A Veiled Lymphatic Malformation: Stridor in a Child.

Lymphatic malformation (LM) is a congenital lymphatic dysplasia associated with the p110α subunit of PI3K (PIK3CA) mutation. A two-year-old boy presented with a history of noisy breathing from the age of two months, which was progressively worsening. Inspiratory stridor was audible with subcostal recession. Flexible nasopharyngolaryngoscopy (FNPLS) revealed an enlarged right arytenoid. Other supraglottic structures were normal, and bilateral vocal cords were mobile. Direct laryngoscopy showed that the right arytenoid was enlarged with a smooth surface. On the subsequent visit, there was a painless soft lateral neck swelling, 4 cm x 4 cm in size, with normal skin. MRI confirmed LM with the predominantly macro-cystic component, involving primarily the right neck and upper mediastinum, causing airway compression. Sirolimus therapy was initiated, and at one month of follow-up after the treatment, his stridor had improved. The incidence of stridor secondary to head and neck tumors such as teratomas, hemangiomas, and LM accounts for less than 3%. The typical manifestation of LM often involves a painless, soft, and compressible mass that progressively increases in size. Features of macrocystic LM on MRI are multilocular and hyperintense cystic mass on T2-weighted imaging. The treatment methods for LM include surgical and non-surgical options. Despite being an off-label application, the response rate of sirolimus therapy in children with LM is reported to be 91%, and the first clinical response was observed in less than three weeks. Stridor is frequently encountered in children but rarely due to head and neck tumors. However, as in our case, a large LM may cause recurrent airway obstruction, and the neck swelling may appear later. Atypical airway findings, especially endoscopic examination, in a child with stridor should be complemented with imaging to examine the possibility of extra-laryngeal mass or external compression.

Instruments for evaluating the parental emotional status and ecological support systems among parents who considered cochlear implantation for their children with hearing loss: A scoping review.

OBJECTIVES: Parents of children diagnosed with severe-to-profound sensorineural hearing loss may experience a range of emotions owing to a lack of knowledge and experience in dealing with such children. However, most audiology clinics only attend to children with deaf and hard of hearing (DHH) and not their parents. Thus, parents' emotional and support needs are frequently excluded from the intervention sessions, making their own needs invisible. This study aimed to identify academic and clinical instruments used for assessing parental emotional status (PES) and ecological support systems (ESS) in early intervention and determine the factors affecting PES and ESS among parents of DHH children undergoing cochlear implantation. MATERIALS AND METHODS: This scoping review followed the rigorous methodological framework; searched Medline (via OVID and EMBSCO), Scopus, and Web of Science; and selected studies relevant to validated instruments used to evaluate the PES and ESS among parents of DHH children below 6 years old. Before selecting and reviewing relevant articles, two reviewers independently assessed article titles and abstracts from the data sources. Two reviewers verified half of the first reviewer's extracted data. RESULTS: Overall, 3060 articles were retrieved from the database search, and 139 were selected for full-text review following title and abstract reviews. Ultimately, this study included 22 articles. Among them, 23 and 12 validated instruments, most of which are generic measures, were used for assessing PES and ESS, respectively. Three condition-specific instruments were identified and designed to be administered following cochlear implantation surgery. CONCLUSIONS: This study revealed that healthcare professionals who interact with parents of DHH children lack the necessary instruments, particularly for parents of children undergoing cochlear implantation surgery. Therefore, it is necessary to develop condition-specific instruments for parents who consider cochlear implantation for their children.

Cochlear implantation in older adults: 15 years' experience of a tertiary centre in a developing country.

