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Biomedical subjects

David J David

Publications and source records attributed to David J David.

At least 19 recordsLinked to original sources

Spectrum of features in pterygium syndrome.

Pterygium syndrome is a complex and rare congenital deformity that consists of contractures involving multiple flexural surfaces and associated craniofacial anomalies. It often has associated conditions, including anomalies of the cardiovascular, respiratory, gastrointestinal and genitourinary systems. It may present in different forms, including multiple pterygium syndrome of Escobar, lethal multiple pterygium syndrome, popliteal pterygium syndrome, lethal popliteal syndrome (Bartsocas-Papas syndrome) and arthrogryposis multiplex congenita. The clinical presentation, multidisciplinary management and the long-term outcome in three patients with this condition are presented.

Abnormalities, Multiple↗

From birth to maturity: a group of patients who have completed their protocol management. Part II. Isolated cleft palate.

BACKGROUND: The optimal management of the cleft palate patient from birth to completion of treatment continues to present a formidable challenge to the plastic surgeon. The management by multidisciplinary teams is well established, but long-term outcome data of cases managed by protocol remain sparse. This study continues to present the results of the Australian Craniofacial Unit of patients with isolated cleft palate who completed protocol management at the unit under the care of the senior author (D.J.D.) during the 29-year period from 1974 to 2003. METHODS: A retrospective study of the outcomes in relation to facial growth, speech, hearing, and occlusion is presented of patients with an isolated cleft palate. RESULTS: Thirty-two cases were identified from the departmental database, involving 17 female patients and 15 male patients. Cephalometric analysis at skeletal maturity revealed a range of facial growth, and maxillary advancement surgery was deemed necessary in just two cases. Speech results were evaluated using speech therapy assessments during development and at maturity. At maturity, 18 of 32 patients were assessed as being within normal limits. The hearing was within -20 dB, with just two exceptions. CONCLUSION: Overall, these is a range of outcomes, but the results confirm that facial growth does not appear to be adversely affected by use of the pushback technique to reconstruct the palate.

Articulation Disorders↗

Pyrexia after transcranial surgery.

Pyrexia after transcranial surgery has been observed regularly in clinical practice but does not usually herald any subsequent pathologic process. However, the significance and incidence of this phenomenon remain uncertain. The aim of this study was to evaluate the incidence and timing of any pyrexia after transcranial surgery for craniosynostosis correction and correlate this with the clinical outcome to assess its significance. Retrospective review of sequential case notes collected over a 10 year period identified 136 transcranial operations undertaken for 122 cases of nonsyndromic craniosynostosis. The incidence of postoperative pyrexia of 38 degrees or more in the first 5 days was 76%, whereas that greater than 39 degrees was 11%. Pyrexia was noticed during the first 48 hours and had a bimodal distribution. Only a single case in this series subsequently developed a clinically significant complication, that is, a minor wound infection of the skin, which was treated by antibiotics and dressings. The occurrence of pyrexia did not appear to be related either to sex or to any affected suture but occurred less frequently in those who were under 6 months old. We conclude that this pyrexia should be considered to be a part of the normal physiological response to craniofacial surgery.

Adolescent↗

Scanning electron microscope and micro-CT evaluation of cranial sutures in health and disease.

Current knowledge of suture biology has been ascertained as a result of morphological studies of normal cranial sutures (and rarely those undergoing craniosynostosis). These were initially undertaken often using histological investigations, or more recently using CT scans, as investigative tools, but have often used animal models. However, recent technological advances have provided the potential to refine our understanding of the ultrastructure by the use of new advanced scanning technology, which offers the possibility of more detailed resolution. Our aim was to undertake detailed scans of normal, fusing and fused sutures from patients with craniosynosotosis affecting different sutures, to study the detailed structure at different stages of the fusion process using a modern micro-CT scanner and a microanalytical scanning electron microscope. We wished to include in our study all the human sutures because previous studies have mostly been undertaken using the sagittal suture. Ten sutures from seven patients have revealed a complex ultra-structural arrangement. The different patterns of bone ridging seen on the ectocranial and endocranial surfaces of the fused sagittal suture were not repeated on closer inspection of either fused coronal or lambdoid sutures. Elemental analysis confirmed that the amount of calcium increased and the amount of carbon decreased as sampled areas moved away from the suture margin. We conclude that scanning allowed detailed assessment and revealed the complex arrangement of the structure of the human cranial sutures and those undergoing the process of craniosynostosis, with some differences in final structure depending on the affected suture.

Child, Preschool↗

A three-dimensional computed tomographic analysis of the cervical spine in unoperated infants with cleft lip and palate.

