Endoscopic Optic Nerve Decompression in a Hamamy Syndrome Patient With Bilateral Optic Canal Stenosis.

Hamamy Syndrome is an autosomal recessive syndrome with craniofacial, neurological, and osteological implications. Patients most commonly present with repeated long fractures however, other affected systems with their respective clinical presentations warrant a thorough reporting and understanding of this genetic disorder. Herein, the authors, present a 21-year-old male patient diagnosed with Hamamy Syndrome with bilateral stenosis of the optic canals and associated bilateral vision loss. This case report documents the patient's initial presentation 6 years ago, which included a history of right vision loss for 2 months, followed by a 6-year follow-up period during which the patient underwent 3 optic nerve decompression surgeries. There is currently a limited number of reports in the English literature on Hamamy Syndrome, with the primary focus being on genetic, dental, orthopedic, and neuropsychiatric aspects, but the neural foraminal narrowing with associated neuropathy has never been reported.

Age at diagnosis, neutrophil-to-lymphocyte ratio and platelet-to-lymphocyte ratio as prognoticators in pediatric sinonasal rhabdomyosarcoma.

BACKGROUND: The relationship between pretreatment blood parameters and clinical outcomes of patients with pediatric sinonasal rhabdomyosarcomas has not been described. The purpose of this study was to determine the prognostic factors and certain laboratory parameters that affect the survival and long-term survival in pediatric sinonasal rhabdomyosarcoma. METHODS: Medical records of pediatric sinonasal rhabdomyosarcoma cases who were treated and followed up between 2004 and 2020 in Hacettepe University were retrospectively reviewed. The relationship between clinical features, laboratory parameters and survival was investigated. RESULTS: Age at the time of diagnosis, pretreatment neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR) had significant effect on survival (p < 0,004, p < 0,037, p < 0,016, respectively). Survival rate was higher in patients younger than 10 (≤10 years of age) at the time of diagnosis (p = 0,004), patients with a NLR of 2 or below (≤2) (p = 0,037), and patients with a PLR of 150 or below (≤150) (p = 0,016). ≤ 10 years of age at the time of diagnosis was found as an independent prognostic factor affecting survival (hazard ratio [HR], 5382; 95 % confidence interval [CI], 1476- 19,623; P = 0,011). In addition, a pretreatment PLR of 150 or below (≤150) was found as another independent prognostic factor that affects survival (hazard ratio [HR], 4386; 95 % confidence interval [CI], 1161- 16,567; P = 0,029). CONCLUSIONS: Preoperative NLR and PLR may be important parameters to predict the prognosis of pediatric sinonasal rhabdomyosarcoma. Further research with larger patient groups are warranted.

The Use of Piezoelectric Osteotomy Instruments During a Rhinoplasty Operation in a Patient With a Cochlear Implant.

A cochlear implant is a life-changing operation that significantly improves the lives of patients. Those with cochlear implants require ongoing measures to ensure the longevity of both their devices and their well-being. One concerning issue is the potential device failure due to the use of surgical instruments during procedures. In this report, we present a successful case of utilizing an ultrasonic osteotome for a primary septorhinoplasty in a 22-year-old patient who had undergone cochlear implant surgery 17 years prior. To our knowledge, this is the first recorded instance of applying a piezoelectric tool on a patient with a cochlear implant. Our findings support the safety of using a piezo osteotome in cochlear implant recipients.

Endoscopic Excision of the Intraosseous Hemangioma of the Nasal Bone.

An intraosseous hemangioma is a rare benign tumor of the bone. Moreover, it is rarely observed in the nasal bone. A 58-year-old female patient complained of swelling in the nasal dorsum and mild pain. CT showed a round mass with a honeycomb appearance meanwhile, MRI revealed a hypointense mass in T1 sequences and hyperintense in T2 and gadolinium sequences. Endoscopic removal of the intraosseous hemangioma was performed. No recurrence was observed in the post-op 6th-month follow-up. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-03729-x.

Topical Intranasal Insulin Enhances Healing of Nasal Mucosa: An Experimental Animal Study.

