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Improving Nutritional Outcomes in Symptomatic Pierre Robin Sequence: A Cohort Comparison Study Between Tongue Lip Adhesion and Mandible Distraction
S. Alex Rottgers, MD1, Zoe Macisaac, MD2, Todd Wine, MD3, Lorelei Grunwaldt, MD4, Deepak Mehta, MD5, Anand Kumar, MD6.
1University of Pittsburgh; Department of Plastic and Reconstructive Surgery, Pittsburgh, PA, USA, 2Children's Hospital of Pittsburgh, Pittsburgh, PA, USA, 3Children's Hospital of Pittsburgh; Department of Otolaryngology, the University of Pittsburgh, Pittsburgh, PA, USA, 4Children's Hospital of Pittsburgh; University of Pittsburgh, Department of Plastic and Reconstructive Surgery, Pittsburgh, PA, USA, 5Children's Hospital of Pittsburgh; University of Pittsburgh, Department of Otolaryngology, Pittsburgh, PA, USA, 6Chidren's Hospital of Pittsburgh, University of Pittsburgh, Department of Plastic and Reconstructive Surgery, Pittsburgh, PA, USA.

Background and Purpose: The optimal treatment of Pierre Robin Sequence (PRS) associated dysphagia remains poorly characterized. This study aims to compare the feeding outcomes of symptomatic PRS patients treated with tongue lip adhesion (TLA) or mandibular distraction (DOG).
Methods: A retrospective cohort study of symptomatic PRS patients treated over 36 months was performed to compare feeding outcomes between TLA (Group 1) and DOG (Group 2)
Results: Twelve TLA and 15 DOG patients were identified. The average age at TLA was 0.9 months (0.1-2.2) and 7.2 months (0.03-18.6) at DOG. The average follow-up was 25.7 months (0.3-38.4) for TLA and 7.8 months (0.6-14.8) for DOG. Four (33%) TLA and nine (60%) DOG patients were syndromic. One TLA and 7 DOG patients had gastrostomy tubes placed prior to surgery. All of these patients remain gastrostomy dependent for partial or full nutritional support. The post-TLA gastrostomy rate was 54.5% (6 of 11) compared to the post-DOG gastrostomy rate of 12.5% (1 of 8) (p=.147). In Group 1 (TLA) 60% (3 of 5) of patients grew faster than their projected growth curve after surgery, 20% (1 of 5) grew parallel to their growth curve, and 20% (1 of 5) grew slower than their growth curve. In Group 2 (DOG) 57% (4 of 7) of patients grew faster than their projected growth curve after surgery, 14% (1 of 7) grew parallel to their growth curve, and 28.5% (2 of 7) grew slower than their growth curve. No statistical difference in growth rates was observed. The major complication rate was 8% in Group 1 (TLA), including one mortality and 0% in Group 2 (DOG). Minor complication rates were 9% in Group 1 (TLA) and 27 % in Group 2 (DOG), including 4 pin site infections treated successfully with antibiotics and hardware retention, and one transient marginal mandibular nerve palsy.
Conclusions: Mandibular distraction is superior compared to tongue lip adhesion in avoiding gastrostomy for supplemental alimentation in symptomatic Pierre Robin Sequence. This represents a significant decrease in health care burden for the patient and family in the neonatal period. Neither intervention was successful in achieving gastrostomy independence in early follow-up in those patients with prior tube insertion. Growth rates were similar for both treatment modalities if gastrostomy was avoided.


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