Although early complication of airway obstruction following pharyngoplasty is well recognised, there have been few reports of late modifications following this procedure. We retrospectively review cases with late complications which have required either revision or division of an existing pharyngoplasty at the Australian Craniofacial Unit over the last twenty-five years. We assess the outcome of further surgical intervention in each case, with case note and nasendoscopy video review. Fourteen cases were identified where records were complete. There were 12 males and 2 females. The cases are a heterogeneous group of cleft lip and palate patients and include three cases with a diagnosis of Pierre-Robin sequence and one case with a cleft palate as part of an underlying syndrome. Those cases requiring flap division had undergone either superiorly or inferiorly based pharyngeal flaps in contrast to dynamic (Orticochea) pharyngoplasties which required revision. This series of cases demonstrates the need for thorough assessment and planned tailoring of the pharyngoplasty procedure, with ongoing review of speech and airway function. This management philosophy results in the acceptance that a pharyngoplasty may only be required for a limited period of time and ultimately may be redundant.

The concept of the cantilever bone graft, popularised by Millard (1966), and its subsequent modification by Chait et al. (1980), led to the idea of the costochondral cantilever graft for nasal dorsum augmentation. Over 150 costochondral nasal grafts have been performed at the Australian Craniofacial Unit (ACFU) over the last 25 years, and the aim of this study was to review a cohort of fractured costochondral graft in patients treated at the ACFU. The notes of patients with nasal costochondral grafts were reviewed, those with a fractured graft forming the basis of this study. Patients with Binder Syndrome accounted for nearly 30% of the cohort. Other diagnoses included Opitz syndrome, frontonasal dysplasia and other craniofacial abnormalities. 3 patients were identified with late fractured costochondral grafts. The prominent position of the nose makes it easily susceptible to trauma, and very often, low energy impact can produce fractures of the nasal bones. To the best of our knowledge, this is the first reported series of fractures of costochondral nasal grafts and their subsequent management. We would advocate early surgical management of the fractured grafts when conservative treatment has failed. In our present series, all 3 patients still maintained a satisfactory cosmetic and functional result after a mean of 8.5 years following the reparative surgery.

Apert syndrome is a rare acrocephalosyndactyly syndrome characterised by craniosynostosis, midface hypoplasia and syndactyly of the hands and feet. The majority of cases arise as the result of one of two mutations of the fibroblast growth factor receptor 2 gene (FGFR2). Due to the involvement of both the cranial and the facial sutures, the keystone of the craniofacial skeleton, the sphenoid bone, is affected by the disease process and as a result is dysmorphic. This may significantly affect craniofacial morphology but it is recognised that there are marked variations in this between different affected individuals. This is a retrospective study examining the morphology of the sphenoid bone using three dimensional reconstructions of computed tomography (CT) scan data. Shape analysis was performed using generalised Procrustes analysis and principal component analysis (GPA/PCA). Comparisons were made between the individuals with Apert syndrome and a group of normal individuals, and between the two genotypic groups. The sphenoid bone in those with Apert syndrome showed marked differences in morphology compared to the normal individuals with a restriction in height and increased angulation of the lesser wings; however, there were no consistent differences between the two genotypic groups. It is possible that fronto-orbital advancement (FOA) surgery indirectly releases the sphenoid bone and allows compensatory growth in this direction.
Acrocephalosyndactylia

The function of a genioplasty is to produce an aesthetically pleasing chin contour and improve facial proportions. The aim of this study was to review the role of osseous genioplasty in the management of patients with craniofacial deformities. 52 patients (24 males and 28 females) treated at the Australian Craniofacial Unit in Adelaide, Australia over a 25-year period; who required a genioplasty as part of their craniofacial management were reviewed. Patients ranged from 17-44 years (median: 26 years) and the age at which the patients underwent genioplasty was between 9 and 36 years (median: 17 years). Pre and 6 months post op cephalograms were compared, showing a mean chin advancement of 7mm and a mean osseous resorption of 20%. One patient was under-corrected and another had post-operative asymmetry, both requiring repeat genioplasty. No long-term nerve dysfunction was noted. The osseous genioplasty is an effective procedure for correcting the chin deformity often seen in patients with craniofacial abnormalities. It is an easy technique to master and is associated with a low degree of morbidity.

The free muscle-sparing transverse rectus abdominis myocutaneous (MS-TRAM) and deep inferior epigastric perforator (DIEP) flaps involve transferring skin and subcutaneous tissue from the lower abdominal area and have many features that make them well suited for breast reconstruction. The robust blood supply of the free flap reduces the risk of fat necrosis and also enables aggressive shaping of the flap for breast reconstruction to optimize the aesthetic outcome. In addition, the free MS-TRAM flap and DIEP flap require minimal donor-site sacrifice in most cases. With proper patient selection and safe surgical technique, the free MS-TRAM flap and DIEP flap can transfer the lower abdominal skin and subcutaneous tissue to provide an aesthetically pleasing breast reconstruction with minimal donor-site morbidity.
Breast ; Diclofenac ; Fat Necrosis ; Female ; Free Tissue Flaps ; Mammaplasty ; Mastectomy ; Patient Selection ; Rectus Abdominis ; Skin ; Subcutaneous Tissue