OBJECTIVE: To investigate anatomical variations and abnormalities of cervical spine morphology in unoperated infants with cleft lip and palate. DESIGN: Retrospective cross-sectional investigation of infants born with nonsyndromic cleft lip and palate using computed tomography scans acquired for investigation of a spectrum of clinical conditions. SETTING: Computed tomography scan data were obtained from 29 unoperated cleft lip and palate infants and 12 noncleft infants of Malay origin, ages 0 to 12 months. METHODS: Observational study of cervical spine computed tomography scans. Heights of cervical vertebral bodies (C2-C7) and intervertebral spaces were measured from landmarks identified from computed tomography reformats and three-dimensional computed tomography reconstructions. Linear modeling of heights and spaces, with age as a covariate, was undertaken to identify differences between the samples. RESULTS: Anomalous features observed in the cleft lip and palate sample included short posterior arch of C1 (2/29), abnormal development of the anterior arch of C1 (2/29), and fusions of the posterior arch of C2 and C3 (2/29). No anomalies of the cervical spine were observed in the noncleft sample. Although the heights of three cervical vertebral bodies were significantly smaller and two intervertebral spaces were significantly larger in infants with cleft lip and palate compared with noncleft infants (p < .05), overall length of the cervical spine did not differ significantly between the samples. CONCLUSION: There was evidence for subtle upper spinal anomalies in the infant cleft lip and palate population. Our finding of reduced size of some cervical vertebral bodies may reflect delayed upper spinal development in infants with cleft lip and palate.

Cervical Vertebrae↗

The hyoid bone in malay infants with cleft lip and palate.

OBJECTIVE: To compare morphological and positional variations of the hyoid bone in unoperated infants with cleft lip and palate (CL/P) with those in noncleft infants. DESIGN: Retrospective, cross sectional. PATIENTS AND METHODS: Three-dimensional computed tomography scans were obtained from 29 unoperated CL/P infants of Malay origin aged between 0 and 12 months and from 12 noncleft infants in the same age range. Observations were made and measurements were obtained with a software package developed at the Australian Craniofacial Unit. The sizes of the hyoid bones and the position of the hyoid body and epiglottis in relation to the cervical spine were measured. Anatomical anomalies of the hyoid and prevalence of aspiration pneumonia were also documented. RESULTS: The hyoid bones and epiglottis were found to be located more inferiorly in CL/P infants compared with the noncleft infants. Also, 17% (5/29) of the CL/P infants had nonossified hyoid bodies. CONCLUSION: The results suggest that there are differences in the location and genesis of the hyoid bone in infants with CL/P that warrant further investigation.

Age Distribution↗

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.

Accidents, Traffic↗

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.

Acrocephalosyndactylia↗

Familial Parry-Romberg disease.

Parry-Romberg disease (or hemifacial atrophy) is a rare condition affecting the face. It commences in childhood but its aetiology remains unknown, and is sporadic. Two cases are presented who were biological first cousins. We believe that this is the first recorded example of this condition occurring in family members.

Adolescent↗

Ophthalmic sequelae of Crouzon syndrome.

PURPOSE: To document the frequency of ophthalmic sequelae in patients with Crouzon syndrome before the influence of craniofacial surgery. DESIGN: Retrospective observational case series. PARTICIPANTS: Seventy-one consecutive patients with a clinical diagnosis of Crouzon syndrome assessed before craniofacial or ophthalmic surgery at the Australian Craniofacial Unit between 1984 and 2000. METHODS: Review of clinical records with documentation of patient age, gender, visual acuity, refractive error, diagnosis of amblyopia, squint, eye movement dysfunction, nystagmus, fundus examination, examination of the anterior segment, interpupillary distance, and intercanthal distance. MAIN OUTCOME MEASURES: The frequency of ophthalmic signs and visual impairment, defined as a visual acuity of 6/12 or less. RESULTS: Visual impairment in at least 1 eye occurred in 35% of patients and was bilateral in 9%. The most common cause of visual impairment was amblyopia, which was present in 21% of patients, followed by optic atrophy in 7%. Ametropia occurred in 77% of patients; 57% had hypermetropia of > or =+2 diopters (D) and 20% had myopia of > or =-0.5 D. Strabismus occurred in 39% of patients. Although exposure keratopathy was observed in 15% of patients, this complication was well managed and caused no reduction in visual acuity. CONCLUSIONS: Early detection to reduce amblyopia by correction of refractive errors, timely treatment of strabismus, and patching should be a priority for ophthalmologists and a goal of the craniofacial teams managing patients with Crouzon syndrome. Optic atrophy remains an important cause of visual impairment in these patients before decompressive craniectomy.

Adolescent↗

Late results after unicoronal craniosynostosis correction.

