Tubulointerstitial Nephritis and Uveitis Syndrome During the COVID-19 Pandemic: A Case Series.

Objectives: To report the ocular findings, laboratory results, and management of patients with tubulointerstitial nephritis and uveitis syndrome (TINU), whose numbers increased during the 2019 coronavirus disease (COVID-19) pandemic. Materials and Methods: Demographic characteristics, ophthalmic examination findings, laboratory results including polymerase chain reaction (PCR) test for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), serum SARS-CoV-2 immunoglobulin G (IgG) antibody, and treatment of patients diagnosed with TINU between March 2020 and March 2022 were evaluated retrospectively. Results: The study included 19 eyes of 10 patients (6 female/4 male). The mean age was 13.5±2.4 years (range: 8-16 years). The mean follow-up duration was 13.5±6.1 months (range: 6-24 months). All patients presented with anterior uveitis. Anterior uveitis was bilateral in 9 patients (90%) and unilateral in 1 patient (10%). Posterior segment findings were normal in 8 patients (80%), and bilateral optic disc edema was observed in only 2 patients (20%). None of the patients had a previous SARS-CoV-2 infection and/or vaccination history. The SARS-CoV-2 PCR test was negative in all patients at presentation. The SARS-CoV-2 IgG antibody test was reactive in 7 patients (70%). Recurrent uveitis developed in 8 patients (80%) during follow-up. Systemic immunomodulatory therapy was required for the control of ocular inflammation in 7 patients (70%) with severe uveitis flare-ups. Conclusion: TINU is a multisystemic autoimmune disease, especially in response to environmental triggering factors such as viral infections. Although TINU is a rare disease, the number of cases increased during the COVID-19 pandemic. SARS-CoV-2 antibodies were detected at a significant rate of 70% in these patients, who did not have a history of SARS-CoV-2 infection and vaccination. Previous asymptomatic SARS-CoV-2 infection in children may be a triggering factor in the development of TINU.

Neonatal Hearing Screening: Challenges of COVID-19 Pandemic.

This is a retrospective chart review of newborns referred to audiology between June and December 2020. Newborns who failed or did not have an initial newborn hearing screening (NBHS) at an academic, tertiary care medical center, either in the nursery or in the neonatal intensive care unit (NICU) prior to discharge, were referred for a follow-up audiology appointment. Forty-three patients were included who failed or did not receive an NBHS. Of 43 patients, 15 (34.9%) did not receive an initial hearing test due to coronavirus disease 2019 (COVID-19 protocols). Of the 15, 7 (16.3% of total 43) newborns had mothers who refused COVID-19 screening, and 5/7 were lost to follow-up (11.6% of total). Another 7/15 newborns (16.3% of total) had a COVID-19-positive mother, and 1 of these 7 was lost to follow-up (2.3% of total). One of the 15 newborns (2.3% of total) had a pending maternal COVID-19 test result at discharge, and this patient was also lost to follow-up.

The status of the choroid in patients with optic disc drusen.

PURPOSE: To investigate the subfoveal and peripapillary choroidal thickness (CT) and the choroidal vascularity index (CVI) in patients with optic disc drusen (ODD). METHODS: This cross-sectional study examined the eyes of 17 patients with ODD and 18 healthy control subjects. The CT values were calculated manually from the images captured by enhanced depth imaging-optical coherence tomography (EDI-OCT). The CVI was defined as the proportion of the vascular area to the total choroidal area at the subfoveal and peripapillary areas after binarization of the EDI-OCT images. RESULTS: It was found that the mean subfoveal CVI value in the ODD group was significantly lower than that in the control group (p = 0.006). The mean peripapillary CVI values were significantly lower in all of the quadrants in the ODD group when compared with the control group (p = 0.008 for the temporal quadrant, p = 0.014 for the nasal quadrant, p = 0.024 for the superior quadrant, and p = 0.038 for the inferior quadrant). Regarding the CT, there were no significant differences in the subfoveal and peripapillary CT values between the ODD group and the control group (p >  0.05 for all values). CONCLUSION: The findings of this study indicate ODD to be associated with decreased subfoveal and peripapillary CVI, even though the subfoveal and peripapillary CT values were within the normal range. This result may prove important in relation to identifying a choroidal vascular network that appears to be morphologically normal but microstructurally impaired due to ODD. Further studies are required to verify the significance of CVI in the pathogenesis and complications of ODD.

