Cimetidine for recurrent respiratory papillomatosis in pregnancy as an alternative adjuvant treatment.

BACKGROUND: We report the first case of cimetidine as an alternative adjuvant therapy in a pregnant woman with recurrent respiratory papillomatosis (RRP). A 40 year old woman at 19 week gestation presented with progressive hoarseness and shortness of breath for 1 month. Flexible nasopharyngolaryngoscopy revealed multiple papillomatous lesions over both vocal cords and subglottic area obstructing 60% of her airway. She had previously been diagnosed with juvenile onset RRP at the age of 5 and underwent endoscopic clearance regularly every 6 months. METHOD: The patient was started on a trial of oral cimetidine at a dose of 30 mg/kg and responded well, eventually requiring endoscopic excision only after 2 years. Subsequently, she underwent in vitro fertilisation treatment and stopped taking her cimetidine. After undergoing endoscopic clearance of her papillomata under general anaesthesia, she restarted on cimetidine during her 2nd and 3rd trimester. RESULTS: Ensuing follow-up demonstrated stable minimal papillomata lesions on her right inferior surface of her vocal cord with no recurrence on her left vocal cord and subglottic area. CONCLUSION: Cimetidine is generally safe and not known to be associated with any major teratogenic risks during pregnancy. RRP is postulated to worsen in pregnant women due to the increase in oestrogen levels during pregnancy. Hence, adjuvant therapy was imperative for our patient to reduce recurrent papillomata formation during her pregnancy. Larger scale studies are warranted to assess the use of long-term high-dose cimetidine in terms of efficacy and safety in pregnancy.

Findings from a Malaysian multicentre study on oropharyngeal squamous cell carcinoma.

BACKGROUND: In addition to the conventional aetiologic agents of oropharyngeal squamous cell carcinoma (OPSCC) such as tobacco usage, alcohol consumption and betel quid usage, it has been established that a proportion of OPSCC are driven by persistent oncogenic human papillomavirus (HPV) infections. Currently, there is a lack of data on the burden of HPV- associated OPSCC in Asian countries including Malaysia. METHODS: A cross-sectional multicentre study with tissue analysis of Malaysian patients diagnosed with primary OPSCC within a five-year period, from 2015 to 2019 between 01/01/2015 to 31/12/2019 was undertaken. Determination of HPV status was carried out using p16INK4a immunohistochemistry on tissue microarrays constructed from archived formalin-fixed paraffin-embedded tissue. RESULTS: From the cases identified, 184 cases had sufficient tissue material for analysis. Overall, median age at diagnosis was 63.0 years (IQR = 15) and 76.1% of patients were males. In our cohort, 35.3% of patients were Indian, 34.2% were Chinese, 21.2% were Malay and 9.2% were from other ethnicities. The estimated prevalence of HPV-associated OPSCC in our cohort was 31.0% (CI 24.4-38.2%). The median age for the HPV-associated OPSCC sub-group of patients was not significantly lower than the median age of patients with HPV-independent OPSCC. More than half of HPV-associated OPSCC was seen in patients of Chinese ethnicity (54.4%). Patients with HPV-associated OPSCC had a much better overall survival than patients with HPV-independent OPSCC (Log rank test; p < 0.001). Patients with HPV-associated OPSCC with no habit-related risk factors such as smoking, were found to have much better overall survival when compared to all other sub-groups. CONCLUSIONS: The findings from our study suggests that prevalence of HPV-associated OPSCC in Malaysia, though not as high as some developed countries, is however on an upward trend. HPV-associated OPSCC appears to be more frequently encountered in patients of Chinese ethnicity. Conventional risk-factors associated with OPSCC such as smoking, alcohol consumption and betel quid chewing should still be considered when estimating prognosis of patients with HPV-associated OPSCC.

Floor of Mouth Squamous Cell Carcinoma Presenting as an Abscess.