INTRODUCTION: Cochlear implant (CI) is a viable option of treatment for older patients with severe to profound deafness in resource-rich countries. Implantation is limited in developing countries. OBJECTIVE: To review factors and outcomes of elderly patients that underwent CI in a pioneer centre in a developing country. MATERIAL AND METHODS: An observational retrospective review of patients older than 60, implanted between 2005 to 2020. RESULTS: Eleven patients were included. Patient were aged 60-74 years old with median of 66 years old. Average duration of deafness prior to implantation is 22 years. All implantation was unilateral except for one case that was implanted sequentially after 5 years Three patients were privately funded. Analysis of the hearing aided level with CI and hearing aid showed substantial improvement provided by the CI. The Categories of Auditory performance (CAP-II) scale were in the range of 6-9. DISCUSSION: Cochlear implant is safe and stable intervention in providing improvement of hearing and self-esteem in the elderly patients. Social isolation and depression also improved with better hearing and communication. CONCLUSION: Awareness of the CI in elderly patients must be advocated among policy- makers, clinicians, and patients to mitigate the clinical and public health burden of hearing loss among older patients.

The Mysterious Whistling Breath: Foreign Body Aspiration.

Foreign body aspiration is commonly seen in the pediatric age group and can be a life-threatening condition. Typical presentations include coughing, wheezing, and choking and can often masquerade as asthma causing misdiagnosis and treatment delay. Most of the time, the actual aspiration event is unnoticed, and patients may remain asymptomatic until they present with recurrent infections with or without positive radiological findings. Aspirated objects tend to migrate distally, and organic objects may induce edema and inflammation. Diagnosis is crucial as near-total or total obstruction of the airway may cause asphyxia and, subsequently, death if no immediate intervention is taken.

Blue Parotid Unveiled: A Rare Case of Traumatic Hemorrhagic Parotid Lymphangioma in an Eight-Year-Old Boy.

Parotid lymphangioma is a benign lymphatic malformation commonly observed in infancy or early childhood. It often grows insidiously and presents as a painless, soft fluctuant mass. We report a case of an eight-year-old boy who was diagnosed from another center with right parotid lymphangioma of one-year duration. He presented with right painful preauricular swelling and trismus for nine days after a recent history of blunt trauma to the preauricular caused an acute expansion of the swelling and subsequently, the patient developed ipsilateral facial nerve palsy. Examination showed right preauricular swelling measuring about 6 x 6 cm that extended posteriorly until the mastoid region, superiorly until the zygoma, and inferiorly until the angle of the mandible, pushing the ear lobule anteromedially. There was bluish discoloration of the overlying skin. The swelling was warm and tender on palpation. Multiple shotty lymph nodes were palpable at the posterior triangle. Aspiration of the swelling revealed blood content, but it reaccumulated after a few hours. A magnetic resonance imaging (MRI) of the neck showed a lesion confined within the parotid gland. There was a presence of air-fluid level with dependent layers of hyperintense on the T1-weighted image (T1WI) and T2-weighted image (T2WI) with clumps of isointensity on T1WI, which are hypointense on T2WI, which is suggestive of acute-late subacute blood product. A diagnosis of lymphatic malformation complicated with hemorrhage was made. Hence, the patient underwent surgery for the evacuation of blood clot and right superficial parotidectomy. Histopathological examination of the intraoperative tissue biopsy revealed evidence of venolymphatic malformation of the parotid gland. Postoperatively, he was discharged home after three days. The facial nerve function recovered from House and Brackmann grade II to grade I three weeks after the surgery.

A Diagnostic Conundrum: Tuberculosis or Salmonella Neck Abscess?

Granulomatous neck abscesses are commonly associated with tuberculosis (TB). These chronic inflammatory reactions are rarely seen in Salmonella non-typhi (SN) infections. We report two cases of SN granuloma presenting as neck abscesses in poultry farmers. TB polymerase chain reactions (PCR) were negative. Histopathology reported necrotizing granulomatous inflammation. Salmonella species are known to cause true granulomas in bone marrow, liver, and spleen. To the best of our knowledge, true granulomas have not been described in cervical lymph nodes. The aim of this report was to highlight the importance of considering other causative microbiological agents in cases of granulomatous neck abscesses. The patients recovered after receiving treatment with surgical drainage and intravenous antibiotics.

Longitudinal outcomes for educational placement and quality of life in a prospectively recruited multinational cohort of children with cochlear implants.

