Management of sleep-disordered breathing in patients with syndromic hemifacial macrosomia.

PURPOSE: Patients with syndromic hemifacial microsomia (SHFM) are at risk of obstructive sleep apnea (OSA). The aim of the study was to describe the prevalence of OSA and its management, especially in patients with Goldenhar syndrome (GS). METHODS: The respiratory polygraphies and clinical management of 15 patients, aged 2 to 23 years, evaluated at a national reference center, were analyzed. RESULTS: Four (27%) patients had no OSA, 4 (27%) had mild OSA, and 7 (46%), of whom 5 were ≤ 2 years old, had severe OSA. None of the patients had central apneas. Only one patient had alveolar hypoventilation, and another one had nocturnal hypoxemia. Two patients had severe OSA despite prior adenoidectomy or mandibular distraction osteogenesis. Median duration of follow-up was 3.5 years (range 0.5-9 years). None of the patients without OSA or with mild OSA at baseline respiratory polygraphy developed OSA during the follow up. Among the 7 patients with severe OSA, 3 required continuous positive airway pressure or noninvasive ventilation, and one patient required a tracheostomy. CONCLUSION: In conclusion, patients with SHFM are at high risk of severe OSA at any age, underlining the importance of systematic sleep studies to diagnose and evaluate the severity of OSA. Individualized treatment should be privileged, based on a careful examination of the entire upper airway, taking in account potential associated risk factors. All patients with SHFM should be managed by a pediatric expert multidisciplinary medical/surgical team until the end of post pubertal growth.

Mandibular symphyseal distraction osteogenesis: 20 years of experience treating transverse deficiencies with an internal hybrid device.

INTRODUCTION: Transverse mandibular deficiency has been traditionally managed by orthodontic compensation. However, without resolving the underlying skeletal hypoplasia it leads to high relapse rates. Few studies have reviewed the long-term experience and potential complications of mandibular symphyseal distraction osteogenesis (MSDO) as an alternative treatment method. MATERIALS AND METHOD: A retrospective review of 20 patients (range: 4-19 years; mean: 11.9 years) treated with a hybrid MSDO device over the period of 1996-2017 was conducted. Epidemiological data, medical and dental history as well as photographic and cephalometric analyses were carried out. Furthermore, pre-operative and long-term post-operative status including dental occlusion and tooth-jaw discrepancies were recorded. RESULTS: The distraction amount ranged from 3 mm to 15 mm (mean: 10 mm) with an average distraction period of 30.9 days. In long-term follow-up (mean: 7.3 years), 14 patients presented a class I and 6 patients presented a class II relationship. Correction of overjet, tooth jaw discrepancy and arch length discrepancy were obtained in 18, 20 and 17 cases respectively. A device malfunction was experienced in 6 patients. CONCLUSION: Mandibular widening using a hybrid MSDO device can be efficiently and safely performed in a paediatric population. Further studies confirming our results and analysing facial aesthetic outcomes are warranted.

Dental consequences of pterygomaxillary dysjunction during fronto-facial monobloc advancement with internal distraction for Crouzon syndrome.

Crouzon syndrome is a syndromic faciocraniosynostosis that can be associated with severe fronto-facial retrusion leading to major functional impairments: extreme exorbitism may be vision-threatening and severe respiration impairment can be life-threatening. The procedure of choice for the primary correction of this retrusion is fronto-facial monobloc advancement (FFMBA) with internal or external distraction. FFMBA involves pterygomaxillary dysjunction (PMD), using either a superior or an intra-oral approach. This step is at risk of damaging the germs of the decidual and permanent molars. Here we considered a series of 15 patients with Crouzon syndrome who benefited from FFMBA performed by the same surgeon, using a superior approach through the infra-temporal fossa for PMD. Based on pre-operative, early post-operative and late post-operative CT-scans, we recorded missing teeth, morphological dental anomalies and the Nolla stage for the first and second permanent maxillary molars. We showed that early FFMBA has significant dental consequences, and that these dental effects are correlated with an early age at surgery. Although the indications of early FFMBA in Crouzon syndrome with severe functional repercussions are not questionable, our results should be compared to dental outcomes of FFMBA performed with an intra-oral PMD.

