[Recurrent respiratory papillomatoses despite vaccination]. / Larynxpapilloom ondanks vaccinatie.

BACKGROUND: Recurrent respiratory papillomatosis (RRP, also known as laryngeal papillomatosis) is a debilitating chronic disease induced by a human papilloma virus (HPV). Wart-like lesions develop in the airways. Patients suffer from dysphonia, coughing and ultimately dyspnea. There is no curative treatment. Recurrent surgical intervention is necessary to keep the airways free of disease. CASE REPORT: We describe a 25-year-old woman who developed RRP, despite having been vaccinated according to the national vaccination program. She underwent 11 surgeries in the past 5 years. CONCLUSION: RRP is an invalidating disease, necessitating repeated surgical interventions. Despite worldwide use and availability of adequate preventive HPV vaccines against this disease, the Dutch government choose to use a vaccine which does not prevent against this chronic disease.

Chronic rhinitis in South Africa - more than just allergy!

Chronic rhinitis is a troublesome condition for sufferers. It is tempting to label all patients with chronic nasal symptoms as having allergic rhinitis (AR), but many such patients have other causes of chronic rhinitis that need a specific diagnosis and management strategy. Even when the patient fully fits the definition of AR, their condition will be best served by combining medication with ongoing patient education.

Juvenile-Onset Recurrent Respiratory Papillomatosis Diagnosis and Management - A Developing Country Review.

Recurrent respiratory papillomatosis (RRP) is a condition caused by human papillomavirus (HPV), usually HPV types 6 and 11, which is characterized by recurrent papillomas of the respiratory tract, mainly the larynx. Patients usually present between the ages of 2 and 6 years. The initial presenting symptom is progressive dysphonia, followed by stridor and respiratory distress. Treatment consists of repeated microlaryngoscopic procedures to remove the papillomas as there is no cure. The poor availability and accessibility of appropriate healthcare services in developing countries are barriers to the early diagnosis and appropriate management of patients with juvenile-onset recurrent respiratory papillomatosis (JoRRP), requiring many patients to have a tracheostomy. The introduction of prophylactic vaccines that include HPV6 and HPV11 is necessary in order to reduce the incidence of JoRRP.

Knowledge of the audiological effects, symptoms and practices related to personal listening devices of health sciences students at a South African university.

OBJECTIVE: This study aimed to determine the knowledge of first year health sciences students at a South African university regarding hearing loss and symptoms attributable to personal listening devices and their practices concerning the use of personal listening devices. METHOD: This was a cross-sectional study carried out using an anonymous self-administered questionnaire. RESULTS: Of 336 students, 269 (80.1 per cent) completed the questionnaire. While most participants could identify symptoms that could be caused by extensive use of personal listening devices, almost 30 per cent did not know that it could cause permanent hearing loss. Personal listening devices were used by 90.7 per cent of participants, with 77.8 per cent having used them for more than five years. Use was at a high volume in 14.9 per cent of participants and for more than 2 hours per day in 52.7 per cent. CONCLUSION: The findings indicate the need for an educational programme to inform students as to safe listening practices when using personal listening devices.

3D printed titanium prosthesis reconstruction following subtotal maxillectomy for myoepithelial carcinoma - a case report.

We present the case of a 43 year old male with a myoepithelial carcinoma of the hard palate who underwent a subtotal maxillectomy, resulting in a significant midfacial defect. The defect was successfully reconstructed with a titanium prosthesis using Additive Manufacturing (AM), better known as 3D printing; the process used to manufacture the prosthesis being Direct Metal Laser Sintering (DMLS). A maxillary denture was fitted onto the titanium DMLS frame post-operatively. This method of reconstruction of a large midfacial defect proved to be successful both functionally and cosmetically, and resulted in a good quality of life 3 years post-operatively.

Assessment of metered-dose inhaler technique: A study at the pulmonology clinic of a tertiary hospital in the Free State, South Africa.

