Patient motivation and satisfaction from orthognathic surgery-a case series.

OBJECTIVE: This study attempted to understand the specific motivations of patients who undergo orthognathic surgery and determine their satisfaction with the surgery. The study also assessed the prevalence of complications and how they may impact patient satisfaction. STUDY DESIGN: Patients who underwent orthognathic surgery at the University of California between 2016 and 2019 and had completed postoperative orthodontic treatment for ≥9 months were interviewed. They responded to an open-ended telephone interview regarding their motivations, satisfaction, and complications. RESULTS: The patients showed a high level of satisfaction with the surgery, but there were persistent complications that affected satisfaction. The predominant complication was paresthesia over the distribution of the inferior alveolar nerve. The majority of patients who reported prior headaches and temporomandibular joint problems described improvement in those areas. Comparing the patients' motivations before and after surgery showed that before surgery, patients reported functional concerns, whereas postoperatively they were much more likely to recall aesthetic reasons for the surgery. CONCLUSION: This study showed that although patients are generally satisfied after orthognathic surgery, patients need to be realistically informed of their expectations and adequately informed of possible complications.

Distribution and factors associated with salivary secretory leukocyte protease inhibitor concentrations.

OBJECTIVES: This cross-sectional study examined the distribution and correlates of salivary secretory leukocyte protease inhibitor (SLPI) concentrations within a multinational cohort of men. METHODS: Extracellular SLPI was measured in oral gargle cell supernatants of 378 men from three countries using an ELISA-based assay. Risk factor data were collected by a questionnaire. Factors associated with SLPI were assessed using linear and logistic regression for continuous and categorical SLPI, respectively. RESULTS: Among men aged 18-73 years, the median SLPI concentration was 492.0 ng ml-1 (range: 2.3-1919.9). In multivariable modeling, men in Brazil and younger men (18-30 years) were more likely to have higher levels of SLPI [adjusted odds ratio (aOR) 3.84; 95% confidence interval (CI): 1.94-7.59, and aOR 3.84; 95% CI: 1.98-7.43, respectively]. Men with a self-reported sexually transmitted diseases diagnosis in the past 6 months were more likely to have higher SLPI levels (aOR 2.98; 95% CI: 1.1-7.83) and men reporting bleeding/swollen gums were less likely to have higher SLPI (aOR 0.34; 95% CI: 0.15-0.79). Similar results were observed for linear regression models. CONCLUSIONS: Secretory leukocyte protease inhibitor concentrations varied significantly by country and decreased with increasing age. The interaction between SLPI, modifiable factors, and oral infections that influence cancer risk warrants further investigation.

A clinical study of Aicardi syndrome in Northern Ireland: the spectrum of ophthalmic findings.

PurposeAicardi syndrome is a rare disorder, affecting ~1 in 100 000 live births. Chorioretinal lacunae feature alongside agenesis of the corpus callosum and spasms in flexion to make up a diagnostic triad. Recently ophthalmic findings such as microphthalmia and optic disc anomalies have been recognised in association with Aicardi syndrome. This population study aims to determine the presence of ocular findings and identifies some novel associations in these patients.MethodsA retrospective review of charts for seven patients with Aicardi syndrome was carried out.ResultsThe incidence of Aicardi syndrome in Northern Ireland was found to be 1 in 110 000 live births. Four patients who had microphthalmus also had iris abnormalities; two patients with bilateral microphthalmus had partial aniridia and two patients with unilateral microphthalmus had iris coloboma in the same eye. Optic disc abnormalities were found in 11 eyes of six patients. Two patients were found to have areas of fibrovascular proliferation with a thickened white ridge and avascular zone beyond. Both of these patients developed retinal detachments.ConclusionsOur review of patients with Aicardi syndrome in Northern Ireland has revealed some novel clinical findings, including aniridia in two cases. We also found a higher than previously reported rate of excavated disc anomalies of 50% in our cohort. We found two cases of peripheral retinal dysplasia, which has not been previously reported. This finding was associated with microphthalmus and severe optic disc abnormalities, and we feel this warrants early EUA to enable early treatment and hopefully result in better visual prognosis.

Mesna-induced photodistributed dermatosis.