Relationship between chewing ability and malnutrition, sarcopenia, and frailty in older adults.

BACKGROUND: Several studies reported that impaired nutrition is associated with reduced muscle mass, muscle strength, and physical performance. Chewing ability is essential to maintain balanced oral nutrient intake. The study was designed to define the possible relationship between chewing ability and nutrition-related problems (malnutrition, sarcopenia, and frailty) in a holistic perspective. METHODS: This cross-sectional study recruited adults aged ≥65 years. All patients were evaluated with comprehensive geriatric assessment. Sarcopenia was diagnosed according to European Working Group on Sarcopenia in Older People criterion. Malnutrition was determined according to body mass index, calf circumference, and Mini Nutritional Assessment short form (MNA-SF). Frailty status was diagnosed with the Clinical Frailty Scale. Masseter and gastrocnemius muscle thicknesses (MTs) were measured via ultrasonography imaging. Oral examinations were carried out by a dentist, and chewing performance was examined with a color-changeable chewing gum. RESULTS: Overall, 135 older adults (76 females) were analyzed. Mean ± SD age was 75.7 ± 7.2 years; 37.0% of the patients were frail, 3.7% were malnourished, 12.6% were sarcopenic, and 20.0% had poor chewing function. In the poor chewing function group, age and frailty scores were increased and the MNA-SF scores, handgrip strength, skeletal muscle index, and masseter MT were reduced (all P < 0.05). After adjusting for confounders, regression analysis showed that low grip strength and low gastrocnemius MT were independently associated with poor chewing ability. CONCLUSIONS: Chewing ability was related to sarcopenia. Age and low grip strength in females and low cognitive scores and having low gastrocnemius MT in males were independent variables affecting chewing ability.

Peripapillary and Macular Choroidal Vascularity Index in Eyes with Fuchs' Uveitis.

BACKGROUND: To investigate the choroidal vascularity alterations of macula and peripapillary area in eyes with unilateral Fuchs uveitis (FU). METHODS: This study included 18 eyes with unilateral FU and 18 healthy fellow eyes. The choroidal vascularity index (CVI) was analyzed, and the results were compared between eyes with FU and healthy fellow eyes (control group). RESULTS: The mean subfoveal (s)CVI (%) value was significantly lower in the FU group as compared with the control group: 60.92 ± 2.63 vs. 64.01 ± 2.54, respectively (p = .001). The mean pCVI (%) values of superior and inferior quadrant were significantly lower in the Fuchs group as compared with the control group:58.87 ± 4.48 vs. 61.98 ± 3.32, respectively, in superior quadrant (p = .035); and 57.29 ± 6.65 vs. 61.44 ± 4.76, respectively, in inferior quadrant (p = .014). CONCLUSION: The chronic continuous inflammation in FU causes inflammation-mediated thinning in the vascular area of the subfoveal and peripapillary choroid. The changes in the peripapillary region are remarkable in terms of the further glaucoma risk of these eyes.

Steroid eluting biocompatible stent for subglottic stenosis.

OBJECTIVE: Develop a prototype steroid eluting stent suitable for endoscopic treatment of subglottic stenosis. METHODS: Rectangular-shaped spoke design stents thermally molded into horseshoe-shaped stents were developed using AutoCAD program, and printed on a Lulzbot 3D printer with polycaprolactone (PCL). Kenalog saturated AEROSIL 200 was embedded in the PCL filament. Horizontal radial force measurements were measured at baseline, 1 day, and 1 month when deformation switched from bending to compression. Amount of Kenalog eluted after 1 day, 1 week and 1 month were measured using HPLC. RESULTS: Horizontal pressure applied to the PCL stent corresponding to a 5-0 ET were 1.27 ± 0.38 lb. at baseline, 1.79 ± 0.045 lb. at 1 day, 1.94 ± - 0.22 lb. at 1 week and 2.07 ± 0.11 lb. at 1 month. The horizontal pressure applied to PCL stent corresponding to an 8-0 ET tube were 0.82 ± 0.018 lb. at baseline, 1.008 ± 0.045 lb. at 1 day, 0.95 ± - 0.064 lb. at 1 week and 1.078 ± 0.021 lb. at 1 month. The amount of Kenalog eluted increased from 5.78 µg/mL at 1 day to 15.01 µg/mL at 1 week to 19.35 µg/mL at 1 month. CONCLUSION: This proof-of-concept project is an initial step to demonstrate and create a novel stent in the treatment of subglottic stenosis that applies expansile force on the trachea, elutes steroids and dissolves. Over time the expansile force along the trachea increases allowing the PCL to mucosalize, while it dissolves and continues to elute steroids. The limitations of this in vitro study necessitate experiments on animal models, such as rabbit tracheas to observe for complications and histologic changes. LEVEL OF EVIDENCE: This proof-of-concept project is a Level 5 mechanism-based reasoning study.