OBJECTIVES: The aim of this study was to report on the educational placement, quality of life and speech reception changes in a prospectively recruited group of children after they received a cochlear implant (CI). METHOD: Data was collected on 1085 CI recipients of as part of a prospective, longitudinal, observational, international, multi-centre, paediatric registry, initiated by Cochlear Ltd (Sydney, NSW, Australia). Outcome data from children (≤10 years old) implanted in routine practice was voluntarily entered into a central, externally hosted, e-platform. Collection occurred prior to initial device activation (baseline) and at six monthly follow-up intervals up to 24 months and then at 3 years post activation. Clinician reported baseline and follow up questionnaires and Categories of Auditory Performance version II (CAP-II) outcomes were collated. Self-reported evaluation forms and patient information were provided by the parent/caregiver/patient via the implant recipient baseline and follow up, Children Using Hearing Implants Quality of Life (CuHIQoL) and Speech Spatial Qualities (SSQ-P) Parents Version questionnaires. RESULTS: Children were mainly bilaterally profoundly deaf, unilaterally implanted and used a contralateral hearing aid. Prior to implant 60% used signing or total communication as their main mode of communication. Mean age at implant was 3.2 ± 2.2 years (range 0-10 years). At baseline 8.6% were in mainstream education with no additional support and 82% had not yet entered school. After three years of implant use, 52% had entered mainstream education with no additional support and 38% had not yet entered school. In the sub-group of 141 children who were implanted at or after three years of age and were thus old enough to be in mainstream school at the three-year follow up, an even higher proportion (73%) were in mainstream education with no support. Quality of life scores for the child improved statistically significantly post implant compared to baseline and continued to improve significantly at each interval up to 3 years (p < 0.001). Parental expectation scores reduced statistically significantly from baseline compared to all intervals (p < 0.028) and then increased significantly at 3 years compared to all post baseline follow-up intervals (p < 0.006). The impact on family life was reduced post implant compared to baseline and continued to reduce between annual intervals (p < 0.001). At three years post follow up median CAP II scores were 7 (IQR 6-7) and mean SSQ-P scores were 6.8 (SD1.9) 6.0 (SD1.9) and 7.4 (SD 2.3) for speech spatial and qualities scales respectively. SSQ-P and CAP II scores improved statistically and clinically significantly compared to baseline by one year post implantation. CAP II scores continued to improve at each test interval up to three years post implant. Speech and Qualities scores improved significantly between years 1 and 2 (p < 0.001), but only the Speech scores improved significantly between years 2 and 3 (p = 0.004). CONCLUSIONS: Mainstream educational placement was achievable for most of the children, including those implanted at an older age. Quality of life for the child and the wider family improved. Future research could focus on the impact of mainstream school placement on children's academic progress, including measures of academic attainment and social functioning.

Where are the vesicles? A case report of Ramsay Hunt syndrome.

A 1-year-10-month-old child developed left-sided Ramsay Hunt syndrome (RHS) without vesicles 1 month after an episode of varicella zoster infection. No ear symptoms, including hearing loss, tinnitus, or imbalance, were reported. The external ear and otoscopic examinations were unremarkable. He achieved adequate recovery with corticosteroid treatment. This case report discusses the unusual presentation of RHS without vesicles, the diagnostic dilemma in young children, varicella zoster virus hepatitis, treatment modalities, and the role of vaccination in its prevention.

Case series of tuberculous otitis media: Spectrum of clinical presentation and outcome.

OBJECTIVE: Tuberculous otitis media (TB OM) is a rare disease. We present here our experience of TB OM on its clinical presentation, investigation and treatment outcome. METHODS: A retrospective observational study was performed and clinical data of patients in whom TB OM was diagnosed at our center between 2008 and 2019 was analyzed. RESULTS: Five cases of TB OM were identified. Otorrhoea was the most consistent presenting chief complaint while one of the cases presented with right post-auricular swelling. All the cases were successfully treated with anti-tuberculous medication with outcome of dry middle ear but only one case achieved full recovery of hearing loss. CONCLUSION: Due to the disparity of presentations in all our patients, we would like to emphasize the high index of suspicion of TB OM in acute or chronic cases of otitis media with unusual presentation or poor response to standard treatment.