BACKGROUND: Poor pressurised metered-dose inhaler (pMDI) technique remains a challenge in the management of airway diseases. OBJECTIVES: To assess pMDI technique among respiratory outpatients and identify the main indications for pMDI use and factors associated with improper use. METHODS: This was a prospective, quantitative descriptive study conducted at the adult respiratory clinic of Universitas Academic Hospital in Bloemfontein, South Africa. A convenience sample of 100 participants was used. Each participant was interviewed and required to demonstrate the use of a placebo pMDI, either alone or with a large-volume spacer. Inhaler technique was evaluated according to the UK Inhaler Group standard for inhaler therapy. RESULTS: Chronic obstructive pulmonary disease and asthma were the most common indications for pMDI use. Of the 100 participants, 97 preferred a pMDI without a spacer (pMDI alone) and three preferred using the inhaler with a spacer. In the pMDI-alone group, 13 participants (13.4%) demonstrated correct technique and 65 (67%) made more than one error. CONCLUSION: Poor inhaler technique is common among respiratory outpatients. Every contact with the patient should be an opportunity to reinforce correct pMDI technique.

Recurrent parotid cavernous haemangioma in an adolescent, successfully treated with Propranolol.

INTRODUCTION: Parotid haemangiomas account for the majority of salivary gland tumours in children. This articles highlights the use of medical treatment for the treatment of parotid haemangioma. CASE SUMMARY: A 4 year old male underwent a superficial parotidectomy for a parotid mass that was found to be a a parotid haemangioma on histology. At the age of 14 years he presented again with a parotid mass. He had imaging which aided the diagnosis of a parotid haemangioma being made. He was treated with successfully propranolol over a period of 6 months. DISCUSSION: There are no clear guidelines for treatment of parotid haemangiomas. These lesions can represent a clinical challenge and optimal management is controversial. It was proposed that propranolol be used for treatment in this particular case. This is a non-invasive form of treatment with minimal side effects.

Age of diagnosis, incidence and prevalence of recurrent respiratory papillomatosis-A South African perspective.

OBJECTIVES: To determine the distribution of the age of diagnosis of recurrent respiratory papillomatosis (RRP) and to determine the incidence and prevalence of RRP in both adults and children in the Free State province of South Africa in order to provide data from a population in sub-Saharan African. DESIGN: Retrospective record review. PARTICIPANTS: All patients with RRP in the Free State province of South Africa between 2011 and 2015. MAIN OUTCOME MEASURES: Distribution of the age of diagnosis of RRP and incidence and prevalence of RRP. RESULTS: The best fitting mixture distribution for the age of diagnosis of RRP was a two-component mixture of log-normal distributions. Within the first component (JoRRP), the age of diagnosis was significantly lower in patients with human papillomavirus (HPV)11 disease (median 3.2 year) than those with HPV6 disease (median 5.6 years) (P = .021), while in the second component (AoRRP), there was no significant difference in the age of diagnosis between HPV11 disease (30.7 year) and HPV6 disease (median 44.0 years) (P = .0696). The incidence and prevalence of JoRRP were 1.34/100000 population/year and 3.88/100000 population, respectively, while the incidence and prevalence of AoRRP were 0.18/100000 population/year and 0.38/100000 population, respectively. CONCLUSION: Recurrent respiratory papillomatosis in the Free State province of South Africa is a disease with a predominantly juvenile onset, with AoRRP having a lower prevalence than in Europe. This is probably reflective of the situation in sub-Saharan Africa.

Determination of the complete genome and functional analysis of HPV6 isolate VBD19/10 from a patient with aggressive recurrent respiratory papillomatosis.

