CYSTOID MACULAR EDEMA AFTER FOUR-POINT SCLERAL FIXATION OF INTRAOCULAR LENS.

PURPOSE: To assess the incidence of cystoid macular edema (CME) associated with 4-point Gore-Tex suture intraocular lens (IOL) scleral fixation, before and after institution of routine intravitreal triamcinolone acetonide prophylaxis and long-term topical nonsteroidal anti-inflammatory drug usage. METHODS: Consecutive patients were included after IOL implantation with concurrent pars plana vitrectomy for spontaneous IOL dislocation due to pseudoexfoliation syndrome. We compared short-term prophylactic nonsteroidal anti-inflammatory drugs only (Group A) to prophylactic intravitreal triamcinolone acetonide and long-term nonsteroidal anti-inflammatory drugs (Group B). RESULTS: Twenty-six eyes of 26 patients with pseudoexfoliation syndrome and spontaneous IOL dislocation were studied. Mean logMAR visual acuity improved from 1.27 ± 0.80 (20/375 Snellen equivalent) preoperatively to 0.46 ± 0.39 (Snellen 20/43) postoperatively (P < 0.001). Visual outcomes were similar for Groups A and B. In Group A, 10/16 eyes had CME, 4/16 had chronic CME longer than 6 months, and 1 longer than 12 months. In Group B, 1/10 had CME (which was both chronic and refractory). CONCLUSION: In eyes with pseudoexfoliation syndrome and spontaneous IOL dislocation, 4-point Gore-Tex suture IOL ab externo fixation yielded good visual outcomes, although CME was observed more than reported elsewhere. Prophylactic intravitreal triamcinolone acetonide and long-term nonsteroidal anti-inflammatory drugs seem to reduce the risk of postoperative CME.

The Craniofacial and Upper Limb Management of Nager Syndrome.

INTRODUCTION: Nager syndrome is a rare condition characterized by craniofacial and upper limb abnormalities. It is commonly mistaken for Treacher Collins syndrome, with which it shares the same craniofacial phenotype. However, patients with Treacher Collins do not exhibit hand anomalies, which are seen in patients with Nager syndrome. This paper reviews the multidisciplinary management of patients with Nager syndrome who were treated at the Australian Craniofacial Unit, Adelaide and the Erasmus Medical Centre, Rotterdam. METHODS: The database of both units was scrutinized and the case-notes of the patients with Nager syndrome were reviewed. Data was collected on patient demographics, surgical management, complications, and outcome. RESULTS: Nine patients (6 M:3 F) were identified with Nager syndrome, with a mean age at presentation of 3.7 years (range 8 days to 11.8 years). The mean follow-up time was 2.2 years (2 months to 19 years). SF3B4 mutation was noted in 2 patients and 1 patient had an X:9 translocation. Seven (77.8%) had obstructive sleep apnoea, with 5 patients diagnosed as severe obstructive sleep apnoea. Four patients had pollicization of their index, 2 patients had excision of extra radial digits and 1 patient underwent thumb duplication correction. Craniofacial surgery included mandibular advancement in 5 patients, temporo-mandibular joint reconstruction in 2 patients, and a genioplasty in 1 patient. CONCLUSION: Nager syndrome is a rare acrofacial dysostosis syndrome that is best managed within the realms of a multidisciplinary team. The authors would advocate early pollicization in patients with thumb anomalies to prevent any impairment in manual dexterity.

Facial aesthetics and perceived need for further treatment among adults with repaired cleft as assessed by cleft team professionals and laypersons.