Oral squamous cell carcinoma (OSCC) is the most common malignancy encountered in oral cavity. Etiology is often multifactorial but strongly associated with chronic tobacco smoking, excessive alcohol consumption and tobacco chewing. Factors such as poor oral hygiene, ill-fitting dentures causing chronic mucosal irritation can also contribute to the cancer development. In recent years, studies shown association of HPV infections, especially subtypes 16 and 18 in malignant transformation of head and neck squamous cell carcinoma, as it present may change prognosis, outcomes and management strategies from HPV-negative OSCC. OSCC have variable presentation, and most of the patients tend to seek treatment at later stage of disease. The lesion may present as leukoplakia or erythroplakia and may be ulcerated with indurated base and rolled out edges. We describe an uncommon presentation of floor of mouth squamous cell carcinoma which presented as localized abscess.

A Novel Methylene Blue Guided Neopharynx Closure and a Review of Essential Applications in Otolaryngology.

Methylene blue has many usages in the field of otolaryngology. We describe a preliminary finding of methylene blue guided neopharynx closure after total laryngectomy. This technique can potentially reduce the risk of pharyngocutaneous fistula and has not been described in the literature. It is also handy in assisting junior surgeons to perform modified Connell suture during neopharynx reconstruction. Concurrently, we reviewed other essential applications of methylene blue in otolaryngology.

Isolated cervical lymph node sarcoidosis: a diagnostic challenge of a case report.

BACKGROUND: Sarcoidosis is a granulomatous disorder involving multi-systemic organs. Patients invariably have lung involvement but some may have extrapulmonary disease. Rarely, cervical lymphadenopathy is the only sign without mediastinal or pulmonary abnormalities. CASE PRESENTATION: We report a Malay male who complained of neck swelling exclusively. On imaging, multiple enlarged cervical lymph nodes deep to the sternocleidomastoid muscle were seen. An excision biopsy revealed non-caseating granulomas with epithelioid macrophages. Extensive investigations led to the diagnosis of isolated cervical lymph node sarcoidosis. CONCLUSIONS: Sarcoidosis can present as cervical lymphadenopathy alone, without mediastinal or lung disease. The presence of epithelioid granulomas on histopathology warrants the exclusion of other granulomatous diseases. Isolated cervical lymph node sarcoidosis is only diagnosed in the presence of consistent clinical and radiological findings. In this case, close monitoring for systemic sarcoidosis is important as it can manifest later in life.

A Single Institution Cadaveric Study on Anatomical Variation of the Sublingual Gland Duct.

The purpose of this study was to conduct a cadaveric dissection study on the anatomical variation of the sublingual (SLG) excretory ducts and dictate an improved understanding of the anatomical communication between the SLG duct and submandibular (SMG) ducts. This study is carried out by standardized dissection of anterior floor of mouth in 6 formalin-fixed adult cadavers in Silent Mentor Workshop University Malaya in August 2020. The cadavers had no trace of scars, adhesions, signs of trauma or operation. SMG duct opening was identified lateral to the lingual frenulum through a papilla in the floor of mouth behind the lower incisor tooth. A horizontal incision line was done over floor of mouth just lateral to the opening. SMG duct and SLG was traced and skeletonized. Any presence of major duct arising from the SLG and its communication with SMG duct were investigated. We found there are 3 patterns of SLG excretory duct variants; (1) One major (Bartholin's) duct which open independently at its own orifice adjacent to the orifice of the Wharton's duct of SMG. (2) One major (Bartholin's) duct which joined into the Wharton's duct of SMG. (3) Absent of a major duct arising from SLG. The overall mean diameter of SLG ducts were 1.3 ± 0.41 and the mean length of SLG ducts were 18.5 ± 6.55. The overall mean diameter of SMG ducts was 2.6 ± 0.74 and the mean length of SMG ducts were 46.5 ± 6.57. Excretory ductal system of SLG showed great variations, not only between the different cadavers but also within the different sides of the same cadaver. Awareness of potential anatomical variations can aid in the accurate diagnosis and treatment of patients with salivary gland pathology as well as help surgeons reveal potential risk factor and avoid complications during surgical procedures in the floor of mouth.

Five-year Survival Data on the Role of Endoscopic Endonasal Nasopharyngectomy in Advanced Recurrent rT3 and rT4 Nasopharyngeal Carcinoma.

