Neonatal Outcomes of Pierre Robin Sequence: An Institutional Experience

Abstract

Neonates with PRS who do not require surgical airway intervention are more likely to require assisted feeding, have slower weight gain, and a shorter average hospital stay compared to neonates undergoing mandibular MDO.

Pierre Robin sequence: Management of respiratory and feeding complications during the first year of life in a tertiary referral centre

“…The proportion of sPRS (14.6%) was similar to the findings of Bü tow et al [10]. Other authors described higher numbers of syndromic diagnoses between 27 and 46% [11,12]. These findings are somewhat unexpected since 91.7% of all patients were examined by a geneticist and underwent genetic testing in the search for syndromes.…”

“…This was done by means of a search of the Leuven Multidisciplinary Cleft database, identifying 48 PRS patients and 314 CP patients over an 11 year period. Subsequently, the medical records of the PRS patients were retrospectively analyzed.…”

“…This high complication rate in combination with the doubtfulness and limited size of effect, make glossopexy a temporizing measure that can only be used in cases without response on non-surgical interventions [2]. The technique has been used in 2.9 to 27% of the PRS population [9][10][11]14].…”

Early Mandibular Distraction to Relieve Robin Severe Airway Obstruction in Two Siblings with Lymphedema–Distichiasis Syndrome

“…The lack of major surgery-related adverse effects in our two patients treated with distraction osteogenesis agrees with that previously reported for other syndromic patients with micrognathia phenotype. In their retrospective review, AlSamkari [17] reported successful outcomes regarding upper airway and feeding management in patients with PRS independently of the syndromic status of the patients. Similarly, Taub et al, reported successful distraction for the hypoplastic mandible in patients with muscular dystrophy and amniotic band sequence, settings known to induce pathologic changes in the native bone [18,19].…”

Clinical and Molecular Characterisation of Children with Pierre Robin Sequence and Additional Anomalies

“…table 6). These children are sometimes grouped with syndromic PRS [Shprintzen, 1988[Shprintzen, , 1992Marques et al, 1998;Cruz et al, 1999;van den Elzen et al, 2001;Li et al, 2002;Printzlau and Andersen, 2004;Evans et al, 2006;de Buys Roessingh et al, 2007;Izumi et al, 2012;Patel et al, 2012;Gomez-Ospina and Bernstein, 2016] but also grouped with nonsyndromic children in other studies [Sheffield et al, 1987;Bütow et al, 2009;Al-Samkari et al, 2010]. These differences in classification of the PRS-Plus group make interstudy comparisons challenging.…”