Facial Onset Sensory and Motor Neuronopathy-Like Syndrome: A Case Report.

OBJECTIVES: Facial onset sensory and motor neuronopathy syndrome (FOSMN) is a rare motor neuron disorder characterized by facial sensory and motor aberrations that progress to the upper limbs. We present a case of FOSMN-like syndrome that has characteristics of FOSMN but is confined to the craniofacial region. METHODS: Retrospective chart review and review of the literature. RESULTS: A 70-year-old woman presented with a 1-month history of progressive bilateral facial sensory loss and weakness affecting the trigeminal and hypoglossal nerves. Within 12 months, she developed debilitating weakness affecting her lower and midface bilaterally. After an extensive workup, a diagnosis of FOSMN-like syndrome was made, as symptoms failed to progress to the upper extremities. CONCLUSIONS: This case demonstrates a unique presentation of FOSMN that we classify as FOSMN-like syndrome. Clinicians must maintain a high index of suspicion when a patient presents with clinical features characteristic of FOSMN syndrome without progression of symptoms distal to the craniofacial region because it may represent a FOSMN-like syndrome.

Initial outcomes at a nascent tertiary pediatric thyroid surgical center.

OBJECTIVES: Previous studies on pediatric thyroid surgical complications suggest that high-volume centers achieve improved outcomes. We hypothesize that initial outcomes from a nascent pediatric surgical practice may be comparable to higher volume centers. Furthermore, we determine whether a low-volume center can safely transition to an intermediate or high-volume center. METHODS: A retrospective chart review was performed for all pediatric patients undergoing thyroid surgery at a single institution from 2014 to 2020. Surgeries were performed by two pediatric otolaryngologists. All patients were managed postoperatively by a multidisciplinary team of physicians that included pediatric otolaryngologists and endocrinologists. Data collection focused on patient demographics and postoperative complications, including rates of recurrent laryngeal nerve injury and permanent hypoparathyroidism. RESULTS: From 2014 to 2020, a total of 31 patients underwent thyroid surgery at our pediatric thyroid surgery center, 9 of whom underwent neck dissection. The mean age of our cohort was 14.4 ± 3.9 years (range 8 months-20 years). Postoperative pathology results revealed that 15 patients (46.9%) were diagnosed with PTC, 6 (18.8%) with follicular adenoma, and 4 (15.6%) with benign thyroid tissue. One (2.0%) patient had permanent unilateral recurrent laryngeal nerve paralysis and one patient experienced permanent hypoparathyroidism (2.7%). CONCLUSIONS: Our initial low complication rate as a nascent pediatric thyroid surgery center suggests that favorable outcomes can be achieved at lower volume surgery centers. In order to increase patient access to high-volume pediatric thyroid surgery centers, new centers must start with lower volumes before ultimately becoming high-volume centers. Our study shows that this can be safely achieved. LEVEL OF EVIDENCE: IV.