A Review of Tracheal Bronchus in Universiti Kebangsaan Malaysia Medical Centre (UKMMC).

Tracheal bronchus (TB) is a rare congenital anomaly described as a abnormal bronchus that originates directly from the trachea above the carina directed towards the upper lung lobe. We analysed all paediatric rigid endoscopies of the airway from January 2015 until August 2020 to determine the incidence and characteristic of TB. In total, 68 rigid endoscopic airway examination record from children aged 0 to 12 years were analyzed. Endoscopic examination was performed from supraglottic region to carina using a 0 degree Hopkins rod lens telescope. Patients with a TB were identified and the site of TB origin was noted. Data of the identified patients was reviewed for the presence of preoperative airway findings such as stridor, upper lobe pneumonia, other congenital anomalies, intraoperative findings and complications and postoperative general condition outcome. TB was detected in 8 (11.8%) of 68 airway endoscopic examinations. 6 children (75%) were syndromic. 5 patients (62.5%) has congenital malacic airway and 2 patients (25%) has congenital tracheal stenosis. All TB originated from the right lateral wall of the trachea. All children had stridor unrelated to TB as presentation and 4 (50%) of children had preoperative upper lobe pneumonia. Tracheal bronchus is not a rare finding and is highly associated with syndromes and other airway anomalies. Although children with TB can be asymptomatic, upper lobe pneumonia is a common presentation. TB should be included in the differential diagnosis in patients with recurrent right upper lobe pneumonia or collapse and patients with unexplained oxygenation problem during endotracheal intubation, particularly in children with syndromes or other congenital anomalies.

Paediatric Adenotonsillectomy: Its Safety and Outcome in a Malaysian Tertiary Medical Center.

Adenotonsillectomy is the commonest otorhinolaryngology surgery for paediatrics. The purpose of this study was to determine the safety and outcome of adenotonsillectomy in a tertiary center in Malaysia. This was achieved by studying the demographics, indications, co-morbidities of patients and its association with the complications of the procedure. This retrospective study was conducted from November 2011 until October 2016. Medical records of patients aged 2-12 years old who had adenotonsillectomy done in our center were retrieved for data collection. A total of 143 patients who fulfilled the inclusion and exclusion criteria were recruited. Median age was 7.52 (± 2.98 SD) years old. Allergic rhinitis was the commonest co-morbidity (40%). Recurrent tonsillitis was the main indication (60%), followed by sleep disordered breathing (SDB) without apnea (29%), obstructive sleep apnea (OSA) (7%) and other indications (4%). Post-operative paediatric intensive care unit (PICU) admission was very low (1.4%) and was reserved for those with severe OSA with complications. None of the patients admitted to PICU post-operative had respiratory complications. The prevalence of post-tonsillectomy bleeding was only  2.1%. There was no significant association between patient's co-morbidity with operative complications (p = 0.269). Adenotonsillectomy is indicated for children with recurrent tonsillitis and paediatric SDB. The two most severe complications, namely post-tonsillectomy haemorrhage and respiratory complications occurred but incidence was low. Criteria of post-operative PICU or paediatric high dependency unit admission for paediatric SDB has to be based on multiple clinical and logistics factors and not only patients co-morbidity and indication of surgery.

Pediatric Langerhans Cell Histiocytosis: An Aggressive Presentation.

Encountering a young child with an enlarging painless facial swelling often raises concerns in the treating physician about the possibility of a congenital lesion or an unfavorable pediatric tumor. We discuss a case of a female child who presented with multiple craniofacial swellings, which turned out to be Langerhans cell histiocytosis (LCH). She was subsequently diagnosed with multisystem LCH (MS-LCH) with risk-organ involvement, which included the craniofacial bones, skin, hemopoietic system, and liver. We analyze the various presentations and systemic complications of this rare pediatric tumor, LCH, with an aim to address the diagnostic dilemma associated with this great masquerader.