OBJECTIVE: Aim of this study was to evaluate the effect of topical intranasal insulin on healing of nasal mucosa in a rat model. METHODS: Forty-eight Wistar rats, weighing between 250 and 300 g and aged 10-12 weeks were used and randomized into two equal groups. 1.9 mm curette was introduced through the left nostril and 1.9 mm mucosa from the left nasal septum was curetted. Postoperatively, animals in the control group received 1 mL of physiologic saline, 3 times a day in a nasal irrigation fashion. Animals in the experimental group received 1 mL of 5 IU/mL regular insulin in saline solution. Subjects were sacrificed after 5, 10, and 15 days and macroscopic and histomorphometric evaluations were performed. RESULTS: There were no mucosal synechiae and septal perforation macroscopically. Histological examination revealed that the defect size reduction was 21% in the saline group versus 56% in the insulin group on the fifth day (p = 0.006). There was 62% defect reduction in the saline group versus 79% in the insulin group on the 10th day (p = 0.034). On the 15th day, only 67% of saline group animals had complete defect closure, whereas 100% of animals treated with insulin had complete closure (92% vs 100% mucosal defect reduction, p = 0.036). Both edema and inflammation were less in the insulin group on 15th day (p = 0.006; p = 0.023, respectively). CONCLUSION: The results from this study support the safety and efficacy of topical insulin on wound healing in the literature. This study could guide further experimental studies that examine human sinonasal wound healing.

Supine or non-supine apnea; which can be treated better with expansion sphincter pharyngoplasty?

OBJECTIVES: Expansion sphincter pharyngoplasty is a well-known palatal correction surgery applied for obstructive sleep apnea patients. Even though high success rates are reported, in some patients, the desired outcome cannot be achieved. Therefore, patient selection is crucial to avoid undesirable outcomes. In this study we present our results with expansion sphincter pharyngoplasty, and we aim to show the difference between supine and non-supine apnea in terms of their response to the surgery, in order to utilize position dependency for selection of surgical candidates. METHODS: Pre- and post-operative polysomnography results of patients who underwent expansion sphincter pharyngoplasty were analyzed retrospectively. Total AHI, supine AHI, non-supine AHI, supine/non-supine AHI ratio and surgical success values are compared. RESULTS: 85 patients were included to the study. Mean AHI significantly decreased from 48.7 ± 27.99 to 26.37 ± 21.16 with the surgery. Surgical success rate was found to be 51.8%. Both supine and non-supine apnea decreased significantly with the surgery, but the decrease was significantly higher in non-supine apnea (20.6% to 39.1% respectively, p = 0.016). There was significant negative correlation between pre-operative supine to non-supine AHI ratio and the change in AHI, showing that supine dominant patients had less improvement with the surgery (r = 0.274, p = 0.01). CONCLUSION: Expansion sphincter pharyngoplasty is an effective surgery which achieves significant improvement in AHI. Non-supine respiratory events respond better to the surgery than supine events.

Rare occupational cause of nasal septum perforation: Nickel exposure.

Many etiologies are held accountable for nasal septum perforations. Topical nasal drug usage, previous surgeries, trauma, nose picking, squamous cell carcinoma, some rheumatological disorders such as granulomatosis with polyangiitis (Wegener granulomatosis), some infectious diseases such as syphilis and leprosy are among the causes of the perforations. Occupational heavy metal exposures by inhalation rarely may also cause nasal septum perforation. Here, we present a 29-year-old patient without any known diseases, who is a worker at a metallic coating and nickel-plating factory, referred for investigation of his nasal cartilage septum perforation from an otorhinolaryngology clinic. The patient questioning, physical examination and laboratory assessment about rheumatic and infectious diseases were negative. There was a metallic smell in the breath during the physical examination. The analysis showed serum nickel level at 31 µg/l and urine nickel at 18 µg/l (84.11 µg/g creatinine). Other possible serum and urine heavy metal levels were within normal ranges. Nickel exposure is usually together with other heavy metals (chromium or cadmium), it is rarely alone. Nickel ingested by inhalation usually leads to respiratory problems such as reduced olfactory acuity, ulcers, septum perforation or tumors of the nasal sinuses. This case demonstrates the importance of occupational anamnesis and awareness of diagnosis. Int J Occup Med Environ Health 2017;30(6):963-967.