Radial forearm free flap reconstruction in a 3-month-old patient with undifferentiated pharyngeal sarcoma.

There is minimal information regarding free tissue transfers in very young infants, especially those less than a year old. It is often thought that that age remains a limit to free tissue transfers, with younger patients having smaller vessels, making the operation technically challenging. In this case report, we discuss the youngest and smallest recorded case of a free flap reconstruction. A 3-month-old patient with a malignant parapharyngeal undifferentiated round cell sarcoma underwent a resection and reconstruction with a radial forearm free flap (RFFF). The defect was 35 by 20 by 15 mm, and required a pharyngeal "patch," as opposed to a "tube," reconstruction. The defect was templated, and the RFFF then raised in a standard subfascial fashion, and inset with resorbable sutures. The patient was observed in the ICU postoperatively. The patient was subsequently diagnosed with Stage IV primary undifferentiated sarcoma with regional metastasis and received adjuvant chemotherapy. Fifteen-month follow up revealed no signs of recurrence, full oral intake, a well-reconstructed pharynx on nasoendoscopic examination, and minimal donor site morbidity. This report illustrates several unique adaptations of free flap transfer in infants and adds to the emerging body of evidence that age is not a contraindication for head and neck reconstruction.

Otolaryngological burden of disease in children with primary ciliary dyskinesia in Victoria, Australia.

OBJECTIVES: The aim of this study was to summarize the otolaryngological manifestations amongst children with primary ciliary dyskinesia (cwPCD) to improve diagnosis, investigations and management amongst otolaryngologists. METHODS: A retrospective review of primary ciliary dyskinesia (PCD) diagnoses at our institution over an 8-year period between January 2014 and October 2022 was conducted. Patient characteristics, diagnosis, otolaryngological symptomatology, treatment and outcomes were recorded. RESULTS: 24 patients were identified. Thirteen patients (54%) had documented conductive hearing loss on audiological evaluation; with 11 (85%) requiring hearing aids. Six patients (25%) underwent middle ear ventilation tube (MEVT) insertion with 67% experiencing post-MEVT otorrhoea. Twenty children (83%) reported chronic nasal discharge however only 3 (13%) reported nasal obstruction. Nine patients (38%) had symptoms consistent with sleep disordered breathing with 79% of them requiring operative management with adenotonsillectomy. CONCLUSION: Middle ear effusion is common amongst cwPCD and should be managed with conservative measures due to the significant burden of post-MEVT otorrhoea. Sinonasal symptoms rarely need surgical intervention. Many otolaryngological symptoms of PCD are often underreported, particularly sleep-disordered breathing. Paediatric PCD patients should be managed in a multidisciplinary team with routine and tailored therapies to manage all aspects of the condition.

Laryngeal myxedema as a cause of reversible subglottic stenosis in a newborn.

Myxedema is a manifestation of severe hypothyroidism, and when affecting the larynx it constitutes a rare cause of upper airway obstruction. We present a case of laryngeal myxedema in a newborn causing subglottic stenosis. The atypical aspect of the stenosis along with a thyroid goiter and a positive screening for neonatal hypothyroidism allowed the diagnosis of laryngeal myxedema. Thyroid hormone replacement and non-invasive ventilation led to quick resolution while avoiding invasive approaches. Laryngeal myxedema is possibly underdiagnosed. Physician awareness is essential to avoid pitfalls in such children with reversible airway obstruction.

Airway management in patients with lingual thyroid: a case report and review of the literature.

PURPOSE: To review the management of patients with lingual thyroid (LT) causing upper airway obstruction and to suggest a diagnostic and therapeutic workflow. METHODS: A PubMed review of published cases from January 1980 up to December 2020 of LT causing upper airway obstruction. We selected cases of confirmed LTs that presented with non-state-dependent airway obstruction. An illustrative case report is presented. RESULTS: Twenty-one articles fulfilling the inclusion criteria were found, reporting 24 cases (7 neonatal, 2 pediatric and 15 adults). The main presenting symptoms was dyspnea with increased work of breathing, followed by dysphagia and stridor most commonly in neonates. At least one imaging modality was performed in all patients. Thyroid function was altered in half the patients and normal in the other half. The LT was the only thyroid tissue in all cases except 2. Altogether, 5/24 patients required tracheostomies and two-thirds of the patients underwent surgical resection of the LT (mostly transoral). Also 2/3 of the patients received thyroid replacement therapy. After a median follow-up of 17 months, airway symptoms had fully resolved for all patients but one. CONCLUSION: While rare, ectopic LTs should be considered in the differential diagnosis of stridor, dyspnea and airway obstruction. In neonates, concomitant presence of hypothyroidism on neonatal screening and airway obstruction should prompt the search for a LT. Early identification and thyroid replacement therapy seem to significantly relieve symptoms of upper airway obstruction, but severe obstruction and concomitant airway lesions may require more definitive management approaches.

Postoperative admission to paediatric intensive care after tonsillectomy.