Spectrum of upper airway pathology in children with Down syndrome in a single tertiary centre.

BACKGROUND: Down syndrome (DS) is the most common human chromosomal genetic disorder; caused by extra copy of chromosome 21-one out of 792 babies globally and one in 950 in Malaysia. OBJECTIVES: To obtain spectrum of upper airway pathology in DS children-prevalence, causes and management practices. MATERIALS AND METHODS: A cross-sectional study of children with DS aged less than 18 years old with signs of UAO was conducted. Diagnosis of UAO, management and final outcome was observed and documented. RESULTS: Ninety-one patients were recruited. 20 cases (22%) had significant noisy breathing which indicates the prevalence of upper airway pathology (p=.025). Laryngomalacia (34.5%) was the most common pathology, followed by paediatric SDB (24.1%), tracheal bronchus (17.2%), tracheal stenosis (6.9%), and tracheomalacia (6.9%). Seven cases (7.7%) presented with respiratory distress. 12 cases were managed conservatively, while eight cases required further investigations under general anaesthesia (GA) with/without surgical intervention. Six cases showed symptoms resolution within a week post-surgical intervention, and another case resolved after 30 days and one case resolved after 8 months of post-surgical intervention. CONCLUSION AND SIGNIFICANCE: Early detection and high index of suspicion is required to diagnose and treat these anomalies timely.

Postcricoid haemangioma with laryngomalacia in infancy.

Infantile haemangioma represents a congenital vascular anomaly commonly observed in the head and neck region. Such an occurrence over the postcricoid region, however, is rather unusual. Herein, the authors report a case of a synchronous postcricoid haemangioma in a 7-week-old newborn diagnosed with severe laryngomalacia. In addition to the floppy redundant arytenoid mucosa, flexible laryngoscopy revealed a lobulated bluish mass at the postcricoid. The lesion was hyperintense on T1-weighted sequence and was enhanced with contrast, supporting the diagnosis of a haemangioma. She underwent surgical excision of the haemangioma with intralesional steroid injection. Surveillance at 6-month postoperation did not show disease recurrence.

Case series of tuberculous otitis media: Spectrum of clinical presentation and outcome.

OBJECTIVE: Tuberculous otitis media (TB OM) is a rare disease. We present here our experience of TB OM on its clinical presentation, investigation and treatment outcome. METHODS: A retrospective observational study was performed and clinical data of patients in whom TB OM was diagnosed at our center between 2008 and 2019 was analyzed. RESULTS: Five cases of TB OM were identified. Otorrhoea was the most consistent presenting chief complaint while one of the cases presented with right post-auricular swelling. All the cases were successfully treated with anti-tuberculous medication with outcome of dry middle ear but only one case achieved full recovery of hearing loss. CONCLUSION: Due to the disparity of presentations in all our patients, we would like to emphasize the high index of suspicion of TB OM in acute or chronic cases of otitis media with unusual presentation or poor response to standard treatment.

Tracheal cartilaginous sleeve in Pfeiffer syndrome: lesson learnt from its rarity.

Pfeiffer syndrome is a rare inherited craniofacial disorder. Upper airway obstruction is common among patients with Pfeiffer syndrome due to craniosynostosis. They may also present with lower respiratory tract obstruction due to a rare congenital airway malformation called tracheal cartilaginous sleeve (TCS). We report the case of a patient with Pfeiffer syndrome who presented with recurrent bronchopneumonia, discovered incidentally to have TCS via direct visualisation during tracheostomy. Relevant literature for this rare clinical condition are reviewed and discussed. Clinicians should be aware of TCS when encountering patients with craniosynostosis who present with recurrent lower respiratory tract infections. Careful and meticulous investigations should be performed to look for TCS, especially in patients with craniosynostosis.

Challenges faced by parents when seeking diagnosis for children with sensorineural hearing loss.

