Weight Gain in Infants With Pierre Robin Sequence in the First Year of Life

2020 ◽  
Vol 163 (5) ◽  
pp. 1032-1037
Author(s):  
Leo Li ◽  
Andrew R. Scott

Objective To compare growth trends among infants with Pierre Robin sequence (PRS) to normal World Health Organization (WHO) growth standards. Study Design Case series with chart review. Subjects and Methods Twenty-four infants with syndromic and nonsyndromic PRS (54% male) treated at an urban academic medical center between 2009 and 2017 were included. Infants with symptomatic hypoventilation underwent mandibular distraction osteogenesis (71%). Weights were recorded at roughly 1- to 3-month intervals from birth to 12 months, with ages adjusted for prematurity. The 50th percentile (P50) for this cohort was calculated and compared to WHO standards. Results In total, 135 weight entries for 24 subjects were included. The birth weight P50 was similar to the WHO standard (females: 0.09 kg above WHO [95% CI, –0.25 to +0.43; z score = 0.19]; males: 0.38 kg below WHO [95% CI, –0.77 to 0.00; z score = −0.79]). A slower growth rate was noted among female and male infants with PRS: in month 5, the PRS P50 among females was 1.42 kg below the WHO standard (95% CI, –1.77 to −1.07; z score = −1.64). Among males in month 3, the PRS P50 was 1.68 kg below the WHO standard (95% CI, –2.12 to −1.24; z score = −2.19). By month 12, weight deficiencies had resolved in both groups. Conclusion Newborns with and without PRS may have similar birth weights, but the growth rate among male and female infants with PRS may lag behind that of unaffected infants, even when upper airway obstruction has been addressed in early infancy.

2021 ◽  
pp. 105566562110118
Author(s):  
Darren B. Abbas ◽  
Christopher Lavin ◽  
Evan J. Fahy ◽  
HyeRan Choo ◽  
Mai Thy Truong ◽  
...  

Objective: Mandibular distraction osteogenesis (MDO) is frequently performed to address airway obstruction in patients with Pierre Robin sequence (PRS), though more recently the technique of orthodontic airway plating (OAP) has gained traction. We aimed to evaluate OAP compared to MDO for airway obstruction in PRS. Design: A systematic literature search across PubMed, Embase, and Google Scholar identified all studies published in English, which involved MDO or any form of OAP as treatments for PRS. All relevant articles were reviewed in detail and reported on, adhering to PRISMA guidelines. Main Outcome Measures: Airway (tracheostomy avoidance, decannulation rate), feeding (full oral feeding tolerance). Results: Literature search identified 970 articles, of which 42 MDO studies and 9 OAP studies met criteria for review. A total of 1159 individuals were treated with MDO, and 322 individuals were treated with OAP. Primary outcomes appear similar for MDO and OAP at face value; however, this must be interpreted with different pretreatment contexts in mind. Conclusions: Orthodontic airway plating may be considered for airway obstruction in PRS, as some airway-related and feeding-related outcomes appear similar with MDO, per existing evidence in the literature. However, since PRS severity differed between studies, OAP cannot be uniformly considered a replacement for MDO. Further research is required to more comprehensively assess these treatment modalities inclusive of metrics that allow for direct comparison.


2018 ◽  
Vol 56 (3) ◽  
pp. 298-306
Author(s):  
Artur Fahradyan ◽  
Beina Azadgoli ◽  
Michaela Tsuha ◽  
Mark M. Urata ◽  
Stacey H. Francis

Objective: The workup of patients with Pierre Robin sequence (PRS) consists of a physical examination, O2 saturation, and polysomnography to determine the severity of respiratory obstruction and need for surgery. We suggest that capillary blood gas (CBG) may be a better physiologic representation of airway obstruction and should be routinely used in the management of patients with PRS. Design: This is a multicenter study based on a retrospective review of medical records. Setting: The study was performed at tertiary care centers. Interventions: Patients with PRS <1 year old underwent mandibular distraction osteogenesis. Main Outcome Measure: Using successful treatment outcome as a reference standard, receiver operating characteristic (ROC) curve was used to determine the accuracy of the diagnostic test and values for the best sensitivity and specificity to determine the need for surgical intervention. Results: Of 73 patients, 48 had sporadic PRS, 23 had syndromes, 2 had micrognathia, not otherwise specified. Mandibular distraction osteogenesis was performed in 62 patients at a mean age of 39 days. The mean initial Apnea-Hypopnea Index (AHI) in nonsurgical versus surgical groups was 10 versus 31 ( P = .063), pH 7.41 versus 7.34 ( P = .003), pCO2 43 versus 56 ( P < .001), and HCO3 27 versus 30 ( P = .022). The ROC curve showed that pCO2 of 49.5 has the best specificity (100%) and sensitivity (72.6%) profile in terms of need for definitive airway. Conclusion: A simple CBG heel stick may better predict the physiologic effects of obstructive apnea; therefore, it should be added to the algorithm of PRS workup.


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