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

Abstract

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.

The Effect of Timing of Mandibular Distraction Osteogenesis on Weight Velocity in Infants Affected by Severe Robin Sequence

“…Recently, in another review case series, the weight of 24 infants with isolated RS and syndromic RS was compared with the normal controls. In this review study, investigators reported that newborns with and without PRS had similar birth weights, but the growth rate of infants with PRS lagged behind the normal controls even if addressed at an early stage [34].…”

Retrospective study on growth in infants with isolated Robin sequence treated with the Tuebingen Palate Plate

“…A slower growth rate compared to WHO norms was also observed in 24 RS infants predominantly treated with mandibular distraction osteogenesis. These infants showed a marked decrease in growth rate in the first 6 months after birth, with Z-scores at 3 and 5 months of age being − 2.19 and − 1.64, respectively [ 17 ]. After a subsequent period of accelerated growth in most infants, boys had caught up with their unaffected peers by 12 months of age, while girls continued to show a weight below WHO norms [ 17 ].…”

“…These infants showed a marked decrease in growth rate in the first 6 months after birth, with Z-scores at 3 and 5 months of age being − 2.19 and − 1.64, respectively [ 17 ]. After a subsequent period of accelerated growth in most infants, boys had caught up with their unaffected peers by 12 months of age, while girls continued to show a weight below WHO norms [ 17 ]. In a Dutch study on 69 infants with RS, growth in the first 2 years was significantly lower than in patients with isolated cleft palate [ 11 ].…”

“…Impaired weight gain in the first postnatal months has been reported in several studies on RS [ 5 , 11 , 15 – 17 ]. Malnutrition is defined as an imbalance between nutrient requirements and intake that results in cumulative deficiencies in energy, protein or micronutrients that can negatively impact growth, development and other relevant outcomes [ 18 ].…”

Pierre Robin and breathing: What to do and when?

“…Another group calculated standard deviation (Z‐)scores for weight throughout the first year of life in 24 infants with RS, with 17 being treated by MDO. Their Z‐scores changed from + 0.1 and −0.8, respectively, in girls and boys at birth to a nadir of −1.6 at 5 months in girls, and −2.1 at 3 months in boys, translating in a mean weight deficit compared to the World Health Organisation standard of 1.7 and 1.4 kg at these time points 61 . Catch‐up growth following MDO onset has also been reported in a retrospective analysis from Yale, in parallel to an increase in the proportion of infants being fully orally fed from 50% pre‐operatively to 73% at 6 months after surgery 62 …”