INTRODUCTION: The Joint Committee of Infant Hearing (JCIH) recommended hearing screening by one month of age, diagnosis of hearing loss by three months of age, and intervention initiated by six months of age. In Malaysia however, the age of diagnosis of hearing loss in children is relatively late. This study aimed to identify the challenges faced by parents in seeking a diagnosis of hearing loss for their children. METHOD: The study utilized a semi-structured interview with open-ended questions to obtain information about parents' experiences during the diagnosis period and their challenges when going through that process. In this study, a total of 16 parents of children who were diagnosed with moderate to profound sensorineural hearing loss and received intervention within three years at the time of the study participated. Ten of the children were cochlear implant users, and six were hearing aid users. RESULTS: Thematic analysis was used to analyse themes generated from the data according to the study objective. Four main themes and 17 subthemes were identified from this study. The four main themes were 1) Parents' emotion; 2) Parental knowledge; 3) Others; 4) Profesional services. Challenges that parents faced often include emotional behaviours such as feeling guilty and devastated during the diagnosis, lack of information-sharing from healthcare givers, lack of knowledge on childhood hearing loss among parents, support from families, seek for a second opinion, worry about others' acceptance, longer time for diagnosis to confirm, late referral to other related profesionals and no priority for the appointment. CONCLUSION: Emotion is identified as the biggest challenge faced by parents in the process of diagnosis for their children with hearing loss. Hence, management of parental emotion needs to be emphasized by health profesionals as it influences the acceptance of parents towards their child's diagnosis.

Retropharyngeal Abscess in a Neonate After Extravasation Injury: To Drain or not to Drain?

Neonatal retropharyngeal abscess (RPA) is a rare and life-threatening entity. Most of the cases are idiopathic in nature. We encountered a case of RPA in a newborn secondary to extravasation injury. The presence of neck swelling with clinical deterioration following extravasation of total parenteral nutrition (TPN) infused via a peripherally inserted central catheter at the right upper limb raised the suspicion of neck abscess. This was later confirmed to be RPA based on magnetic resonance imaging of the neck. She was treated with prolonged intravenous antibiotics in the Neonatal Intensive Care Unit (NICU). Her condition gradually improved, evidenced by resolution of the collection on serial imaging. Early recognition and prompt management are crucial to reduce the morbidity and mortality from RPA.

Vincristine-induced vocal cord palsy: case report and review of the literature.

Vincristine-induced vocal cord paralysis is a rare but serious complication. We report 2 patients with acute lymphoblastic leukemia who developed progressive stridor during induction chemotherapy. There were no clinical features of peripheral or autonomic neuropathy. Flexible laryngoscopy confirmed the diagnosis of bilateral vocal cord palsy; interestingly, the nerve conduction test revealed axonal motor neuropathy involving the median and common peroneal nerves in both patients. The first patient required prolonged ventilatory support necessitating unilateral cordectomy before extubation, whereas the second only required supplemental oxygen therapy. There was resolution of stridor in the first patient after cordectomy and gradual clinical improvement in the second. These cases illustrate that a high index of suspicion of vincristine-induced vocal cord palsy with prompt otolaryngology consultation for laryngoscopy is required in the diagnostic evaluation of a patient who has received vincristine.

Intermittent Suprasternal Neck Mass Caused by Herniation of Ectopic Thymus: Report of Two Cases.

INTRODUCTION: Ectopic thymus is an uncommon cause of neck masses in children that frequently present as lateral cervical swelling especially on the right side. CASE REPORT: We report two cases with atypical clinical presentation of ectopic thymus and superior herniation of normal thymus. Both of the patients manifested as intermittent midline mass at the suprasternal region during Valsalva manuevre. Unique ultrasound features with the location along the thymic descent together with dynamic assessment of the organ movement were essential to reach the correct diagnosis. Conservative approach was considered in these patients considering the necessity of thymus in the process of puberty. CONCLUSION: High index of suspicion is of utmost importance when encounter patient with similar clinical manifestation to avoid unnecessary diagnostic modalities and surgeries. Accurate diagnosis will also alleviate parents' anxiety.