The objectives of this study were to compare the ratings of professionals and laypeople with and without a cleft regarding the facial aesthetics of adult patients previously treated for orofacial clefting. The necessity for further treatment, as perceived by the respective groups, is also compared. The design of the study was a cross-sectional study. Professionals (two plastic surgeons, one dentist, one orthodontist, and one psychologist) and laypeople (one male and one female adult without a cleft and one male and one female adult with a cleft) were recruited to rate photographs of 80 non-syndromic cleft patients treated by the Australian Craniofacial Unit from 1975 to 2009. Facial aesthetics were measured by a visual analogue scale (VAS; 0-100 mm). High values indicated good aesthetics. Necessity for further treatment was also measured by a VAS (0-100 mm). High values indicated high perceived need for further treatment. The professionals rated facial aesthetics significantly lower and had a lower perception of need for further treatment than the raters with and without a cleft. The laypeople with a cleft rated facial aesthetics significantly higher and had a lower perceived need for further treatment than laypeople without a cleft. The non-surgical professionals rated facial aesthetics significantly lower and had a lower perceived need for further treatment than the surgical professionals. Differences exist in the facial aesthetics ratings and perceived need for further surgery between professionals and laypeople with and without a cleft. This should be considered when managing cleft treatment expectations.

Binder syndrome.

UNLABELLED: Patients with chondrodysplasia punctata (CDP) usually present with Binder-type features, and often CDP is misdiagnosed as Binder syndrome. This study reviewed the management and outcome of patients with Binder syndrome and CDP in a multidisciplinary setting. METHODS: The notes and radiographs of the patients managed at the Australian Craniofacial Unit with a multidisciplinary setting since 1976 were reviewed, and data were collected on patient demographics, associated medical and surgical problems, subsequent management, and complications. RESULTS: Seventy-seven patients were treated over the 30-year period (5 patients were lost to follow-up); of the remaining 72 patients, 60 (83%) had Binder syndrome, and 12 (17%) were patients with CDP. Forty were males, and 32 were females, with an age range of 6 months to 47 years. Thirteen patients (18%) had a strong family history, and 65 patients (90%) have so far undergone surgical correction, and of those, 35 (54%) have completed their treatment, the longest follow-up time being 18 years. The mean number of surgical procedures was 2.4, and 18 patients (28%) had postoperative complications, which included partial necrosis of the maxilla, osteomyelitis of the mandible, facial nerve and inferior alveolar nerve neuropraxia, nasal bone graft exposure, and cellulitis. DISCUSSION: Because of the phenotypic characteristics shared by both Binder syndrome and CDP, it is most likely that Binder syndrome is not a syndrome, nor is it an entity, but most likely to be an "association." We would advocate that these patients should be managed in a multidisciplinary setting.

Modified transoral approach for resection of skull base chordomas in children.

PURPOSE: Chordomas are rare slow growing, locally destructive tumours originating from remnants of the primitive notocord and are found most commonly in the clivus and saccrococcygeal region. These tumours usually present in early adult life but on occasion can present in childhood. The combination of the skull base location and paediatric patient size makes access to these tumours particularly challenging. METHODS AND RESULTS: We report a multidisciplinary technique used in two cases in children where a modified extended palatal split was undertaken to allow greater access for tumour excision. CONCLUSION: This approach allows for good access to the skull base region to allow for maximal tumour resection. This technique also appears to have minimal impact on palatal function and no adverse effects on the upper airway management.

Treacher Collins syndrome: protocol management from birth to maturity.

BACKGROUND: : Management of patients with Treacher Collins syndrome is complicated and involves multiple disciplines working in concert to achieve a common outcome. This article reviews the experience at the Australian Craniofacial Unit and describes the protocol for management. METHODS: : Fifty patients were treated during the last 30 years. The records of these patients were reviewed to establish what interventions they required and how these fit into a protocol for management. RESULTS: : The protocol for management of Treacher Collins syndrome can be divided into 3 epochs. In the first epoch from birth to age 2, airway and feeding problems were the main focus. Four patients required tracheostomy. Of these, 1 died and the others received mandibular distraction. Hearing is evaluated and addressed early. Eleven patients (23%) required repair of a cleft palate. In the second epoch (aged 2-12 y), speech therapy is critical as is a focus on integrating into the education system. During this epoch, reconstruction of the upper face was performed either with bone grafts or with vascularized bone flaps. Both required repeat bone grafts later. In the third epoch (aged 13-18 y), orthognathic surgery was performed. Revision surgery and further bone grafting were performed again at around age 18. Patients reported being generally happy with their appearance and with few exceptions were able to complete education, gain employment, and feel socially accepted. CONCLUSIONS: : Management of patients with Treacher Collins syndrome should be through a multidisciplinary protocol to achieve good results while minimizing confusion and unnecessary surgery.

