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Introduction
Patients with Pierre-Robin Sequence (PRS) suffer from micrognathia, glossoptosis, and upper airway obstruction, which is sometimes associated with cleft palate and feeding issues. To overcome these symptoms in our full-term male neonate patient with PRS, mandibular distraction osteogenesis was performed.
Methods
The patient was intubated after airway endoscopy. A submandibular incision was carried down to the mandible. A distractor was modified to fit the osteotomy site that we marked, and its pin was pulled through an infrauricular incision. Screws secured the plates and the osteotomy was performed. The mandible was distracted 1.8 mm daily for twelve days.
Results
During distraction, the patient worked with speech therapy. Eventually, he adequately fed orally. He showed no further glossoptosis or obstruction after distraction was completed.
Conclusion
In our experience, mandibular distraction is a successful way to avoid a surgical airway and promote oral feeding in children with PRS and obstructive symptoms.
By: Ravi W Sun, BE
Surgeons:
Megan M Gaffey, MD
Adam B Johnson, MD, PhD
Larry D Hartzell, MD
Department of Otolaryngology – Head and Neck Surgery, University of Arkansas for Medical Sciences, Little Rock, AR, USA
Arkansas Children’s Hospital, Little Rock, AR, USA
Recruited by: Gresham T Richter, MD
1. A tongue stitch was placed using a 3-0 silk suture, so that the tongue could be retracted to assist with visualization.
2. We then performed a microlaryngoscopy and tracheobronchoscopy.
3. A 3.0 endotracheal tube was placed nasally and secured.
4. The patient was marked for bilateral submandibular incisions.
5. We marked the angle of the mandible, and marked and incised 1 finger-breadth below the body of the mandible as to protect the marginal mandibular nerve.
6. The skin was incised down to the platysma with a 15 blade. Dissection was carried out deep to the superficial layer of the deep cervical fascia, until we identified the mandible. Care was taken to protect the marginal mandibular nerve while elevating the tissues superiorly.
7. We cut the pterygomasseteric sling was inferiorly and elevated it off the medial and lateral surfaces of the angle, distal ramus, and proximal body of the mandible in a subperiosteal plane. A freer was used to identify the sigmoid notch and elevate the periosteum over the bony retromolar trigone.
8. Bovie cautery was used to mark an obtuse backwards seven over the planned osteotomy sites.
9. We made an infrauricular exit site for the distractor pin.
10. The 20mm Micro-Zurich distractor (KLS Martin, Germany) was modified to fit the preplanned osteotomy.
11. The pin was grasped and pulled through the infrauricular incision. The device was then tested.
12. The plates were positioned at about a 10-15-degree angle from the plane of the mandible to provide vertical gain in the distraction, and then secured using 3 7mm self-tapping screws on either side of the plate.
13. The Sonopet was used with power at 50, suction at 50%, and irrigation at 8 mL/min to perform the osteotomy.
14. We confirmed that the osteotomy was complete by distracting the 2 segments and then returning them to the original position with the segments touching.
15. The device was locked.
16. The skin was closed.
Pierre-Robin Sequence
Micrognathia
Glossoptosis
Upper airway obstruction
Dysphagia
Low birth weight
Abnormal mandible (particularly condyles)
Bovie Cautery
Sonopet Ultrasonic Aspirator
20 mm Micro Zurich Distractor (51-424-20-09)
Preparation for microlaryngoscopy and bronchoscopy, difficult airway
Mandibular distractors
Standard ENT setup
Sonopet, power at 50, suction at 50%, irrigation at 8 ml/min
Maxillofacial CT scan
Flexible fiberoptic laryngoscopy
Marginal mandibular nerve
Sigmoid notch
Retromolar trigone
Alternate procedures:
Tongue-lip adhesion
Nasal airway
Tracheostomy
Malunion
Nonunion
Airway compromise
None
Roy S, Patel PK. Mandibular lengthening in micrognathic infants with the internal distraction device. Arch Facial Plast Surg. 2006 Jan-Feb;8(1):60-4.
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