Skeletal and soft tissue changes and stability in cleft lip and palate patients after distraction osteogenesis using a new intraoral maxillary device.

BACKGROUND: The authors have recently reported on the use of an internal maxillary distraction device. In this study, we report on the hard and soft tissue movements achieved with this intraoral distraction device, and the stability changes after distraction osteogenesis for maxillary hypoplasia in patients with cleft lip and palate. METHODS: Ten male patients with severe hypoplasia of the maxilla, with complete uni- or bilateral cleft lip and palate were included. The mean age of the patients at the time of operation was 11.91 years (±3.41). To evaluate the distraction process and stability, superimpositions on the preoperative lateral cephalograms were performed. The mean follow-up (FU) was 15.42 months (±3.94). RESULTS: Cephalometric measurements at all of the maxillary hard and soft tissue points improved significantly. Maxillary point A was advanced by 8.25 mm (±3.17; P < 0.001). After distraction soft tissue point A' had advanced 7.10 mm (±2.69; P < 0.001). The soft tissue to hard tissue ratio at point A was 0.86:1 after distraction. Maxillary horizontal relapse at point A was 14.1% at FU. Vertical relapse was not significant. CONCLUSION: This rigid intraoral distraction device can be successfully used in the correction of severe maxillary hypoplasia. The marked aesthetic improvement and low psychological encumbrance make this device viable for the treatment of cleft-related hypoplasia of the maxilla.

True hemifacial microsomia and hemimandibular hypoplasia with condylar-coronoid collapse: diagnostic and prognostic differences.

INTRODUCTION: Long-term results after orthopedic or surgical treatment of hemifacial microsomia (HFM) have shown a tendency toward recurrence of the facial asymmetry. However, the literature contains a number of successful case reports that show surprising changes in the morphology of the condyles. In addition, patients with similar mandibular asymmetries, treated early with surgery, have excellent long-term follow-ups, especially those who have little or no soft-tissue involvement, but only severe mandibular ramal deformities. The phenotypes of these cases are unexpectedly similar, with a consistent collapse of the condyle against the coronoid and a deep sigmoid notch. The objectives of this article were to help distinguish true HFM from this peculiar type of hemimandibular asymmetry morphologically and to quantify their differences before treatement and in the long term. METHODS: Panoramic radiographs taken at pretreatment and the long-term follow-up of 9 patients with hemimandibular hypoplasia, characterized by the collapse of the condyle against the coronoid, were compared with those of 8 patients with severe type I and type II HFM; these records were collected before and at least 10 years after distraction osteogenesis. RESULTS: Ratios and angular measurements before and after treatment differed significantly between the 2 groups. CONCLUSIONS: Perhaps these patients were misdiagnosed and actually had secondary injuries of the condyle, which have a normal functional matrix. Therefore, with growth and functional stimulation, they would tend to grow toward the original symmetry. To make a differential diagnosis between true HFM and this peculiar type of hemimandibular hypoplasia, the collaboration between not only orthodontists and surgeons, but also geneticists and dysmorphologists, is of great importance because of the different prognoses.

Five years experience with a new intraoral maxillary distraction device (RID).

Maxillary distraction osteogenesis is well established for the treatment of severe retromaxilla. We report our experience since 2004 of the treatment of 19 patients using a new intraoral maxillary distraction device. Maxillary advancement was successful in all patients with mean advancement of 9.6mm (range 4-17) measured at a point in lateral cephalograms. The new device limited surgical exposure and the amount of materials implanted, and improved control in every phase of the distraction. It was psychologically accepted by patients and was more comfortable than existing devices.