Human papillomavirus (HPV) types 6 and 11 are the aetiological agent of recurrent respiratory papillomatosis (RRP). The complete genome of an HPV6 isolate with a 170 base pair (bp) duplication identified within the long control region (LCR) from a patient with aggressive recurrent respiratory papillomatosis was determined. The promoter sequence from the HPV LCR including the 170 bp duplication was placed upstream of a heterologous reporter gene and the activity of the reporter gene product determined using transfected cells. In total, mutations were observed at 157 nucleotide positions of the complete genome and included nucleotide substitutions, deletions and insertions, resulting in amino acid changes at 43 residue positions. Reporter gene activity using an HPV-derived LCR region with a 170 bp duplication was significantly higher than that using an HPV-derived LCR region with no duplication within this region. The results suggest that novel HPV variants warrant further investigation for potential biomarkers of aggressive disease.

The incidence and prevalence of juvenile-onset recurrent respiratory papillomatosis in the Free State province of South Africa and Lesotho.

BACKGROUND AND OBJECTIVE: Although the estimated incidence and prevalence of juvenile-onset recurrent respiratory papillomatosis (JORRP) has been determined in countries in North America and Europe and in Australia, no studies have attempted to determine the incidence or prevalence of JORRP in African countries. The aim of this study is to determine the incidence and prevalence of JORRP in the Free State province of South Africa and Lesotho. METHODS: This was a retrospective study in which the records of all patients with JORRP from the Free State province of South Africa or Lesotho treated at Universitas Academic Hospital or by otorhinolaryngologists in private practice between 1 January 2011 and 31 December 2013 were reviewed. RESULTS: The estimated incidence and prevalence of JORRP in the Free State were 1.34 and 3.88 per 100,000 population respectively while the estimated incidence and prevalence in Lesotho were 0.49 and 1.04 per 100,000 population respectively. However, these figures are probably an underestimation. CONCLUSION: The incidence and prevalence calculated for the Free State were generally higher than those found in other studies, while those calculated for Lesotho was similar to those obtained in other studies.

Paediatric chronic suppurative otitis media in the Free State Province: clinical and audiological features.

BACKGROUND: Chronic suppurative otitis media (CSOM) is a chronic infection of the middle ear cleft. In sub-Saharan Africa >50% of cases occur in children <10 years of age. OBJECTIVES: To describe the otological, audiological and bacteriological findings in children with CSOM. METHODS: We conducted a prospective study at the Ear, Nose and Throat (ENT) Clinic at Universitas Academic Hospital between August 2009 and December 2010. We included all children with CSOM over this period. Patients underwent ENT and paediatric examination, and were tested for HIV. Pus swabs were taken after an ear toilet for routine microbiology, fungal and Mycobacterium tuberculosis culture. We performed audiological testing after the otorrhoea had resolved. RESULTS: Eighty-six children (113 ears) were included, with a median age of 4.6 years (range 1 - 12 years). The mean duration of otorrhoea was 161.7 weeks (range 4 - 572 weeks). Nine patients (10.5%) presented with coalescent mastoiditis and/or intracranial complications of CSOM. Of the 153 organisms identified; Gram-negative bacteria were present in 93 (82.3%) ears, with 94.8% of these being sensitive to quinolones. Only 1 case of tuberculous otitis media was identified. HIV infection was present in 54.6% of patients tested. There was a hearing loss in 44 (66.7%) of the tested affected ears. CONCLUSIONS: There was a long delay between the onset of symptoms and accessing ENT services. Most cases of CSOM were due to quinolone-sensitive Gram-negative aerobes. There was a high prevalence of cholesteatoma, hearing loss and other complications in children in this study.

The histology of nasopharyngeal masses: a comparison between HIV positive and HIV negative patients.