We present the case of a 41-year-old man who developed a photodistributed eruption 1 month after being started on 2-mercaptoethane sulfonate sodium (mesna) for treatment of cyclophosphamide-induced haemorrhagic cystitis. The patient had a background history of Wegener granulomatosis, and had been taking cyclophosphamide at a stable dose of 150 mg daily for the previous 3 years. Complete resolution of the rash occurred within 8 weeks of cessation of mesna. This is the first reported case, to our knowledge, of mesna-induced photodistributed dermatosis. Early recognition of this condition will ensure prompt cessation of the culprit medication, and consideration of alternative management of haemorrhagic cystitis.

A study of pre-operative type and screen in breast surgery: improved efficiency and cost saving.

INTRODUCTION: Patients undergoing major breast surgery have an almost negligible need for blood transfusions. However, type and screen requests are still routinely performed. This represents an inefficient utilization of resources and unnecessary workload for laboratory staff. The aim of this study was to ascertain whether pre-operative blood typing is justified in patients undergoing surgical procedures with an intermediate transfusion probability. METHODS: A retrospective analysis of all patients undergoing a mastectomy and axillary clearance, with or without breast reconstruction in the last 2 years was undertaken. The number of group and hold and cross-match samples that were performed were identified and compared to the number of patients requiring a blood transfusion. The overall cost of routine pre-operative blood typing was analysed. RESULTS: A total of 229 patients were identified. Of these, a group and hold was performed on 192 (83.8%) patients. Cross-matching was undertaken in thirty-one patients (13.5%). In total, five patients (2.1%) required transfusion. No patient was transfused intra-operatively. The overall cost of routine group and hold blood requests was 1,920 euros and of pre-operative cross-matching was 465 euros. Forty-seven units of blood was returned unused to the blood transfusion service at an estimated cost of 23,500 euros. CONCLUSION: The need for routine group and hold blood requests is not justified for patients undergoing elective breast surgery and represents a waste of clinical resources. A more targeted approach will not only reduce the demand on blood products but also reduce the associated costs to blood transfusion services.

Merkel cell carcinoma metastasis to the iris in a 23 year old female.

Merkel cell carcinoma (MCC) is a rare, aggressive neuroendocrine malignancy which predominantly affects elderly caucasians. The pathogenesis is poorly understood but ultraviolet light and immunosuppression have both been implicated. MCC most commonly arises on sun exposed areas of the head and neck or extremities and has a propensity for local recurrence and regional lymph node metastasis. It may present as a violaceous or non specific firm nodule or as a small plaque. Histological diagnosis can be difficult and electron microscopy or immunohistochemistry are frequently required in addition to light microscopy. Management is stage dependant and frequently involves wide surgical excision with or without chemotherapy or radiation therapy. Early diagnosis and complete surgical excision is associated with a favourable prognosis. However, aggressive tumours with regional recurrence or distant metastases result in a median survival of 9 months. Reports of primary MCC of the eyelids have been reported in the literature. Intra-ocular metastases to the choroid and ciliary body have also been reported.

Training in the private sector: what works and how do we increase opportunities?

In Australia 61% of elective surgery takes place in private hospitals where current opportunities for surgical education and training (SET) are limited. The situation will shortly be compounded because of the large increase in local medical graduates, many of whom will aspire to be surgeons. How and where to train these extra surgeons to meet the expanding needs of the community must be addressed. Two models of private sector training are reviewed both of which involved combined training in both private and public sectors. Two second-year (SET 2) positions were created from one public hospital SET position by using the private sector for 3.5 days per week for 3 months of a 6-month rotation. The second model was applicable to post-fellowship training with a fairly even split between public and private sector responsibilities. In the first year, four registrars shared the two 6-month rotations for the SET 2 position. Trainees did the required minimum procedures (range 109-139) with primary operating targets of 20-25% (range 21-32%). The post-fellowship position in colorectal surgery was greatly enhanced by the private sector involvement with regard to operating experience as well as meeting part of the remuneration of the trainee. Successful models for training within the private sector in Australia can be found. To expand training in the private sector there will need to be a cultural shift in the perceptions of surgeons, patients, administrators, and trainees. Funding for posts may be available to those private hospitals that can meet the Royal Australasian College of Surgeons' accreditation standards for posts and hospitals.

Postcataract surgery outcome in a series of infants and children with Down syndrome.