Hyperostosis as a late sequel of parasymphyseal mandibular fractures in 2 children.

BACKGROUND: The potential problem of growth anomalies affecting a mandible following a fracture of a mandibular condyle in childhood is well established. However, there have been no previous reports of this phenomenon affecting other fracture sites in the mandible. PATIENTS: Two patients who had parasymphyseal fractures treated in childhood presented at skeletal maturity with hyperostosis at the fracture site, producing chin asymmetry in their teens. RESULTS: In both cases the hyperostosis produced significant chin asymmetry without disturbance of the occlusion. Both patients were managed with corrective genioplasty. CONCLUSION: These cases reinforce the previous recommendations regarding the need for long-term follow-up of children who sustain facial fractures of the mandible, and that the protocol should be expanded to include parasymphyseal fractures as well as fractures of the condyle.

Management of facial dysmorphogenesis in nemaline myopathy: a case report.

Nemaline myopathy is a rare congenital muscle disease, which is clinically and genetically heterogeneous. Both neonatal and adult onset can occur; in those with neonatal onset, the resulting muscle weakness can also afflict the facial musculature and hence influence facial growth and development. This article reports on a case in which no orthodontic intervention was undertaken during childhood and adolescence. An early decision was made to treat the facial dysmorphogenesis surgically once skeletal maturity had been reached. The authors discuss and illustrate the untreated facial growth in this condition and the surgical outcome following orthodontic treatment and orthognathic surgery.

Herbert Moran Memorial Lecture. World War I: the genesis of craniomaxillofacial surgery?

Herbert Moran enlisted in the Royal Army Medical Corps early in World War I. His autobiography captures the impact of contemporary experience of wartime gunshot wounds, seen in vast numbers and with little understanding of the requirements of wartime surgery. Wounds of the face and brain were numerous, especially in trench fighting. In France, Germany, Britain and elsewhere, surgeons and dentists collaborated to repair mutilated faces and special centres were set up to facilitate this. The innovative New Zealand surgeon Harold Gillies developed his famous reconstructive techniques in the Queen's Hospital at Sidcup, with the help of dental surgeons, anaesthetists and medical artists. The treatment of brain wounds was controversial. Many surgeons, especially on the German side, advocated minimal primary operative surgery and delayed closure. Others advocated early exploration and immediate closure; among the first to do so was the Austro-Hungarian otologist Robert Bárány. In 1918, the pioneer American neurosurgeon Harvey Cushing published well-documented proof of the desirability of definitive operative management done as soon as possible. Few World War I surgeons developed their knowledge of plastic surgery, neurosurgery and oral surgery in post-war practice. An exception was Henry Newland, who went on to pioneer the development of these specialties in Australasia. After World War II, the French plastic surgeon Paul Tessier created the multidisciplinary subspecialty of craniomaxillofacial surgery, with the help of his neurosurgical colleague Gérard Guiot, and applied this approach to the correction of facial deformities. It has become evident that the new subspecialty requires appropriate training programs.

From birth to maturity: a group of patients who have completed their protocol management. Part III. Bilateral cleft lip-cleft palate.