No abstract available.
Anesthesia* ; Dexmedetomidine* ; Lidocaine* ; Spine*

OBJECTIVE: Clinicopathological studies over the last decade have broadened the clinical spectrum of progressive supranuclear palsy (PSP) to include several distinct clinical syndromes. We examined the cognitive profiles of patients with PSP-Richardson's syndrome (PSP-RS) and two atypical ‘brainstem predominant' PSP phenotypes (PSP-parkinsonism, PSP-P; and PSP-pure akinesia with gait freezing, PSP-PAGF) using a comprehensive neuropsychological battery. METHODS: Fourteen patients diagnosed as PSP-RS, three patients with PSP-P and four patients with PSP-PAGF were assessed using a comprehensive battery of neuropsychological tests.
Cognition ; Executive Function ; Freezing ; Gait ; Humans ; Neuropsychological Tests ; Neuropsychology ; Paralysis ; Phenotype ; Prospective Studies ; Supranuclear Palsy, Progressive

BACKGROUND/AIMS: Nocturnal reflux is a largely undiagnosed and unmanaged condition predisposing to multiple esophageal complications. We evaluated the effects of rabeprazole and pantoprazole on nocturnal intragastric pH and gastric acid output during Day 1 of therapy following the consumption of standard meals. METHODS: The study had a double-blinded, randomized, two-way crossover design, and involved 15 patients with a history of mild reflux. Following an overnight fast, patients were given either rabeprazole (20 mg) or pantoprazole (40 mg) prior to the first of three standard Western meals. They then underwent overnight continuous intragastric pH monitoring and gastric acid output measurement. The drug effect was analyzed using a two-treatment, two-period crossover mixed model. RESULTS: The percentage of time during which the mean intragastric pH was greater than 4.0 and gastric acid output was less than 2.0 was higher for oral rabeprazole (p<0.05). The inhibition of acid output was greater for rabeprazole at almost all time points. Furthermore, the mean time-matched pH values differed significantly over the first 8.3 hours (p<0.05). CONCLUSIONS: On day 1, oral rabeprazole inhibited acid output to a greater extent and for a longer period than pantoprazole, and the intragastric pH was significantly higher for rabeprazole than for pantoprazole over the first 8.3 hours.
2-Pyridinylmethylsulfinylbenzimidazoles ; Cross-Over Studies ; Gastric Acid ; Humans ; Hydrogen-Ion Concentration ; Meals

PURPOSE: The response to haloperidol as a first-line neuroleptic and the pattern of neuroleptic rotation after haloperidol failure have not been well defined in palliative care. The purpose of this study was to determine the efficacy of haloperidol as a first-line neuroleptic and the predictors associated with the need to rotate to a second neuroleptic. MATERIALS AND METHODS: We conducted a retrospective review of the charts of advanced cancer patients admitted to our acute palliative care unit between January 2012 and March 2013. Inclusion criteria were a diagnosis of delirium and first-line treatment with haloperidol. RESULTS: Among 167 patients with delirium, 128 (77%) received only haloperidol and 39 (23%) received a second neuroleptic. Ninety-one patients (71%) who received haloperidol alone improved and were discharged alive. The median initial haloperidol dose was 5 mg (interquartile ranges [IQR], 3 to 7 mg) and the median duration was 5 days (IQR, 3 to 7 days). The median final haloperidol dose was 6 mg (IQR, 5 to 7 mg). A lack of treatment efficacy was the most common reason for neuroleptic rotation (87%). Significant factors associated with neuroleptic rotation were inpatient mortality (59% vs. 29%, p=0.001), and being Caucasian (87% vs. 62%, p=0.014). Chlorpromazine was administered to 37 patients (95%) who were not treated successfully by haloperidol. The median initial chlorpromazine dose was 150 mg (IQR, 100 to 150 mg) and the median duration was 3 days (IQR, 2 to 6 days). Thirteen patients (33%) showed reduced symptoms after the second neuroleptic. CONCLUSION: Neuroleptic rotation from haloperidol was only required in 23% of patients with delirium and was associated with inpatient mortality and white race.
Chlorpromazine ; Continental Population Groups ; Delirium* ; Diagnosis ; Haloperidol ; Humans ; Inpatients ; Mortality ; Palliative Care ; Retrospective Studies ; Treatment Outcome

Allelic loss of the short arm of chromosome 1 has been observed frequently in a wide spectrum of cancers, most frequently in oligodendroglioma. In our previous studies, we evaluated 177 oligodendroglial tumor samples and identified the AJAP1 gene (formerly Shrew1) in the consensus region of deletion. AJAP1 is a transmembrane protein found in adheren junctions and functions to inhibit glioma cell adhesion and migration. Whereas a putative tumor suppressor gene, we did not detect AJAP1 gene mutations. In subsequent studies, we found that AJAP1 was underexpressed in oligodendrogliomas relative to normal brain tissues. Bioinformatic analysis revealed the presence of CpG islands in the promoter of AJAP1. Methylation analysis of the AJAP1 promoter identified hypermethylation in 21% of oligodendrogliomas (n =27), and the degree of methylation correlated with low levels of AJAP1 expression (P = 0.045). The AJAP1 promoter was also highly methylated in a wide spectrum of cell lines (n = 22), including cell lines of glioblastoma. Analysis of the National Cancer Institute's REMBRANDT dataset, which contains 343 glioma samples, indicated that low AJAP1 gene expression was associated with decreased survival. Thus, both genetic (gene deletion) and epigenetic alterations (promoter methylation) are likely mechanisms that inactivate the putative tumor suppressor AJAP1 in gliomas, which contributes to poor prognosis.
Astrocytoma ; genetics ; metabolism ; pathology ; Cell Adhesion Molecules ; genetics ; metabolism ; Cell Line, Tumor ; Central Nervous System Neoplasms ; genetics ; metabolism ; pathology ; CpG Islands ; genetics ; DNA Methylation ; Down-Regulation ; Gene Deletion ; Glioblastoma ; genetics ; metabolism ; pathology ; Humans ; Oligodendroglioma ; genetics ; metabolism ; pathology ; Promoter Regions, Genetic ; genetics ; Survival Rate