AIM: Endoscopic endonasal nasopharyngectomy (EEN) for recurrent nasopharyngeal carcinoma (rNPC) is being increasingly used due to the added high magnification, reduced morbidities associated with open procedures and good survival outcomes. Most studies looked at usage of EEN in patients with lower recurrent staging (rT1 and rT2) although more and more surgeons are studying the outcome of EEN in advanced rNPC (rT3 and rT4). The aims of this study were to report the long-term 5-year survival outcome of EEN performed in patients with advanced rNPC, and to determine any prognostic factors for patients' survival. METHODS: All patients who underwent EEN for advanced rNPC between January 2003 and December 2015 inclusive were analyzed. All surgeries were performed in University Malaya Medical Centre in Kuala Lumpur and Queen Elizabeth Hospital in Sabah. We reported the 5-year overall survival (OS), disease-free survival (DFS) and disease-specific survival (DSS) and any related complications and significant prognostic factors. RESULTS: Twelve patients with rNPC (2 rT3 and 10 rT4) were followed-up over a mean duration of 44.8 months (range, 40-440 weeks). The 5-year OS was 50.0% (mean 44.75 months), DFS was 25.0% (mean 35.25 months) and the DSS was 58.3% (mean 43.33 months). No severe operative complications were encountered and no independent prognostic factors for survival outcome were identified. CONCLUSION: This is the first report in English that exclusively described the long-term 5-year survival data in patients with both rT3 and rT4 recurrent NPC after EEN. The data suggest that EEN is a feasible treatment to improve survival with minimal morbidities in patients with rT3 and rT4 recurrent NPC. However, more studies with larger patient size is recommended.

Acoustic neuroma with orofacial paresthesia: description of an atypical presentation.

Acoustic neuroma (AN) usually manifests with asymmetric hearing loss, tinnitus, dizziness and sense of disequilibrium. About 10% of patients complain of atypical symptoms, which include facial numbness or pain and sudden onset of hearing loss. Patients with atypical symptoms also tend to have larger tumours due to the delay in investigation. We report a particularly interesting case of a patient presented to us with numbness over her right hemifacial region after a dental procedure without significant acoustic and vestibular symptoms. Physical examination and pure tone audiometry revealed no significant findings but further imaging revealed a cerebellopontine angle mass. The changing trends with easier access to further imaging indicate that the presentation of patients with AN are also changing. Atypical symptoms which are persistent should raise clinical suspicion of this pathology among clinicians.

Primary Adenotonsillar Tuberculosis in an Immunocompetent Child -A Case Report.

INTRODUCTION: Primary tuberculosis (TB) of the oropharynx and nasopharynx is an extremely rare form of extra-pulmonary TB in children. Primary tuberculosis occurs more likely secondary to pulmonary TB and is more common in immunocompromised patients. CASE REPORT: We reported the case of a young male presented with the symptoms of non-specific chronic adenotonsillitis, mild obstructive sleep apnoea, and cervical lymphadenopathy. Subsequently, he underwent adenotonsillectomy and excision of the cervical lymph node with the tissue specimens came back strongly positive for TB. Then, he started using antituberculous medication and recovered well. CONCLUSION: The authors would like to highlight this rare clinical entity in which accurate diagnosis is essential for complete treatment.

Neck stiffness in a post-irradiated nasopharyngeal carcinoma adult: An unusual diagnosis.

Nasopharyngeal carcinoma is a neoplasm commonly found in population of South East Asia. The mainstay of treatment is high dose irradiation. Complications from radiotherapy are not uncommon especially to those nearby structures such as vertebrae and spinal cord. A 57 year-old gentleman with nasopharyngeal carcinoma (NPC) who was treated with chemo-radiation (total of 35 fractions,70Gy) presented to us 6 months post therapy with bilateral nasal discharge and progressive neck stiffness. Nasoendoscopy showed inflamed nasophayngeal mucosa and Computed Tomography (CT) brain and cervical spine showed retropharyngeal and anterior epidural collection with extension into atlantoaxial bone and spinal cord compression. Histopathological specimen revealed features of chronic inflammations with multiple actinomycetes colonies. Our patient suffered severe neck stiffness and loss of sensations on both upper limbs. He was treated conservatively with Halo vest and intravenous antibiotics for 8 weeks and recovered fully. Irradiation in NPC is known to cause devastating complications to cervical spine such as osteoradionecrosis, osteomyelitis. It also renders tissues hypoxic and risk of getting rare infection like actinomycosis. This report can represent a great diagnostic and therapeutic challenge with differentials of tumor recurrence, osteoradionecrosis or osteomyelitis. Patients must be regularly followed up to look for possible cervical complications as a result from irradiation, to prevent devastating outcome or prognosis.

