Non-Tuberculous Mycobacterial Cervicofacial Lymphadenitis in Children-10-Year Experience in a Tertiary Pediatric Center.

BACKGROUND: Lymphadenitis is the most common manifestation of non-tuberculous mycobacteria (NTM) infection in children. We describe the epidemiology and clinical characteristics of NTM lymphadenitis, determine diagnostic yield from tissue sampling, and review management and outcomes. METHODS: This was a 10-year retrospective review of children aged 0-16 years diagnosed with NTM cervicofacial lymphadenitis who were seen in a pediatric infectious disease clinic in a tertiary public hospital. Data relating to patient demographics, clinical features, surgical and antimicrobial treatment, complications, and outcomes were retrieved from patients' electronic medical records and analyzed. RESULTS: There were 48 episodes of NTM cervicofacial lymphadenitis in 45 children (17 males and 28 females). Of these episodes, 43.7% manifested as a unilateral single node, mostly parotid (39.6%) and submandibular (29.2%). All patients underwent diagnostic fine-needle aspiration or surgery. Surgical excision more frequently yielded positive histological findings (P = .016). NTM was identified in 22/48 episodes (45.8%) via culture or molecular sequencing. Mycobacterium abscessus was most commonly found (47.8%). Thirty-eight children (79.2%) received antibiotics. Outcomes in 43 episodes revealed full resolution in 69.8%, while 25.6% had de novo disease and 4.6% experienced recurrence at the same site. Overlying skin changes and multiple or bilateral nodal diseases were significantly associated with de novo disease or recurrence (P = .034 and .084, respectively). Complications occurred in 11/70 (15.7%) procedures. Antibiotic-associated adverse effects occurred in 14/38 (36.8%) episodes. CONCLUSIONS: NTM lymphadenitis remains a challenging condition. More aggressive management with surgical excision and antibiotics is recommended for those with overlying skin changes and extensive nodal disease.

Predictive Factors for Perinatal Outcomes of Infants Diagnosed With Micrognathia Antenatally.

INTRODUCTION: Advances in fetal imaging have allowed us to identify abnormalities previously not appreciated. With this study, we hope to identify factors predicting a difficult airway at birth and review the perinatal outcomes of these patients. METHODS: Sixteen patients with antenatally diagnosed micrognathia were reviewed from a tertiary care hospital database from 2011 to 2016. Jaw index (JI), amniotic fluid index (AFI), glossoptosis, gastric size, and oropharynx obliteration were assessed. The airway support required at birth, specialist team involvement, and outcomes were evaluated. RESULTS: Nine (56.3%) of 16 patients had JI <5th percentile, 3 (33.3%) of 9 had difficult intubation, 2 (22.2%) of 9 needed an emergency tracheostomy, and 1 (11.1%) of 9 died. Seven patients had polyhydramnios, 2 (28.6%) of 7 had difficult intubation, 2 (28.6%) of 7 required tracheostomy, and 1 (14.3%) of 7 died. Twelve patients had either JI <5th percentile or abnormal AFI, 5 (41.7%) of 12 had difficult intubation, 2 (16.7%) of 12 required tracheostomy, and 1 (8.33%) of 12 died. For the group without otolaryngology consultation, 8 (50%) of 16, 1 (12.5%) of 8 had difficult intubation and 1 (12.5%) of 8 died because airway was not secured after 45 minutes of resuscitation. CONCLUSION: Jaw index <5th percentile or abnormal AFI predicts a difficult airway. A multidisciplinary approach with otolaryngology involvement for airway intervention may be required at birth.

Pediatric Oncology Patients With Vincristine-Induced Recurrent Laryngeal Nerve Palsy: Two Case Reports and a Brief Review of Literature.

INTRODUCTION: Vincristine (VCR) is a chemotherapeutic agent used widely in the treatment of hematologic and solid tumors, known to result in neurotoxicity, especially with cumulative administrations. Bilateral vocal fold palsy (VFP) is a rare but life-threatening complication of VCR. We report 2 patients with hepatoblastoma presenting with stridor following VCR treatment and propose a management plan. METHODS: Electronic medical records of oncology patients treated at a tertiary hospital with VCR-induced VFP were reviewed. Literature review was performed in PubMed using the terms: hoarseness, VFP, stridor, vincristine. RESULTS: A total of 23 children with VCR-induced VFP were identified from the literature review and adding on our 2 cases. Seventeen (77.3%) were male and 5 (22.7%) were female. The median presenting age was 36.0 months (5-204 months). Acute lymphoblastic leukemia, 15 of 23 (65.2%), was the most common malignancy. Eighteen patients (78.3%) had bilateral VFP and 5 (21.7%) had unilateral VFP. The mean time to VF function recovery was 167.3 days (median: 200.5 days, range: 7-270 days) in the intervention group versus 72.1 days (median: 31.5 days, range: 3-240 days) in the conservative group. One patient in the intervention group had persistent VFP. Sixteen patients (69.6%) were observed, 4 (17.4%) underwent tracheostomy, 1 (4.35%) was intubated, 1 (4.35%) underwent cordectomy, and 1 (4.35%) required positive pressure support. Vincristine was restarted in 12 patients (54.5%), of which 4 developed recurrence of airway symptoms and had to stop VCR. CONCLUSION: A new-onset hoarseness or stridor in a child on VCR should raise the suspicions of VFP. The assumption of an upper respiratory-induced hoarseness or stridor should be avoided. Decisions regarding readministration of VCR and possible airway interventions should be made via a multidisciplinary team approach.