AIMS: To report the visual and refractive outcome and complications in children with Down syndrome undergoing cataract extraction. METHODS: The case notes of 18 infants and children with lens opacities and Down syndrome who underwent cataract extraction between January 1981 and August 2006 were reviewed. RESULTS: Over the 25-year study period, 7% (33 eyes) of paediatric eyes undergoing cataract extraction had Down syndrome. The average follow-up time was 11.2 (SD 7.5) years with a range of 2.5 months to 25 years. 25 were congenital, and eight were developmental lens opacities. 40% of patients attained a postoperative BCVA between 6/9 and 6/18. There was a large myopic shift of -7.96 (4.7) D for aphakes and -8.06 (7.4) D for pseudophakes with an average increase in axial length of 3.58 (3.14) mm. There was a 30% incidence of posterior capsular opacification (PCO) overall, 38% in eyes without a primary posterior capsulotomy. Five eyes developed aphakic glaucoma, one eventually necessitating an enucleation. Two patients had retinal detachments on follow-up. CONCLUSION: Cataract extraction in our population of children with Down syndrome is a safe and effective procedure with a very encouraging visual outcome.

Necrobiotic xanthogranuloma occurring in an eyelid scar.

We present a case report of necrobiotic xanthogranuloma (NXG) in a 76-year-old Caucasian lady occurring as a nodule in a blepharoplasty scar. NXG is a rare histiocytic disease with progressive orbital and systemic features. Management options of excision biopsy or chemotherapy are discussed.

Co-injection strategies to modify radiation sensitivity and tumor initiation in transgenic Zebrafish.

The zebrafish has emerged as a powerful genetic model of cancer, but has been limited by the use of stable transgenic approaches to induce disease. Here, a co-injection strategy is described that capitalizes on both the numbers of embryos that can be microinjected and the ability of transgenes to segregate together and exert synergistic effects in forming tumors. Using this mosaic transgenic approach, gene pathways involved in tumor initiation and radiation sensitivity have been identified.

Taxane-resistant lymphoepithelial-like carcinoma: excellent response to VIP chemotherapy.

BACKGROUND: Lymphoepithelial-like carcinoma is a rare tumour type. The optimal treatment for this disease is not known. No effective therapies are described in the literature. AIMS: This report describes a case of lymphoepithelial-like carcinoma and documents a therapeutic strategy which has proved effective. RESULTS: The patient was initially treated with a common platinum-based chemotherapy regimen incorporating a taxane (Carbplatin and Docetaxel). Disease stabilization initially occurred but the patient soon progressed. The patient was then treated with VIP chemotherapy and had a complete response. CONCLUSION: VIP chemotherapy appears to be an effective therapeutic strategy in lymphoepithelial-like carcinoma.

RetCam image analysis of optic disc morphology in premature infants and its relation to ischaemic brain injury.

AIMS: To assess optic disc characteristics in premature infants with and without ischaemic brain injury and to evaluate the role of optic disc morphology in dating the injury. METHODS: RetCam fundal images, cranial ultrasounds and magnetic resonance imaging (MRI) of 109 premature infants were analysed. The study cohort was divided into subgroups depending on the presence or absence of periventricular leucomalacia (PVL) and intraventricular haemorrhage (IVH). The control group consisted of infants with normal neuroimaging at term and 2 years of age. Using the image analysis software of the RetCam, optic disc diameter (ODD), optic disc area (ODA), and optic cup area (OCA) were measured at 33-34 weeks gestational age. As serial cranial ultrasonography had been performed, it was possible to date the brain injury in those infants with periventricular white matter (PVWM) damage. RESULTS: Although there was a trend towards reducing ODD, ODA, and OCA with increasing severity of IVH, only the IVH 4 group differed significantly from the controls for these parameters (p = 0.002, p = 0.02, and p = 0.04, respectively). 44.4% of infants with grade 4 IVH had small discs. Only one patient had a large cup in a normal sized disc; this patient had IVH 4. In patients with PVWM damage, the median time of insult was 27 weeks in those with small discs and 28 weeks in those with normal discs. This difference was not significant (p = 0.23). CONCLUSIONS: Premature infants with IVH 4 have an increased incidence of optic nerve hypoplasia. We found no association between disc morphology and timing of brain injury.

Ahmed valve drainage implant surgery in the management of paediatric aphakic glaucoma.

BACKGROUND: Paediatric aphakic glaucoma presents months or years after cataract surgery in children and is a major long term complication. The results of surgical treatment are poor and many children require multiple and repeat procedures with poor visual outcomes. METHODS: 13 children (19 eyes) had Ahmed valve implantation surgery, nine of the children had previous procedures such as cycloablation or trabeculectomy. Mitomycin was used at surgery in some patients and valve needling with Healon GV and 5-fluorouracil in some blebs after surgery. SF(6) gas was also used at the time of surgery in most children to reform the anterior chamber. RESULTS: 12 of the children (18 eyes) achieved intraocular pressure control of 15 mm Hg or less with a valve alone or with additional medical therapy. CONCLUSION: Ahmed valve implantation surgery alone or in combination with medical therapy is successful and safe in the management of paediatric aphakic glaucoma.

