and persistent cases) helps resolving feeding difficulties (1,13). Almost 70% of PRS cases respond to conservative management, as our patient's did (4).…”

“…In the outpatient department, patients with PRS usually present with a characteristic bird-like face with variable degrees of breathing/feeding difficulties, as found in both our patients (2,13). When the triad of PRS is spotted, the physician should have an heightened index of suspicion for other anomalies and a detailed family history of congenital anomalies should be elucidated (6,8).…”

Sequência De Robin Isolada: Diagnósticos De Enfermagem

“…[6][7] However, they present improvement with the treatment of the respiratory obstruction. 19 Different treatment modalities have been described in the literature to relieve the respiratory obstruction and to improve the feeding difficulties in infants with IRS. The priority is to maintain the permeability of the upper airways.…”

Failure to Thrive in the Context of Carney Complex

“…These include factors associated with decreased caloric intake (e.g., gastroesophageal reflux, feeding difficulty in Pierre-Robin sequence [16]), factors associated with high caloric requirement (e.g., increased work of breathing in laryngomalacia and in Pierre-Robin sequence [17]), developmental/behavioral disorders, and complex syndromes with several contributing factors (e.g., complex III mitochondrial encephalopathy). Moreover, the large number of patients with chromosome 17 copy number variations in our study might suggest an association between this genetic alteration with a multisyndromic phenotype and the presentation of CNC with FTT.…”