Cutaneous "fibroma-like" perivascular epithelioid cell tumor: A case report and review of literature.

Perivascular epithelioid cell tumors (PEComas) are a group of lesions sharing the common features of co-expression of melanocytic and myogenic markers, with focal association of the cells with vascular walls. The PEComa group exhibits a wide range of morphologies. A "fibroma-like" variant of PEComa has been recently described. The case reported herein is that of an infant with tuberous sclerosis complex (TSC) presenting with a lip mass. Excisional biopsy showed a moderately cellular tumor composed of spindled to stellate cells embedded within a collagenized stroma. The cells showed focal perivascular accumulation and positivity for both melanocytic (HMB-45) and myogenic (desmin) markers. This is the fifth reported case of "fibroma-like" PEComa in literature and the youngest patient to date. All of the "fibroma-like" PEComas were found in patients with tuberous sclerosis-hence, the diagnosis of this entity should prompt a workup for TSC; conversely, a fibroma-like lesion in a patient with TSC or with TSC-related conditions should be evaluated using melanocytic and myogenic markers. Melanocytic and myogenic markers are also useful in differentiating "fibroma-like" PEComa from other differential diagnoses such as fibroma and benign fibrous histiocytoma.

Cervical Myxoid Solitary Fibrous Tumor: Report of an Unusual Variant and a Brief Overview of the Literature.

We present a 49 year old female with a diagnostically challenging myxoid solitary fibrous tumor arising in the soft tissue of the neck. The tumor was diffusely positive for CD34 and STAT6 on immunohistochemistry.

A Ten-Year Review of Audiological Performance in Children with Inner Ear Abnormalities after Cochlear Implantation in Singapore.

OBJECTIVES: To evaluate children with inner ear malformations following cochlear implantation (CI) in a tertiary pediatric hospital in Singapore to identify factors influencing outcomes after CI. METHODS: This is a retrospective cohort study of children aged 0 to 18 years, who had CI between 2000 and 2013. Demographic information, data on risk factors, type of inner ear malformation (IEM), age at implantation, speech pre- and postimplantation, and duration of follow-up were collected from clinical records. Operative details and audiological outcomes were also analyzed. RESULTS: A total of 70 children underwent 83 CI surgeries. The mean age of the patients was 4.05 ± 3.17 years (range 1-18 years). Twenty patients (28.57%) had abnormal CT scan findings. CSF gusher occurred in 15 out of 26 CI (57.69%) in the group with IEM. Nine out of twenty patients (45.00%) had poor IT-MAIS scores prior to implantation. The average preoperative IT-MAIS score for children with anomalous inner ear anatomy was 14.1. The older CI patients, 3/20 (15.00%), mean age 8.33 years (range 7-10 years), were mostly referred for persistently unclear speech following hearing aids. Eleven patients (55.00%) had good speech and aided hearing threshold within speech limits after CI and were eligible for reintegration into mainstream schools. Five patients (25.00%) had improvement in speech but continued to receive education in special schools. Four patients (20.00%) had poor progress after surgery. CONCLUSION: The presence of absent cochlear nerve, electrode folding, and underlying neurological disorders seemed to be associated with poorer outcomes.

A Case Report of Absent Epiglottis in Children With Nager Syndrome: Its Impact on Swallowing.

OBJECTIVE: This clinical report is the first literature report exploring the issues of an absent epiglottis in children diagnosed with Nager syndrome. Absent epiglottis has previously been described in children with Pierre Robin sequence and other syndromes. CONCLUSION: The function of the epiglottis and its contribution to swallowing has always been debated. In this article and from our literature review, we felt that absent epiglottis possibly contributed to the aspiration risk and may partly explain the long-term feeding issues in these children despite corrective surgeries.

Laryngeal inflammatory myofibroblastic tumor (IMT): a case report and review of the literature.

