A.A. Heggie

Complete correction of severe scaphocephaly: the Melbourne method of total vault remodeling.

Background: A new method of total calvarial remodeling for correcting significant scaphocephaly is described, and experience with the first 30 consecutive patients is presented. Methods: Thirty consecutive patients (22 boys and eight girls) with moderate to severe scaphocephaly who underwent total calvarial remodeling using the Melbourne technique were analyzed retrospectively. Mean age at surgery was 7.5 months (range, 4 to 18 months). All had significant frontal and occipital deformities, bitemporal narrowing, a low cranial index, an anteriorly located vertex, and a narrow posteroinferiorly sloping posterior cranium. Clinical photographs, computed tomographic imaging, and cranial index were evaluated preoperatively and postoperatively. A sufficient follow-up period in 17 patients enabled the measurement of changes in cranial indices. Results: A dramatic improvement in head shape was confirmed subjectively by clinical examination and photographs and, in 17 patients, objectively by analyses of the cranial indices. In these 17 patients, mean preoperative cranial index was 64.6 percent preoperatively (range, 56 to 69.3 percent) and increased to a mean of 77.5 percent postoperatively (range, 72 to 81 percent). The mean increase in cranial index was 12.9 percent (range, 9.5 to 19 percent). Among the 30 patients, overall complications were major in one patient (3.3 percent) (presumed air embolus) and minor in three (10 percent). Conclusions: The Melbourne technique of total vault remodeling consistently achieves a virtually normal head shape. After analysis of the first 30 cases, the authors recommend this as their technique of choice for severe scaphocephaly when the full constellation of deformities is present.

LeFort III internal distraction in syndromic craniosynostosis.

Distraction osteogenesis of the mid-face alleviates the requirements of substantial autogenous bone grafts and donor site morbidity and alleviates the restriction of the soft-tissue envelope in gaining advancement of the mid-face. A prospective study, over a 14-month period, was initiated to evaluate the results of seven consecutive patients undergoing mid-facial advancement who were treated with Le Fort III internal distraction using the MID device. All patients had syndromic craniosynostoses and six patients had previously undergone fronto-orbital advancement. Four patients had symptoms of airway obstruction and one patient was tracheostomy dependent. Six of the patients completed the distraction as planned. Lateral cephalograms were analyzed and the mean linear distraction was 18 mm with a range of 8 mm to 23 mm. Complications included infection (one patient), mechanical failure (one patient), intraoperative fragment dysjunction (three patients), velopharyngeal insufficiency (one patient), conjunctivitis (two patients), trismus (six patients), and bony irregularities. It was concluded that in our Units future protocol for managing infant syndromic synostoses, synostectomies and cranial vault remodeling will be undertaken in infants as before, but that in early childhood, patients with severe mid-facial hypoplasia and/or respiratory compromise will be offered distraction osteogenesis as a substitute for the traditional Le Fort III advancement and bone grafting.

Hemifacial Microsomia: Use of the OMENS- Plus Classification at the Royal Children's Hospital of Melbourne

&NA; Hemifacial microsomia is the most common facial congenital disability after cleft lip and palate, but as yet its pathogenesis remains unknown. Clinical classification systems have evolved over the last 30 years from those classifying only single components of the disorder, to those classifying according to the combination of deformities, to the most recent systems that grade each anatomical component separately, such as the Orbit, Mandible, Ear, Nerve, and Soft tissue (OMENS) system. The aim of the present study was to review the classification of patients with hemifacial microsomia treated by the Melbourne Craniofacial Unit at the Royal Childrens Hospital using the OMENS‐Plus system of classification and to correlate the findings with data from other centers. Records of patients treated by the craniofacial unit were reviewed and included in the study if adequate clinical records, photographs, and radiographs (anteroposterior, lateral, basal cephalometry, panoramic views) were available. The data were entered into a database file developed for this purpose. Seventy‐one patients were identified from the hospital database, of which six were excluded because of incomplete data. Of the 65 patients, there were 31 (48 percent) with right‐sided microsomia. 25 (38 percent) with leftsided microsomia, and nine (14 percent) with bilateral microsomia, with an overall male‐to‐female ratio of 1.2:1. The majority of patients had a normal orbit (77 percent), mildly hypoplastic mandibular ramus‐condyle with functioning temporomandibular joint (57 percent with type M1 or M2a), normal facial nerve (76 percent), and mild soft‐tissue hypoplasia (73 percent). There was a similar proportion of patients with mild ear anomalies (53 percent with grade 0 or 1) compared with those with more severe anomalies (47 percent with grade 2 or 3). Correlative analysis demonstrated a slight but positive correlation between the severity gradings of the five individual components. The correlation was lowest between the grading of the nerve and ear and that of the mandible and nerve. The data demonstrate the phenotypic variability of hemifacial microsomia and suggest a degree of relationship among the components of hemifacial microsomia. The OMENS‐Plus system has provided a major advancement in the classification of hemifacial microsomia. The authors suggest refinements to the grading of the orbit and nerve components.

A comparison of the stability of single-piece and segmental Le Fort I maxillary advancements.