A retrospective review of visual outcome and complications in the treatment of retinoblastoma.

The aim of this study was to look at the visual outcome and treatment complications of children diagnosed with Retinoblastoma during the years 1985-2003 inclusive. A retrospective review of all patients records was performed. Patient characteristics, treatment methods and complications were recorded. Twenty eight children presented to Temple street Hospital between 1985-2003. Six of these infants had bilateral tumours. The mean age at presentation was 23.7 months. Sixty-nine percent presented with Leucocoria, of these 33% also had a squint. The mean duration of symptoms was only known in 58% and this figure was approximately 19.8 months. Enucleation was performed in 24 eyes of 24 patients. Three patients required adjuvant chemotherapy post enucleation. Two eyes was treated with external beam radiation and one eye with plaque radiotherapy. One eye (second eye) was treated with systemic chemotherapy and radiation. Five eyes of three patients were treated with systemic chemotherapy followed by adjuvant Argon laser, cryotherapy and diode laser to each eye.The complications of each treatment group was recorded. The visual outcome in the salvaged eyes was favourable. There were no deaths recorded. Though chemotherapy with adjuvant local treatments provide adequate treatment for early tumours, enucleation still plays a major role in the treatment of Retinoblastoma. The total eye salvage rate in this study was 29% with an enucleation rate of 90% in unilateral cases and 33% in bilateral cases. Sixty-six percent of bilateral eyes affected were salvaged. Seventy-one percent of tumours were diagnosed after a parent noticed a gross abnormality of the eye. This highlights the possible need for screening for retinoblastoma in the infant population.

LASIK surgery in children.

AIMS: To report success in the treatment of high myopia in children with LASIK. To report the visual results, complications and postoperative management of children with high myopia. METHODS: Six children (seven eyes) with high myopia were included in this series. Preoperative and postoperative refraction, visual acuity, and pachymetry were compared. RESULTS: Six children with high myopia ranging from -5.00DS to -16DS were treated. There were three males and three females. Five children had improved refraction and visual acuity post-LASIK. Age ranged from 2 to 12 years. Five of the children had unilateral amblyopia preoperatively. One had bilateral high myopia. CONCLUSION: High myopia in children may be treated safely and effectively with LASIK.

Genetic and functional analyses of FH mutations in multiple cutaneous and uterine leiomyomatosis, hereditary leiomyomatosis and renal cancer, and fumarate hydratase deficiency.

Germline mutations of the fumarate hydratase (FH, fumarase) gene are found in the recessive FH deficiency syndrome and in dominantly inherited susceptibility to multiple cutaneous and uterine leiomyomatosis (MCUL). We have previously reported a number of germline FH mutations from MCUL patients. In this study, we report additional FH mutations in MCUL and FH deficiency patients. Mutations can readily be found in about 75% of MCUL cases and most cases of FH deficiency. Some of the more common FH mutations are probably derived from founding individuals. Protein-truncating FH mutations are functionally null alleles. Disease-associated missense FH changes map to highly conserved residues, mostly in or around the enzyme's active site or activation site; we predict that these mutations severely compromise enzyme function. The mutation spectra in FH deficiency and MCUL are similar, although in the latter mutations tend to occur earlier in the gene and, perhaps, are more likely to result in a truncated or absent protein. We have found that not all mutation-carrier parents of FH deficiency children have a strong predisposition to leiomyomata. We have confirmed that renal carcinoma is sometimes part of MCUL, as part of the variant hereditary leiomyomatosis and renal cancer (HLRCC) syndrome, and have shown that these cancers may have either type II papillary or collecting duct morphology. We have found no association between the type or site of FH mutation and any aspect of the MCUL phenotype. Biochemical assay for reduced FH functional activity in the germline of MCUL patients can indicate carriers of FH mutations with high sensitivity and specificity, and can detect reduced FH activity in some patients without detectable FH mutations. We conclude that MCUL is probably a genetically homogeneous tumour predisposition syndrome, primarily resulting from absent or severely reduced fumarase activity, with currently unknown functional consequences for the smooth muscle or kidney cell.