The aim of this study was to compare the histology of nasopharyngeal masses of HIV positive and HIV negative patients and to determine the prevalence of malignancy in nasopharyngeal masses in HIV positive patients. The records of all patients who had nasopharyngeal biopsies performed at the Department of Otorhinolaryngology, Universitas Academic Hospital between January 2006 and December 2011, were reviewed and 151 patients were identified. The HIV status of 110 of these patients was known: 78 (70.9 %) were HIV positive and 32 (29.1 %) were HIV negative. The CD4 count was known in 63 (80.8 %) of the HIV positive patients with the median CD4 count being 275 cells/µl (14-712 cells/µl). Most nasopharyngeal masses in HIV positive patients were benign. Malignancies were significantly more common in the HIV negative group than in the HIV positive group, with six (7.7 %) of the nasopharyngeal masses in the HIV positive group being malignant, while eight (25 %) of those in the HIV negative group were malignant. Most nasopharyngeal masses in HIV positive patients are due to lymphoid hyperplasia. The presence of large cervical lymphadenopathy should alert one to the possibility of a malignancy rather than a benign disease process.

Novel HPV-6 variants of human papillomavirus causing recurrent respiratory papillomatosis in southern Africa.

There is currently no information regarding the genetic diversity of HPV-6 variants circulating in South Africa. The aim of this study was to determine the HPV-6 variants affecting patients with recurrent respiratory papillomatosis, to determine whether mutations correlate with disease severity and identify molecular determinants of virulence with prognostic relevance. HPV-6 variants were identified based on genome changes within the 712-991 bp region encompassing the non-coding region (URR) of the genome, with variations in length resulting from insertions and duplications, and the 453-bp gene encoding the E6 protein. Based on manual comparison of sequence data from the URR, the isolates were identified as HPV-6a and HPV-6vc variants. Three novel HPV-6 variants were identified: one based on a mutation in the E6 region; two based on changes in the URR including a unique substitution detected in three isolates and an insertion and 170-bp duplication in the URR genome in one patient, who had clinical features of severe disease.

Mite and cockroach sensitisation in patients with allergic rhinitis in the Free State.

BACKGROUND: Studies in patients with allergic rhinitis living in the Free State have showed grass pollens to be the predominant allergens, with house-dust mite sensitisation being less prevalent than in the coastal areas and a low rate of sensitisation to the storage mite Lepidoglyphus destructor. No studies have been conducted on sensitisation to the other storage mites, spider mites or cockroaches. We aimed to determine the prevalence of sensitisation to various house-dust mites, storage mites, spider mites and cockroaches in patients with allergic rhinitis living in the Free State. METHODS: Fifty consecutive patients with allergic rhinitis attending the ear, nose and throat clinic at Universitas Hospital underwent skin-prick testing and/or ImmunoCAP RAST testing for common aero-allergens, house-dust mites, storage mites, spider mites and cockroaches. RESULTS: Forty-six per cent of patients were sensitised to one of the house-dust mites, with house mite sensitisation being significantly more common in patients who had previously lived at the coast. Storage mites were not common aero-allergens, while 46% of patients were sensitised to the spider mite Tetranychus urticae. Blatella germanica was the cockroach species to which patients were most commonly found to be sensitised on ImmunoCAP RAST testing, with 38% of patients being sensitised to this aero-allergen. CONCLUSIONS: House-dust mites, T. urticae and the cockroach B. germanica appear to be important aero-allergens in the Free State. Storage mite sensitisation is not common.

HPV types causing juvenile recurrent laryngeal papillomatosis in South Africa.

OBJECTIVES: To identify human papillomavirus (HPV) types associated with juvenile onset recurrent laryngeal papillomatosis (RLP) in southern Africa, to determine if there is a correlation between HPV type and disease aggressiveness and to determine the diagnostic and prognostic value of rapid molecular techniques for detection and typing of HPV using laryngeal biopsies. METHODS: Laryngeal biopsies from patients undergoing surgery for RLP were screened for HPV using conventional and real-time PCR techniques. Amplicons were sequenced to determine the HPV type involved. Clinical features were correlated with HPV type. RESULTS: HPV was identified in papillomata from 18 out of 19 patients. Only HPV-6 and HPV-11 were identified, with no co-infections. There was 100% concordance between conventional and real-time PCR techniques. Patients with HPV-11 disease required more procedures and tended to have higher Derkay scores than those with HPV-6 disease. The HPV types identified in our patients were genetically similar to HPV types from geographically distinct regions. CONCLUSIONS: RLP in our patient population appears to be exclusively due to HPV-6 or HPV-11. HPV-11 disease appears to be more aggressive than HPV-6 disease. Identification of the HPV types provides motivation for inclusion of vaccines against these types in vaccination programs to protect women against infection and subsequently reduce the incidence of RLP.

