A comparison of airway interventions and gastrostomy tube placement in infants with Robin sequence

Abstract

The purpose of this study was to evaluate feeding impairment following non-operative or operative management of airway obstruction in a large series of infants with Robin sequence (RS) by rate of G-tube placement. A retrospective study was conducted at Boston Children's Hospital including 225 patients (47.1% female) with RS treated between 1976 and 2018. Subjects were grouped by intervention required for successful management of airway obstruction: nonoperative only (n = 120), tongue-lip adhesion (TLA, n = 75), mandibular distraction osteogenesis (MDO, n = 21), or tracheostomy (n = 9). The operative group had a higher rate of G-tube placement (58.1%) than the non-operative group (28.3%, P < 0.0001). Subjects in the TLA and tracheostomy groups had higher odds of G-tube placement than subjects in the MDO group: odds ratio (OR) 5.5 (95% confidence interval (CI) 1.8-17.3, P = 0.004) and OR 27.0 (95% CI 3.2-293.4, P = 0.007), respectively. Syndromic patients and those with gastrointestinal anomalies also had higher odds of G-tube placement: OR 3.5 (95% CI 1.7-7.2, P = 0.001) and OR 5.9 (95% CI 1.6-21.0, P = 0.007), respectively. Infants with RS who require an airway operation and those with a syndromic diagnosis or gastrointestinal anomalies are more likely to require placement of a G-tube. Of the operative groups, MDO was associated with the lowest G-tube rate, compared to TLA and tracheostomy.

Do Infants With Robin Sequence Have Improved Feeding and Weight Gain After Mandibular Distraction?

“…In an earlier study, our own group also found a higher rate of gastrostomy tube insertion in individuals who had TLA than those with MDO. 24 As our group's earlier report of gastrostomy tube rates included significant sample overlap with this study, these findings suggest that use of gastrostomy tubes is a poor surrogate marker for feeding ability and weight gain; variables such as the interest of the surgical team to avoid feeding for some time in order to reduce dehiscence rates of TLA, among other factors, may influence feeding tube decisions.…”

Mandibular Distraction Osteogenesis as a Primary Intervention in Infants With Pierre Robin Sequence

“…Tongue-lip adhesion also has been correlated with delays in speech and language production. Our multidisciplinary team uses MDO to primary surgical treatment because it addresses a multifaceted dysfunction in the sequence 4,5,21 . Furthermore, studies have shown that this strategy has a lower cost burden to the system as well with estimated cost saving of US $300,000 over tracheostomy in a 3-year period and a lower cost than tongue-lip adhesion when associated procedures and outpatient care for PRS are taken into account 26 …”

“…The PRS sequence begins with the failure of the mandible to grow anteriorly (micrognathia), which reduces the oropharyngeal space 2 and causes posterior displacement of the tongue (glossoptosis) 3 leading to AO. Respiratory dysfunction due to AO is the main cause of feeding difficulties leading to poor growth 4–6 . Furthermore, PRS can present as one component of a known or suspected syndrome (syndromic PRS [sPRS]) or as an isolated finding (isolated PRS [iPRS]).…”

“…In the absence of treatment, children with PRS may succumb to obstructive sleep apnea (OSA), asphyxia, hypoxia, respiratory failure, cor pulmonale, malnutrition, and death 2,12 . Respiratory dysfunction due to AO is the main cause of feeding difficulties leading to poor growth 4–6 . In addition to feeding and growth issues associated with isolated PRS, children with sPRS can develop other related problems due to an underlying syndrome or associated anomalies, respectively.…”

Study on the Effect of Bilateral Mandible Distraction Osteogenesis About the Nutrition Status of Infants With Pierre-Robin Sequence

“…It is one of the common symptoms appeared in patients with PRS. The causes of malnutrition in PRS are various, which may be related to the high energy consumption of respiratory work caused by upper airway obstruction, or to feeding difficulty (FD) caused by micrognathia and glossoptosis (8). The latter one may also be a direct result of respiratory dysfunction.…”