Intracranial Mass Lesion in a Patient Being Followed up for Amblyopia

A 12-year-old boy being followed up for amblyopia presented to our hospital with visual disturbance in the left eye. The patient's best corrected visual acuity on Snellen chart was 1.0 in the right eye and 0.3 in the left eye. Increased horizontal cup-to-disc ratio was detected on dilated fundus examination. Retinal nerve fiber layer measurement showed diffuse nerve fiber loss and visual field test showed bitemporal hemianopsia. Magnetic resonance imaging revealed a lesion that filled and widened the sella and suprasellar cistern and compressed the optic chiasm. The patient was operated with transcranial approach. The pathologic examination revealed craniopharyngioma.

Needle aspiration as primary surgical treatment of pediatric deep neck space abscesses.

OBJECTIVE: Examination of the outcomes of needle aspiration (NA) under sedation as the primary surgical treatment for pediatric deep neck space abscesses (DNSA) to determine its adequacy, safety, and cost. STUDY DESIGN: Retrospective chart review. METHODS: 10 consecutive pediatric patients (age 4-48 months) that were diagnosed with DNSA starting from August 2008 through October 2015 were included in our review. All patients were on antibiotics and were treated with NA as the primary surgical treatment modality. Procedures were all performed in our pediatric sedation suite. We have examined our outcomes including need to convert to open incision and drainage (I&D), number of aspirations required, hospital stay, if purulence obtained, culture results, and imaging modality used. We also compared our results with previous studies using incision and drainage as the primary treatment modality focusing on the duration of their hospital stay. RESULTS: None of our 10 patients required an open I&D. Two of 10 (20%) did require repeat aspiration once with no patient requiring more than two aspirations. Median hospital stay was 4 days (range 3-8). CONCLUSION: In our small study group NA performed under sedation was an effective treatment modality with duration of hospital stay comparable to other studies that included treatment with I&D under general anesthesia.

Validation of Nutritional Risk Screening-2002 in a Hospitalized Adult Population.

BACKGROUND: Malnutrition in hospitalized patients is a serious problem and is associated with a number of adverse outcomes. The Nutritional Risk Screening-2002 (NRS-2002) tool was designed to identify patients at nutrition risk. The validation of NRS-2002 compared with detailed clinical assessment of nutrition status was not studied before in hospitalized Turkish adults. The aim of this study is to determine validity, sensitivity, and specificity of the Turkish version of NRS-2002 in a hospitalized adult population. METHODS: A total of 271 consecutive hospitalized patients aged >18 years admitted to surgical and medical wards of a university hospital in Turkey were included in this single-center non interventional validity study. Assessment by geriatricians was used as the reference method. Two geriatricians experienced in the field of malnutrition interpreted the patients' nutrition status after the evaluation of several parameters. Patients were divided into "at nutrition risk" and "not at nutrition risk" groups by geriatricians. Concordance between the 2 geriatricians' clinical assessments was analyzed by κ statistics. Excellent concordance was found; therefore, the first geriatrician's decisions were accepted as the gold standard. The correlation of nutrition status of the patients, determined with NRS-2002 and experienced geriatrician's decisions, was evaluated for the validity. RESULTS: NRS-2002 has a sensitivity of 88% and specificity of 92% when compared with professional assessment. The positive and negative predictive values were 87% and 92%, respectively. Testretest agreement was excellent as represented by a κ coefficient of 0.956. CONCLUSIONS: NRS-2002 is a valid tool to assess malnutrition risk in Turkish hospitalized patients.

Auto-obliteration of maxillary sinuses through osteoneogenesis in children with cystic fibrosis: A possible new way to reduce morbidity.