The authors report nine patients with unicoronal synostosis who were treated with the same surgical protocol and who now have reached skeletal maturity. These patients all underwent surgery at the Australian Craniofacial Unit during a 2-year period by one of two craniofacial surgeons and one of two neurosurgeons. The operative procedure in all of these cases was the same technique, in which there was unilateral advancement of the affected side. No cases required reoperation; however, one case subsequently required revision of the coronal scar, and two cases required strabismus correction. All cases were reviewed to evaluate patient results by the clinical staff; recent photographs at skeletal maturity also were reviewed. Two patients had adult computed tomography scans available for skeletal assessment, and one additional patient had undergone serial computed tomography scans during childhood. The patients also completed an anonymous questionnaire to ascertain their assessment of their appearance. The results of the clinical and radiologic assessments and questionnaire suggest that the operative procedure undertaken in these cases has produced satisfactory results in the long term, with few individuals requiring (or even considering) additional surgery, despite some persistent asymmetry.

Adult↗

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.

Adult↗

Spinal anomalies in Goldenhar syndrome.

OBJECTIVE: Goldenhar syndrome consists of the triad of craniofacial microsomia, occular dermoid cysts, and spinal anomalies. The exact nature of the spinal anomalies remains poorly defined in the existing craniofacial literature, possibly due to these anomalies being managed by orthopedic surgeons rather than by craniofacial surgeons. The aim of this study was to clarify the nature and extent of these spinal anomalies. METHOD: Review of case notes of patients who had their diagnosis confirmed following review by a clinical geneticist and in conjunction with radiographs (supplemented by three-dimensional computed tomographic [CT] scans where available). RESULTS: Seven patients fulfilled the entry criteria and had material available for study. A wide range of anomalies was present, including butterfly vertebrae; hemivertebrae, which produced secondary scoliosis; kyphosis; and rib anomalies. Anomalies occurred at all levels within the spine. CONCLUSION: The possibility of spinal anomalies at all levels of the spine should be considered by those treating cases of Goldenhar syndrome, because these anomalies cannot be predicted from the severity of the facial malformation.

Adolescent↗

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.

Adolescent↗

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.

Airway Obstruction↗

Intracranial volume measurement of metopic craniosynostosis.

The authors report 32 patients with nonsyndromic isolated metopic synostosis who have undergone evaluation of their intracranial volumes. Twenty-five were male and seven were female. The measured intracranial volumes were compared with normal age-corrected values established in the authors' unit, and any differences were noted. The authors found that, although there was a range of intracranial volumes, in the male patients, intracranial volumes were significantly smaller than those found in the normal population (P < 0.05). However, this result was not found in the smaller female sample. These results contrast with those of smaller earlier studies, but the authors conclude that intracranial volumes are smaller than average for age-corrected normal values; this finding highlights the need for volume expansion in conjunction with cranial reshaping.

Case-Control Studies↗

From birth to maturity: a group of patients who have completed their protocol management. Part I. Unilateral cleft lip and palate.

The optimal management of the cleft lip and palate patient from birth to completion of treatment presents a formidable challenge to the plastic surgeon and the associated health care system. The multidisciplinary team approach for the management of these patients is widely accepted. However, a paucity of literature exists discussing specific protocol management, interventions, and the long-term outcomes of patients who have completed a strict treatment protocol with a consistent multidisciplinary team. The aim of this study was to present the details of the specific management protocol at the Australian Craniofacial Unit for cleft lip and palate patients and to present a group of patients who have completed this specific protocol and discuss the details of their long-term care. During a 28-year period from 1974 to 2002, the records of 337 patients treated for unilateral cleft lip and palate were evaluated. Of these 337 patients, 22 have completed the same specific protocol management. The same surgeon (David, the senior author) has been responsible for performing all operative interventions and for overseeing the care of each of the 22 patients, ensuring that the treatment protocol has been executed appropriately and without deviation. The interventions and outcomes were analyzed on the basis of speech, hearing, nasal airway, occlusion, psychosocial adjustment, and appearance. Because of the large volume of data and potential differences in outcomes, the authors' intention is to present this as part I of a four-part series beginning with unilateral cleft lip and palate. The results of isolated cleft palate, isolated cleft lip, and bilateral cleft lip and palate will be presented as parts II, III, and IV, respectively. Speech results were assessed as normal speech, mild abnormality, or severe abnormality by objective measures, and intervention for velopharyngeal insufficiency was noted. Seventeen patients were rated as having normal speech. Four patients were rated as having mild speech abnormality, one patient was rated as having severe speech abnormality, and seven patients required surgery for velopharyngeal insufficiency. Hearing results were measured objectively, and good hearing results were obtained in 18 cases. Five patients required tympanoplasty. All patients required alveolar bone grafting. The high Le Fort I osteotomy was performed in six cases. Bimaxillary surgery was performed in one case. Of all the patients assessed from birth to maturity, 13 required between three and five surgical interventions, and nine required six operations or more. Further details and photographs of preoperative and postoperative examples are provided.

Adolescent↗