Congenital nasal cavity stenosis in children with craniosyntosis: A report of 4 rare cases.

Congenital bony nasal stenosis (CBNS) is a very rare but life-threatening cause of airway obstruction in neonates and infants. This review aims to assess the presentation and early airway management of 4 new cases of craniosynostosis with bilateral nasal cavity stenosis. Patients were treated with endoscopic endonasal widening of the nasal cavity and stenting. All patients were extubated well post-operatively with resolution of symptoms. They remained asymptomatic with stents in situ for at least 6 months with no complications reported. Minimally invasive endoscopic endonasal widening of the nasal cavity with stenting is an effective and safe way of addressing nasal cavity stenosis.

A preliminary report on the role of endoscopic endonasal nasopharyngectomy in recurrent rT3 and rT4 nasopharyngeal carcinoma.

Endoscopic endonasal nasopharyngectomy (EEN) has become increasingly used for recurrent nasopharyngeal carcinoma (rNPC) due to reduced functional and cosmetic morbidities compared to conventional external approach. Majority of the existing studies on EEN focused on patients with lower recurrent staging of rT1 and rT2. The aims of this study were to provide a preliminary report on the outcome of EEN performed in patients with advanced (rT3 and rT4) rNPC, and to determine the prognostic factors for patients' survival. All patients who underwent EEN for rNPC between January 2003 and December 2015 inclusive were analyzed. All surgeries were performed in University Malaya Medical Centre in Kuala Lumpur and Queen Elizabeth Hospital in Sabah, by a single surgeon. We reported the 2-year overall survival (OS), disease-free survival (DFS) and disease-specific survival (DSS) and any related complications and significant prognostic factors. Fifteen patients with recurrent NPC (2 rT3 and 13 rT4 tumours) underwent EEN over the 13 years period. The mean age was 50.4 years (range 30-65) and the mean follow-up period was 28.7 months (range 9-81 weeks). The 2-year OS, DFS and DSS were 66.7 % (mean 19.4 months), 40 % (mean 15.7 months) and 73.3 % (mean 20.2 months), respectively. No severe operative complications were encountered. No independent prognostic factors for survival outcome were identified. This is the first preliminary report in English that exclusively looked at the use of EEN in advanced rT3 and rT4 NPCs, showing favourable patient outcome. However, further long-term follow-up of patients is required.

HOPX functions as a tumour suppressor in head and neck cancer.

Head and neck squamous cell carcinoma (HNSCC) is generalized term that encompasses a diverse group of cancers that includes tumours of the oral cavity (OSCC), oropharynx (OPSCC) and nasopharynx (NPC). Genetic alterations that are common to all HNSCC types are likely to be important for squamous carcinogenesis. In this study, we have investigated the role of the homeodomain-only homeobox gene, HOPX, in the pathogenesis of HNSCC. We show that HOPX mRNA levels are reduced in OSCC and NPC cell lines and tissues and there is a general reduction of HOPX protein expression in these tumours and OPSCCs. HOPX promoter methylation was observed in a subset of HNSCCs and was associated with a worse overall survival in HPV negative tumours. RNAseq analysis of OSCC cells transfected with HOPX revealed a widespread deregulation of the transcription of genes related to epithelial homeostasis and ectopic over-expression of HOPX in OSCC and NPC cells inhibited cell proliferation, plating efficiency and migration, and enhanced sensitivity to UVA-induced apoptosis. Our results demonstrate that HOPX functions as a tumour suppressor in HNSCC and suggest a central role for HOPX in suppressing epithelial carcinogenesis.