OBJECTIVES: To review interventions required by children admitted for intensive care management following tonsillectomy or adenotonsillectomy either as elective or unplanned admission in a tertiary children's hospital. METHODS: A retrospective chart review over a 10-year period between April 2007 and March 2017 was performed. Charts were interrogated for treatments that were administered in the paediatric intensive care unit. Respiratory support therapies such as supplemental oxygen administration, high-flow nasal oxygen, positive pressure ventilation, continuous positive airway pressure, airway interventions and tracheal intubation were reviewed. RESULTS: There were 103 children admitted to the paediatric intensive care unit following tonsillectomy or adenotonsillectomy. The average age was 6.2 years (range 7 months-17 years). The main indications for the procedure were sleep disordered breathing or obstructive sleep apnoea syndrome. In all, 53 children had syndromes with medical comorbidities, 31 were current continuous positive airway pressure users and 5 had a tracheostomy in situ. Forty children admitted to paediatric intensive care unit did not require any high-level care. Ten children who had an unplanned admission had their respiratory interventions started in the theatre or in the post-anaesthetic care unit, before paediatric intensive care unit admission, and did not require escalation of care. CONCLUSION: Children may not require admission for intensive care after tonsillectomy if they have had an incident-free period in the post-anaesthetic care unit. Some of those who required high-flow nasal oxygen could have been managed on the ward provided with adequate training and monitoring facilities. The level of care they require in post-anaesthetic care unit reflected the level of care for the immediate postoperative period in the paediatric intensive care unit.

Absence of Ulnar Artery Inflow Detected by Allen's Test Prior to Radial Forearm Free Flap.

Radial forearm free flaps are commonly used for soft-tissue reconstruction after resection of head and neck cancer. It is perfused by the radial artery, leaving the ulnar artery for perfusion of the hand and digits. The absence of distal ulnar artery and associated superficial palmar arch, however, has not been reported in cadaveric dissection. We report a case of unilateral ulnar artery flow absence, detected by Allen's test, during preoperative preparation for a radial forearm free flap. Based on the simplicity, safety, and ease, we recommend Allen's test to be performed preoperatively on every patient for whom such a flap is a consideration.

Using Data Exclusivity Grants to Incentivize Cumulative Innovation of Biologics' Manufacturing Processes.

The pharmaceutical market is divided into two types of compounds: small-molecule chemical compounds and large-molecule biologics. Due to biologics' molecular sizes and the current scientific state of biologics manufacturing, manufacturing facilities and processes require frequent reassessment to ensure production of safe, pure, and potent therapeutics. Manufacturers utilize patent and drug regulatory law to protect their investments and simultaneously signal where innovation and investment are lacking. The current four- and twelve-year regimented structures of the Biologics Price, Competition, and Innovation Act do not keep pace with scientific development; biologics manufacturing processes drift with time, and if a manufacturer can obtain a higher degree of process control, then it should not feel restricted to wait until their exclusivity period lapses. Currently, the FDA rarely grants market exclusivity privileges for manufacturing process improvements alone; hence, manufacturing processes--or at least large portions thereof--are typically withheld as trade secrets or strategically claimed within companion composition claims. As a result, significant opportunity exists in regulatory framework to incentivize the research and development of biologics manufacturing processes. By creating a one- to four-year data exclusivity extension opportunity, manufacturers will feel more comfortable reinvesting their returns on investment towards manufacturing efficiency, and manufacturers can capitalize on the complex-molecule nature of their biologic.

The Incidental Thyroid Lesion in Parathyroid Disease Management.

OBJECTIVE: The incidental thyroid lesion is a common finding during general imaging studies. Their management has been the subject of numerous studies and recommendations. Parathyroid disease workup necessitates imaging investigation of the adjacent thyroid gland and therefore provides a unique window to the perioperative management of thyroid incidentaloma. The specific prevalence of incidental thyroid lesions in the context of parathyroid disease is unknown. We seek to investigate its prevalence during parathyroid workup and surgery and to ascertain if there was a change in management of these patients. STUDY DESIGN: Five-year retrospective database review. SETTING: Tertiary care medical center. SUBJECTS AND METHODS: The source and indication for referral, preoperative investigation findings, and management of the incidental thyroid lesions were examined. The actual procedure performed and final pathology results were assessed. RESULTS: A total of 98 patients and 106 operations, including revision surgeries, were identified. There were 21 incidental thyroid lesions (21.4%) detected, whereby 15 patients underwent fine-needle aspirations and 12 subsequently had diagnostic hemithyroidectomies. This decision was made preoperatively in 5 patients and intraoperatively in 7 patients at the time of parathyroid surgery. Along with other pathologies, there were 7 patients with micropapillary thyroid carcinoma identified. CONCLUSIONS: In our series, the prevalence of incidental thyroid lesion and thyroid malignancy is comparable to the general population. The management of the initial parathyroid disease in our patients was altered by the imaging and cytological findings of these thyroid lesions. This has implications on perioperative counseling of the thyroid and parathyroid disease.

Magnetic resonance imaging findings in children with tinnitus.

OBJECTIVE: Tinnitus in adults is generally investigated by contrast-enhanced magnetic resonance imaging (MRI) to rule out the diagnosis of acoustic neuroma. Acoustic neuroma is rare in children and, therefore, the role of MRI in children with tinnitus is unclear. This study was undertaken to determine the value of MRI in the investigation of tinnitus in children. METHODS: Retrospective study of children younger than 18 years who underwent MRI for the investigation of tinnitus over a 10-year period. RESULTS: Sixty-five patients were identified, but there were only 34 who had also undergone audiologic assessment. Among the 25 patients with normal audiology, MRI abnormalities were present in 9, but these were all thought to be nonspecific. Nine patients had abnormal audiograms and the MRI was abnormal in 4 of these cases, which included 3 children who were found to have multiple sclerosis. CONCLUSION: Magnetic resonance imaging would appear to be mandatory in the investigation of tinnitus in children who are found to have sensorineural hearing loss, particularly to rule out the diagnosis of multiple sclerosis. Although our study does not support the routine use of MRI in children with normal audiology, the numbers in our series are too small for a conclusive recommendation.