BACKGROUND: The optimal management of cleft lip-cleft palate patients presents a formidable challenge to the cleft surgeon. This is especially so in the case of bilateral cleft lip-cleft palate, and the long-term management in a multidisciplinary setting is essential. This study presents the results of the specific management protocol at the Australian Craniofacial Unit for patients with bilateral cleft lip-cleft palate who have completed their protocol treatment under the care of a single surgeon (D.J.D.) during the period 1974 to 2006. METHODS: A retrospective study of the outcomes in relation to facial growth, speech, hearing, and occlusion is presented of patients with bilateral cleft lip-cleft palate. RESULTS: Nineteen cases were identified from the departmental database, 12 male patients and seven female patients. Six patients with severe craniofacial deformities who had bilateral cleft lip-cleft palate were excluded. Cephalometric analysis at skeletal maturity identified that a majority of cases had midface hypoplasia requiring midface advancement in 14 cases. Speech and hearing outcomes were worse when compared with other clefting types. CONCLUSION: Overall, these results demonstrate that facial growth is more affected in bilateral cleft lip-cleft palate patients than in either unilateral cleft lip-cleft palate or isolated cleft palate patients. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Simultaneous multiple vector distraction for craniosynostosis syndromes.

Syndromic craniosynostoses are commonly treated conditions in craniofacial units. The features of the common syndromes (Apert, Pfeiffer and Crouzon) all include craniosynostosis, mid-face hypoplasia and ocular proptosis. The craniofacial management of a child with these syndromes through to adulthood may require a number of surgical interventions to allow brain development, to provide an adequate airway, to prevent corneal ulceration and to provide a functional dental occlusion. The management of these different priorities into timed interventions in our unit is determined by established protocols. We report two cases that underwent simultaneous mid-face (Le Fort III) and fronto-orbital osteotomies followed by distraction but using different vectors to advance the upper and mid-face regions (to achieve all treatment goals) in a 12-year-old boy and a 16-year-old girl.

Fracture patterns and bone healing in recurrent mandibular fractures: a clinical study of 13 patients.

BACKGROUND: Recurrent fractures of the previously treated fractured mandible are rare. METHODS: In this retrospective study, 13 cases of repeated mandibular fractures were evaluated according to age, sex, cause, fracture localizations and patterns, treatment modalities, and complications. RESULTS: All of the patients were men, with a mean age of 27.5 years and 32 years for the first and the subsequent fractures, respectively. The mean time interval between fractures was 4.3 years. In 10 cases, the cause for the second fracture was an assault, with 90 percent related to alcohol intake. Angle, body, and parasymphysis region of the mandible were the most common sites to be involved in the first occasion. In 10 cases, an internal rigid fixation procedure was performed for the treatment. In the recurrent injuries, none of the fractures occurred at exactly the same anatomical site. Fractures were either in the neighboring side or the contralateral side of the previously healed area of mandible. On the second occasion, angle fractures were common and body and subcondylar fracture rates increased. At second presentation, the complication rate increased from 23.1 percent to 53.8 percent and most commonly involving malocclusion. CONCLUSIONS: This study showed that assault is the major etiologic factor for recurrent mandible fractures and that these fractures occur in different locations from the original injury. They necessitate a cautious approach for the prevention of unfavorable outcomes. Further retrospective clinical and experimental studies are necessary to elucidate the fracture patterns and bone-healing quality of these recurrent mandible fractures.

Mandibular lengthening by distraction for airway obstruction in treacher-collins syndrome: the long-term results.

Mandibular lengthening by distraction was performed in a 6-year-old severely affected Treacher-Collins syndrome patient who was tracheostomy dependent. As previously reported, this procedure permitted tracheostomy removal once distraction was complete. Now that the patient is skeletally mature, the long-term results of this intervention are reported with regard to his clinical outcome and an assessment of the anatomical changes in the upper airway during growth. Although the distraction could be considered a success in that it enabled permanent decannulation and improved the minimum cross-sectional area of the upper airway, there was no further increase in the minimum cross-sectional area of the upper airway during childhood growth. It is significant that the abnormal growth pattern of the mandible, which is characteristic of this syndrome, did not alter from its preoperative pattern once distraction was completed.