Micrognathia and retrognathia, as observed in patients with the Hallermann-Streiff-Francois syndrome, might result in obstructive sleep apnea syndrome. When it becomes severe (apnea-hypopnea index [AHI], ≥30), noninvasive treatment options might be insufficient. An orthognathic treatment with mandibular advancement will increase the upper airway volume, which results in a decrease of apneas. A 53-year-old woman with Hallermann-Streiff-Francois syndrome and a history of antiresorptive medication suffered from severe obstructive sleep apnea (AHI, 77.7). She was treated with a combined orthodontic and surgical approach. The AHI decreased to 1, and the patient felt fitter after treatment. No medication-induced osteonecrosis nor inhibition of tooth movement was seen. A combined orthodontic and surgical treatment of a patient with severe obstructive sleep apnea was a good treatment choice. With a history of antiresorptive medication, the risks related to these medications have to be weighed up against the consequences of not treating obstructive sleep apnea syndrome. With a drug holiday, successful surgical treatment can be achieved.
Highlights
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The patient had severe obstructive sleep apnea (apnea-hypopnea index = 77.7).
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Obstructive sleep apnea syndrome was treated with combined orthodontic and surgical treatment.
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Condylar resorption and limited mouth opening were observed after surgery.
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The patient was treated for 2 years.
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The treatment resulted in an apnea-hypopnea index of 1 and a healthier patient.
Hallermann-Streiff-Francois syndrome (Online Mendelian Inheritance in Man no. 234100) is a rare genetic disorder (its inheritance is unclear). It is characterized by several craniofacial abnormalities, including a brachycephalic skull with frontal bossing and micrognathia. Because of the craniofacial features, especially the severe micrognathia and retrognathia of the mandible, a complete or partial constriction of the upper airway is observed and might result in obstructive sleep apnea syndrome (OSAS). , Several noninvasive treatment possibilities (ie, weight loss, decrease in alcohol consumption, sleep positioner trainer [SPT], oral appliances [OA], continuous positive airway pressure [CPAP]) are available to treat OSAS. However, when the OSAS is too severe, these treatment options might not be sufficient and surgical modification of the upper airway could be indicated (ie, uvulopalatopharyngoplasty, uvulopalatoplasty, maxillomandibular advancement [MMA]).
The uvulopalatopharyngoplasty includes excision of the tonsils and posterior soft palate or uvula and closure of the tonsillar pillars. With the laser-assisted uvulopalatoplasty, the uvula is shortened, and the soft palatal tissue is tightened. Another surgical procedure is the mandibular or an MMA. MMA will enlarge the velo-orohypopharyngeal airway and is currently the most effective surgical treatment for managing OSAS in adults, with a success rate of 86% after minimal 2-year follow up. , However, several complications including infections, dysesthesia, and reoperation might occur.
This case report describes a combined orthodontic and surgical treatment to treat severe OSAS.
Patient history
The patient was a 53-year-old woman with Hallermann-Streiff-Francois syndrome and an 8-year history of OSAS. She was a nonsmoker and had a body mass index of 20, which had remained stable since 2010.
The diagnosis and treatment timeline is presented in Table I . The treatment of choice was CPAP because of the severity of the OSAS. However, the treatment was not successful because of unfavorable seating and skin irritation while wearing the appliance. The patient was treated for 2 years (from May 2016 to May 2018) with a mandibular repositioning appliance with nose pillows. Unfortunately, the apnea-hypopnea index (AHI) with the mandibular repositioning appliance in place was still 22 (moderate sleep apnea). In 2015, the AHI increased to 77.7 without MRA, which is defined as severe sleep apnea (AHI ≥30).
| Date (mo/y) | Symptoms, findings, and treatment | Diagnosis | Recommendation and intervention |
|---|---|---|---|
| 09/2010 | First symptoms of OSAS:
|
Mild OSAS | Pneumologist: expectative policy |
| 06/2013 |
|
Severe OSAS | CPAP therapy with nose pillows indicated by pneumologist |
| 08/2013 |
|
MRA therapy with nose pillows indicated by pneumologist | |
| 01/2015 |
|
Moderate OSAS | Recommendations of pneumologist: bimaxillary advancement Intervention: the patient does not want surgery, continuation with MRA |
| 08/2015 |
|
Severe OSAS | Recommendations of pneumologist: consultation by an oral and maxillofacial surgeon |
| 12/2015 | Consultation with an oral and maxillofacial surgeon | Severe OSAS | Bimaxillary advancement with presurgical and postsurgical orthodontic treatment, drug holiday after consultation with a general practitioner |
| 01/2016 | Consultation and documentation at the orthodontic department | Extraction treatment, alignment with fixed appliances | |
| 05/2016 | Wound healing after extraction is good | Presurgical orthodontics: bonding maxillary and mandibular arch 10 d after extraction of teeth for alignment | |
| 04/2017 | Bimaxillary surgery Maxilla: advancement 1 mm, impaction 3 mm, expansion Mandible: advancement 10 mm, counterclockwise rotation |
||
| 05/2017 |
|
Postsurgical orthodontics: closing posterior open bite withwire bendings and vertical box elastics | |
| 06/2017 |
|
Physiotherapy | |
| 09/2017 |
|
Mild to moderate OSAS | SPT indicated by the sleep physician |
| 02/2018 |
|
Referral to gnathologist | |
| 04/2018 |
|
Condylar resorption | Medication Description:
|
| 05/2018 |
|
Debonding, retention check-ups after 6 wk, 3 mo, 1 y, and 2 y | |
| 06/2018 |
|
Masticatory muscle training and automassage of the musculus masseter and temporalis | |
| 07/2018 |
|
No OSAS |
Compounding the situation was the patient’s history of osteoporosis. The first symptoms were observed in 2014, and oral bisphosphonates (BP) in combination with calcium and vitamin D supplements were prescribed. Because of swelling problems and adverse effects, the therapy was switched to denosumab in 2015. A 60-mg dose of denosumab was injected twice. After consultation with the general practitioner, no more injections were given to allow a “drug holiday” to prevent medication-induced osteonecrosis after surgery and to enable tooth movement.
Diagnosis and etiology
The cephalometric analysis showed a severe retrognathia and micrognathia of the mandible and a hyperdivergent jaw complex ( Table II ).
| Measurement | Pretreatment | Posttreatment |
|---|---|---|
| Skeletal sagittal | ||
| SNA | 77.4 | 79.9 |
| SNB | 55.7 | 65.8 |
| ANB | 21.7 | 14.2 |
| Wits appraisal, mm | 40.6 | 2.2 |
| AR-A | 225.8 | 59.2 |
| AR-Pog | 221.2 | 75.8 |
| Skeletal vertical | ||
| NSL/ML | 90.4 | 73.9 |
| NL/ML | 66 | 54.9 |
| NSL/NL | 24.4 | 18.9 |
| Dental | ||
| ILs/NL | 94 | 91.7 |
| Is-APog | 55.7 | 9.1 |
| Interincisal angle | 116.5 | 131.6 |
| Ili/ML | 83.5 | 81.8 |
| li-APog | 21.6 | 6.4 |
| NSL/BOP | 47.1 | 40.4 |
| Soft tissues | ||
| Nasolabial angle | 123.3 | 137.6 |
| Ls to Sn-Pog’ | 28.5 | 4.7 |
| Li to Sn-Pog’ | 33.3 | 6.5 |
| Li to E-line | 28.3 | 2.2 |
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