BACKGROUND: This case report is interesting as cases of children with laryngeal inflammatory myofibroblastic tumor are not common and previously had been presented as isolated case reports. This is the first case report in Asia describing a laryngeal inflammatory myofibroblastic tumor and its removal using an endoscopic approach. CASE PRESENTATION: Our patient is a 12-year-old Malay girl from Singapore who presented with hoarseness without respiratory distress. The initial impression was that of a granuloma or a papilloma. We did a biopsy, which confirmed the histology to be inflammatory myofibroblastic tumor, and a magnetic resonance imaging scan showed a contrast-enhanced lesion. The lesion was excised completely using an endoscopic approach. The child was discharged well on the first postoperative day and she has been on follow-up for a year in the clinic. CONCLUSIONS: This report highlights the importance of understanding the differential diagnosis for a child with hoarseness. It is not uncommon for a pediatrician, a general practitioner, and a pediatric otolaryngologist to see a child presenting with hoarseness. In most cases, the diagnosis made would be screamer's nodules, which is commonly seen in children. In a small group, recurrent respiratory papillomatosis form the diagnosis. Over the past few years, the cases of recurrent respiratory papillomatosis have decreased significantly. Laryngeal tumors are not common in children. However, we must maintain a high index of suspicion when we have a child with hoarseness who does not improve with speech therapy and watchful waiting. In such situations, a stroboscope is usually necessary to diagnose the voice problems and to rule out pathological conditions such as laryngeal tumors. If left untreated, the lesion can grow with time and result in a life-threatening airway condition. We also demonstrate our endoscopic technique in this report, and it has proven to be safe with no increased recurrence and much lower morbidity.

Comparison of the distribution of intranasal steroid spray using different application techniques.

BACKGROUND: Optimizing the intranasal distribution of nasal steroid spray (NS) is important in managing patients with allergic rhinitis (AR). Using a 3-dimensional computational model of the human nose, we found that inspiratory airflow improved particle distribution by 86%. We hence designed a study to determine if the intranasal distribution of NS is improved by (1) simultaneous gentle inspiration or (2) nasal decongestion. METHODS: Twenty patients with AR were recruited. Colored triamcinolone nasal spray Nasacort®, was applied to 1 side of the nasal cavity with simultaneous gentle inspiration (technique1) and the other side with no inhalation (technique 2). Flexible nasoendoscopy with video documentation was performed immediately after each application. The same procedures were repeated 30 minutes after nasal decongestion. The nasal cavity was divided into 10 different regions and presence of the colored medication in each region was given 1 point. RESULTS: Prior to decongestion, the mean total score was significantly higher using technique 1 (4.61 ± 0.25) compared to technique 2 (3.80 ± 0.24), (p = 0.03). Following decongestion, the mean total score for techniques 1 and 2 was (5.20 ± 0.40) and (4.30 ± 0.32), respectively. The differences in total score for both techniques before and after decongestant were not significant. CONCLUSION: The presence of inspiratory airflow, using the gentle inspiration technique, improves the intranasal distribution of NS in patients with AR. The use of correct technique is more important than nasal decongestion in the distribution of nasal spray particles.

Malignant transformation of keratocystic odontogenic tumor: two case reports.

INTRODUCTION: Keratocystic odontogenic tumors (KCOTs) are cystic tumours originating from the dental lamina of the maxilla and mandible that are lined with keratinized epithelium. While benign, they can be locally destructive and have a high recurrence rate despite treatment. Rarely, KCOTs may undergo malignant transformation into Primary Intraosseous Squamous Cell Carcinoma (PIOSCC). CASE REPORT: This study reports the clinical findings, radiological scans and histopathology of 2 patients with KCOTs that underwent malignant transformation into PIOSCC. DISCUSSION: A comprehensive literature review was performed to similar reports documenting the malignant transformation of KCOTs. The potential for KCOTs to undergo malignant change should prompt oral maxillofacial surgeons and otolaryngologists to exercise a high index of suspicion when treating these lesions. Patients persisting with unresolved disease after treatment should be investigated for malignant transformation. Detailed histopathological examination of KCOT specimens is recommended to detect small foci of SCC which may be present in the epithelium.

Epinephrine auto-injector prescriptions as a reflection of the pattern of anaphylaxis in an Asian population.

This study surveyed the prescription patterns of adrenaline auto-injectors (AAs) in Singapore to examine the frequency, triggers, and demographic pattern of anaphylaxis requiring such prescriptions. A 6-year retrospective review of 417 consecutive patients prescribed AAs in Singapore from January 1999 to December 2004, as identified from hospital pharmacy records. There were 417 patients identified, consisting of 295 (70.7%) Singaporeans with the remaining being non-Singaporean residents. Based on population census, the frequency of AA prescriptions was estimated at 1 per 10,000 Singaporeans. Demographic factors associated with AA prescriptions were male gender (OR = 1.361; p = 0.002); minority ethnic groups, which included Eurasians, Caucasians, Koreans, and Japanese (OR = 15.873; p < 0.001); and children <15 years of age (OR = 2.593; p < 0.001). The most common food allergens resulting in AA prescriptions were peanut (41.9%) and shellfish allergy (28.5%). Multiple logistic regression analysis showed that peanut allergy was independently associated with Eurasian ethnicity (OR = 5.045; p = 0.021); and shellfish allergy with Indian ethnicity (OR = 2.757; p = 0.034). The estimated frequency of AA prescriptions in Singapore is relatively low at 0.01%. The incidence of peanut and shellfish allergy in the Asian population appears to differ from that seen in Western populations.