Use of External Distractors and the Role of Imaging Prior to Mandibular Distraction in Infants With Isolated Pierre Robin Sequence and Stickler Syndrome

Abstract

IMPORTANCE Computed tomographic (CT) scans are often obtained before mandibular distraction osteogenesis in patients with isolated Pierre Robin sequence. There is concern regarding the risk of radiation exposure from CT in children. OBJECTIVE To evaluate whether preoperative CT is necessary for adequate airway, feeding, and aesthetic outcomes following mandibular distraction with external distraction devices in infants with isolated Pierre Robin sequence. DESIGN, SETTING, AND PARTICIPANTS In a retrospective review of medical records, infants who underwent mandibular distraction between January 1, 1998, and September 30, 2014, at 2 tertiary children's hospitals were identified using procedure codes. Data analysis was conducted December 1, 2014, to March 31, 2015. Fifty-two patients fit the inclusion criteria of isolated Pierre Robin sequence or Stickler syndrome, of being younger than 9 months at the time of distraction, and of use of external distractors. Forty-two of these infants did not receive preoperative CT imaging. EXPOSURE Mandibular distraction osteogenesis for isolated Pierre Robin sequence or Stickler syndrome. MAIN OUTCOME MEASURES Number of infants who were able to avoid tracheostomy or achieve decannulation, who were able to avoid placement or achieve removal of a gastrostomy tube, and in whom there were no intraoperative complications, no open-bite deformity, no malocclusion, no asymmetry, and no postoperative complications. RESULTS In comparison with the 10 infants who underwent preoperative CT, all 42 of the infants (100%) who did not receive preoperative CT imaging successfully avoided tracheostomy or achieved decannulation (P = .04) and 40 patients (95%) did not require placement of a gastrostomy tube or were able to undergo gastrostomy tube removal postoperatively (P < .001). There were no significant differences between the CT and non-CT groups in the other 5 outcome measures. Two patients (5%) required postoperative gastrostomy tube placement, 2 patients (5%) had minor intraoperative complications that might have been anticipated with CT, 2 patients (5%) demonstrated malocclusion, and 1 infant (2%) had asymmetry at the end of the distraction phase. CONCLUSIONS AND RELEVANCE This series suggests that the absence of preoperative CT does not compromise functional or aesthetic outcomes in mandibular distraction with external distraction devices in infants with isolated Pierre Robin sequence or Stickler syndrome. This finding has implications for cost containment and reduction of radiation exposure to a vulnerable population. LEVEL OF EVIDENCE 4.

Surgical Management and Outcomes of Pierre Robin Sequence: A Comparison of Mandibular Distraction Osteogenesis and Tongue-Lip Adhesion

“…Selected articles were analyzed with regard to level of evidence 40 , patient demographics, length of follow up, diagnosis, distractor type, airway and feeding outcomes. With the exception of two studies 41,42 , all studies included a mixed cohort of syndromic and isolated PRS subjects. We present our search criteria in Table 1, and literature selection and outcomes measurement methodology in Figure 4.…”

Manejo de secuencia de Pierre Robin con distracción externa mandibular y palatoplastia de incisiones mínimas

“…Esto permite realizar el cierre del paladar a la edad de 9 meses, con la ventaja de una mejor respuesta fi siológica de la respiración y la deglución. Las complicaciones de este procedimiento incluyen la lesión de las ramas del nervio facial, del nervio alveolar inferior, lesiones de las raíces germinales dentales, cicatrices hipertrófi cas e infecciones 25 . En general, los pacientes sometidos a esta técnica logran una mejor saturación de oxígeno, con menor incidencia de apnea obstructiva del sueño y necesidad de traqueotomía que los pacientes sometidos a otras técnicas 26 .…”

Three-dimensional imaging of soft and hard facial tissues in patients with craniofacial syndromes: a systematic review of methodological quality

“…In this review, only one article was included in which CBCT was used (to investigate the area of the mandible between the mental foramina). 5 Non-radiation methods are undoubtedly preferable over invasive methods but they have their specific indications. 3D facial surface scanning was the second most used imaging method in the studies of this review.…”