Crouzon syndrome is defined as premature closure of one or more cranial sutures due to the mutation in fibroblast growth factor receptor-2 gene with the autosomal dominant trait,¹ leading to an abnormal growth pattern in the skull base, orbital cavities, and nasomaxillary complex, but there are no digital anomalies such as those seen in Apert syndrome as another craniosynostosis syndrome.² The typical clinical features are midface and maxillary deficiency, short nasal dorsum, undeveloped orbits, and subsequent exophthalmos; moreover, upper airway obstruction can occur in the most severe cases.³,⁴

The management of patients with Crouzon syndrome has two stages; in the first early stage, patients undergo cranial surgery for releasing the prematurely closed sutures usually at the age of 3 to 6 months and based on increased levels of intracranial pressure (ICP). In the second stage, craniofacial reconstructive surgery is done to correct the midface deficiency and subsequent Class III malocclusion, to decrease the scleral show and other anomalies in need of surgery.⁴

Besides the physical problems, quality of life in patients with Crouzon syndrome is highly affected, and as a result, all these problems have negative effects on patients' self-confidence and social relationships; therefore, early correction of craniofacial problems can improve patients' quality of life.⁵,⁶

Modified LeFort III osteotomy is one of the treatment modalities that can be used for proper resolution in adult patients. Historically, the first successful results for correction of midface deficiency using this technique were achieved by Tessier. It can be a good choice of treatment in conditions when the nasomaxillary complex is not involved.⁷

The present case report study aims to describe a patient with midface deficiency due to the Crouzon syndrome who was undergone modified LeFort III osteotomy with a periocular approach, nasal dorsum augmentation, and LeFort I osteotomy accompanied by genioplasty, wholly as a single surgical procedure.

The case reported in this study was a 21-year-old female patient complaining of difficulty in chewing and extreme scleral show of the eyes. Clinical examination showed severe deficiency in the midface area and lower rim of the orbits and subsequently severe exophthalmos. No digital deformity was noted and contour of the forehead was within the normal range, but there was a decrease in the nasofrontal angle which became exacerbated after the modified LeFort III osteotomy procedure. The length of the upper lip was 21 mm. Incisor show was 1 mm at rest and 6 mm at smile Figure 1a, Figure 1b, Figure 1c, Figure 1d, Figure 1e, Figure 1f, Figure 1g, Figure 1h, Figure 1i, Figure 1j, Figure 1k, Figure 1l. A consultation was done with neurosurgeon and ophthalmologist. The patient had no mental problem and ICP was normal. The visual condition was normal. The patient had no upper airway obstruction or respiratory problems. In the intraoral examination, a 5 mm anterior open bite and bilateral posterior crossbite were detected. The dental relationship was Class III (Cl III) bilaterally both in molar and canine region with approximately 10 mm reverse overjet Figure 1m, Figure 1n, Figure 1o. The patient had just undergone orthodontic treatment aiming alignment and levelling of both maxillary and mandibular teeth, obtaining compatible arch forms, resolving crowding/spacing, and removing dental compensations.⁸ Preoperative radiographic evaluation showed no asymmetry in the posteroanterior view and no root resorption in the panoramic view Figure 2a and Figure 2e. The cephalometric evaluation revealed severe maxillary deficiency (Sella-Nasion Angle [SNA] =71) and consequently a concave profile of the face (angle of convexity = ‒3). “A point-Nasion-B point ANB” and “A-B plane angle” measures showed Cl III jaw discrepancy. Obtuse Frankfort plane-mandibular plane angle and increased Y-axis A line from sella turcica to gnathion. It indicates the degree of the downward, rearward or forward position of the chin concerning the upper face showed vertical growth of the mandible and long-face tendency due to the backward rotation of the mandible. Severe retrogenia was noted in both clinical and cephalometric evaluation Table 1 and Figure 1a, Figure 1b, Figure 1c, Figure 1d, Figure 1e, Figure 1f, Figure 1g, Figure 1h, Figure 1i, Figure 1j, Figure 1k, Figure 1l and Figure 2c.{Table 1}Figure 1

Preoperative photography (a) Inferior view, (b and c) frontal lip closed and smile views, (d) Superior view, (e and f) Right oblique lip closed and smile views, (g and h) Left oblique smile and lip closed views, (i and j) Right profile lip closed and smile views, (k and l) Left profile smile and lip closed views; Presurgical intraoral photography. (m) Right, (n) Left, and (o) Frontal views of the occlusion.

Management of patients with craniosynostosis syndromes should be with a multidisciplinary approach. As noted previously, surgical management of Crouzon patients is a two-stage procedure leading to the improvement of the facial aesthetic as well as psychosocial aspects of the patient's life. Ideally, surgical reconstruction of the midface should be done in permanent dentition between the age group of 13–21 years following a comprehensive phase of orthodontic treatment, but if there are high levels of skeletal imbalance, then surgical intervention may be postponed to later ages.⁸

Some authors have recommended that LeFort III distraction osteogenesis (DO) can be considered as a reliable treatment modality for these patients due to the stable results and minimum risk of relapse and low rate of minor distraction-related complications.¹⁰,¹¹ While, patient compliance in utilizing the rigid external distractor device at low ages and socioeconomic status are the factors with paramount importance in achieving desired outcomes; many of these patients have socioeconomic problems which means that they are not economically affordable for receiving such a comprehensive, extended, and timely mannered treatment. On the other hand, in the osteotomy technique, unlike DO, the surgeon has full control over the amount and direction of the movement. Furthermore, there is a need for a second surgery in the DO technique for removing the device.

In another case report study performed by Mohammadi et al., it is reported that severe midface deficiency in an adult patient with Crouzon syndrome was improved by modified LeFort III osteotomy solely and without previous orthodontic treatment due to the low socioeconomic status of the patient.⁹ While performing such surgery in combination with orthodontic treatment and other needed surgical procedures can produce more satisfactory results from the surgeon's point of view as well as the patients if she/he is financially affordable. Moreover, performing genioplasty in cases with severe retrogenia can improve fascial aesthetic in addition to resolving the probable obstructive sleep apnea; also, performing LeFort I osteotomy following modified LeFort III osteotomy may reduce the risk of relapse and achieving more precise occlusal relationship.

Finally, selection between “the modified LeFort III osteotomy with subtarsal approach accompanied by closed-approach nasal dorsum augmentation” and “total LeFort III osteotomy with the coronal approach,” depends on the surgeon's preference and the former approach may be preferred to the coronal one due to the less complication and discomfort for the patient following extensive flap elevation in the coronal area.

Since many of patients with Crouzon syndrome are seeking treatment in older ages when they are missed for multidisciplinary management and DO technique in proper timing, osteotomy technique accompanied by other indicated procedures like LeFort I osteotomy, nasal dorsum augmentation, and genioplasty can result in highly successful outcomes both for patient and surgeon as well as many positive effects on patient's self-confidence and social aspect of his/her life; thus, such an approach is highly recommended, especially if the patient is financially affordable.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.