Pulmonary hypertension due to recurrent juvenile laryngeal papillomatosis.

Although pulmonary hypertension secondary to upper airway obstruction caused by adenotonsillar hyperplasia has been well described, the association between laryngeal papillomatosis and pulmonary hypertension has not previously been documented. We report three patients with pulmonary hypertension due to upper airway obstruction caused by laryngeal papillomatosis. Pulmonary hypertension can contribute to significant preoperative and postoperative morbidity and cause intraoperative complications. Preoperative diagnosis and treatment of pulmonary hypertension is therefore essential in these patients.

Incidence and management of acoustic neuromas in South Africa.

OBJECTIVES: To determine the extent to which the various treatment modalities are used in the management of acoustic neuromas treated in South Africa and to estimate the incidence of acoustic neuromas in South Africa. METHODS: A telephonic survey was conducted of all otorhinolaryngologists, neurosurgeons, and radiotherapy centers in South Africa to determine whether they had treated any patients with acoustic neuromas during the calendar year 2000. RESULTS: One hundred and fifteen patients with acoustic neuromas were treated in South Africa during the calendar year 2000, and 8 patients were referred to other countries for treatment. This resulted in an incidence of approximately 0.3 per 100,000 population per year. There was a marked difference in the incidence between the various racial groups: the incidence was at least 1.76 per 100,000 population per year among whites and at least 0.01 per 100,000 population per year among blacks. Of the 115 patients, 78 (68%) were treated surgically and 24 (21%) by stereotactic radiotherapy. Fourteen (12%) patients had newly diagnosed acoustic neuromas that were managed conservatively in the year concerned. The surgical approach used was middle fossa in 8 (7%) patients, retrosigmoid in 61 (53%) patients, and translabyrinthine in 9 (8%) patients. CONCLUSIONS: The majority of acoustic neuromas in South Africa were treated surgically, mainly by a retrosigmoid approach. The incidence of acoustic neuromas in South Africa was approximately 0.3 per 100000 population per year. There were significant racial differences in the incidence.

Orbital rhabdomyosarcoma presenting as an apparent orbital subperiosteal abscess.

We present a patient with acute sinusitis whose CT scan showed a ring-enhancing lesion within the orbit typical of an orbital subperiosteal abscess. On exploration of the orbit, there was no pus present but a tumour was found, which on histological examination was found to be a rhabdomyosarcoma. The clinical signs and CT characteristics of orbital subperiosteal abscesses and rhabdomyosarcomas are discussed. A ring-enhancing lesion within the orbit on CT scan should not be regarded as being pathognomonic for a subperiosteal abscess and the possibility of other diagnoses should be considered.

A precricoid swelling in a patient treated with Teflon injection in the vocal fold after idiopathic left vocal fold palsy.

Vocal fold palsy is a cause of dysphonia. Due to incomplete glottic closure during phonation, patients with a unilateral vocal fold palsy present with a weak and breathy voice and recurrent aspiration. To lessen the clinical manifestations of unilateral vocal fold palsy, polytetrafluoroethylene (Teflon) paste is one agent which has been injected into the paraglottic region, thus causing the vocal fold to move more medially. One of the complications associated with Teflon paste injection is migration of the paste into the surrounding tissues. We present a patient with idiopathic left vocal fold palsy who underwent Teflon injection to the vocal fold and subsequently developed a precricoid nodule, mimicking a cartilaginous swelling.