This study retrospectively evaluated the stability of Le Fort I maxillary advancements and compared segmental and one-piece maxillary osteotomy procedures. A cephalometric analysis was performed on 26 cases of maxillary advancement. The sample comprised 11 cases of one-piece and 15 cases of segmental maxillary procedures. The tracings were superimposed and digitized by computer software, and the skeletal changes were analyzed before surgery, immediately after surgery, and at a minimum of 1 year of follow-up. Different values were compared by the paired and nonpaired t tests and were correlated by the Pearson correlation test. The significant value was set at a 95% confidence interval. The maxilla was advanced by a mean of 5.0 ± 1.6 mm (P < 0.001), and the anterior maxilla was repositioned inferiorly by a mean of 1.5 ± 3.3 mm (P < 0.05). The maxilla relapsed posteriorly by a mean of 0.6 ± 1.2 mm (P < 0.05) and superiorly at the anterior maxilla by a mean of 0.8 ± 1.1 mm (P < 0.001). Overjet and overbite did not significantly change (P > 0.05). It was concluded that maxillary advancement using rigid fixation and interpositional bone grafting in both groups was a stable procedure, particularly in the horizontal plane. In the one-piece group, there was a significantly higher relapse in the vertical plane than in the segmental group (P < 0.05), however. Minor skeletal relapse was compensated for by postoperative tooth movement, and segmental procedures are recommended when required to enhance occlusal results.

Management of isolated orbital floor blow-out fractures: a survey of Australian and New Zealand oral and maxillofacial surgeons

Background: This is the first report of involvement of Australian and New Zealand oral and maxillofacial surgeons in the management of isolated orbital floor blow‐out fractures and was conducted to obtain comparisons with the results from a recent similar survey of British oral and maxillofacial surgeons.

Prenatal and postnatal management of congenital granular cell tumours: a case report

Congenital granular cell tumour is a rare, benign, soft tissue lesion of the oral cavity. Large or multiple lesions can cause mechanical obstruction of the oral cavity and may result in prenatal polyhydramnios, and postnatal feeding and respiratory problems.We present a case in which prenatal diagnostic imaging was used in the successful management of an infant with a large congenital granular cell tumour.

Difficult tracheal intubation induced by maxillary distraction devices in craniosynostosis syndromes

Background: Difficult intubation occurred during anaesthesia for removal of maxillary distraction devices in five of seven children with syndromal craniosynostoses (four with Apert, two with Pfeiffer and one with Crouzon syndrome).Background: Difficult intubation occurred during anaesthesia for removal of maxillary distraction devices in five of seven children with syndromal craniosynostoses (four with Apert, two with Pfeiffer and one with Crouzon syndrome). Methods: Intubation was assessed in terms of laryngeal view and an established intubation difficulty score and had been straightforward before device insertion. Difficulty was induced by trismus due to device insertion and by increased maxillary prominence. This was compounded by preexisting mandibular hypoplasia. Cephalometric analysis, with each child acting as their own control, demonstrated anterior displacement of the maxilla and increased maxillary vertical height, as well as increased protuberance of the maxillary incisors. Results: All five difficult tracheal intubations were associated with preoperative Mallampati scores of 3 or 4 and the nine straightforward intubations with scores of 1 or 2. Maximal interincisor distance was less than the lower 95% confidence limit for age in all five children who were difficult to intubate at the time of device removal. No child had a failed intubation, but all had significantly increased intubation difficulty. Conclusions: In view of the risks of trauma, hypoxia and aspiration associated with difficult direct laryngoscopy, we recommend elective fibreoptic intubation at anaesthesia for removal of maxillary distraction osteogenesis devices in these children.

The role of distraction osteogenesis in the management of craniofacial syndromes

Distraction osteogenesis (DO) has been established as a useful technique in the correction of skeletal anomalies of the long bones for several decades. However, the use of DO in the management of craniofacial deformities has been evolving over the past 20 years, with initial experience in the mandible, followed by the mid-face and subsequently, the cranium. This review aims to provide an overview of the current role of DO in the treatment of patients with craniofacial anomalies.

Novel variants in GNAI3 associated with auriculocondylar syndrome strengthen a common dominant negative effect.

Auriculocondylar syndrome is a rare craniofacial disorder comprising core features of micrognathia, condyle dysplasia and question mark ear. Causative variants have been identified in PLCB4, GNAI3 and EDN1, which are predicted to function within the EDN1–EDNRA pathway during early pharyngeal arch patterning. To date, two GNAI3 variants in three families have been reported. Here we report three novel GNAI3 variants, one segregating with affected members in a family previously linked to 1p21.1-q23.3 and two de novo variants in simplex cases. Two variants occur in known functional motifs, the G1 and G4 boxes, and the third variant is one amino acid outside of the G1 box. Structural modeling shows that all five altered GNAI3 residues identified to date cluster in a region involved in GDP/GTP binding. We hypothesize that all GNAI3 variants lead to dominant negative effects.

The use of mandibular buccal cortical grafts in bimaxillary surgery.

for an interpositional graft, a decision must be made to select the most suitable bone or bone-substitute material. Autogenous bone (iliac crest, rib, calvarium and mandibular symphysis), freeze-dried bone, and hydroxylapatite blocks all have been used successfully. This report details the use ofmandibular buccal cortical bone in bimaxillary surgery for grafting the advanced maxilla, thus avoiding a separate donor site and facilitating the initiation of the mandibular sagittal osteotomies.