BACKGROUND: Cystic Fibrosis is an autosomal recessive disorder with a mutation in the cystic fibrosis transmembrane regulator gene, leading to dysregulation of epithelial fluid transport, dehydration of airway surfaces and impaired mucociliary function in the sinuses, lungs, pancreas and other organs. This leads to thickened secretion which blocks the sinus ostia and promotes chronic bacterial overgrowth and destruction of the mucosa. Chronic rhinosinusitis in cystic fibrosis patients leads to a decreased quality of life involving increased hospitalizations, infections, pneumonia, and acute exacerbations, warranting multiple sinus surgeries. This study investigates the effects of a more aggressive surgical approach on reducing the need for repeat surgeries. METHODS: A retrospective review of CT scans of nine cystic fibrosis patients who have had aggressive endoscopic sinus surgery for the treatment of chronic rhinosinusitis (CRS) was performed. The decrease in maxillary sinus volume was measured using pre-operative and post-operative scans. RESULTS: A significant decrease in maxillary sinus volume was observed in all subjects. Volume reduction occurred through osteoneogenesis, causing auto-obliteration of the sinuses with cancellous bone. CONCLUSIONS: Aggressive endoscopic sinus surgery for maxillary sinuses incites an osteoneogenetic reaction that leads to auto-obliteration of the sinus and reduces sinus-related morbidity in children with cystic fibrosis. Through this osteoneogenesis, the sinus is transformed into a shallow cup that does not retain secretions and is easy to clear. It is our impression that this may lead to reduced morbidity and a decreased need for repeat maxillary sinus surgery.

Immune thrombocytopenia of childhood responsive to tonsillectomy in the setting of chronic tonsillitis: A case report and literature review.

OBJECTIVES: Immune thrombocytopenia of childhood (platelet count <100,000/µL) is the most common cause of thrombocytopenia in children. Patients typically present with bruising and bleeding in the setting of thrombocytopenia. Although it is usually short-lived, some cases persist and are unresponsive to treatment. This can lead to exposure to a variety of treatment regimens including immunosuppressants and splenectomy. The goal of this report is to present a case of chronic ITP of childhood that responded to tonsillectomy addressing the tonsils as a source of chronic infection and inflammation triggering ITP. METHODS: A 4-year-old male with ITP of childhood presented with enlarged tonsils and obstructive sleep apnea. History and physical were consistent with chronic tonsillitis/adenoiditis including malaise, poor oral intake, congestion, rhinorrhea, tonsil hypertrophy, and lymphadenopathy persisting despite antibiotic therapy. Tonsillectomy and adenoidectomy were performed. RESULTS: One, six, and eighteen weeks post-operatively the platelet count was 371, 215, and 205 respectively. Although at 12months two relapses had occurred, during the observed period, he had decreased incidence and severity of disease. CONCLUSION: In around 60% of ITP there is a history of prior infection within the last month but no systemic symptoms at time of diagnosis. Additionally, chronic ITP is characterized by relapses coinciding with infection. This case is unique because the patient had chronic ITP and a clinical history and physical exam concerning for a subclinical, indolent inflammatory process that responded to surgical intervention. Given that chronic ITP exacerbation has been associated with recurrent acute infections it seems probable that chronic tonsillitis could serve as a trigger for relapse or contribute to a prolonged and/or more severe disease course. Therefore, tonsillectomy may result in earlier treatment and/or an altered disease course with avoidance of the expense and morbidity associated with frequent exacerbations and multiple treatment regimens.

Oral foregut duplication cysts: A rare and fascinating congenital lesion. Case report and review of the literature.

Oral foregut duplication cysts are extremely rare lesions with approximately 57 cases reported. They are congenital cysts, located in the anterior or ventral tongue, and occur predominantly in males. They are lined by one or more types of epithelia which is limited to gastric, intestinal or respiratory epithelium. The differential diagnosis includes lymphangioma, hemangioma, ranula, epidermoid cyst, teratoma and less likely a malignant process.

Advanced airway management strategies for severe OSAS and craniofacial anomalies.

Pediatric OSAS and craniofacial malformations present challenges that require innovative approaches and comprehensive treatment strategies. Synchronous airway lesions, craniofacial malformations, obstructive anomalies of the tongue base, nasal vault and choanae are commonly addressed by subspecialists from various clinical and surgical academic traditions who practice variable levels of required communication. This is not a mere social requirement but an important requisite for intelligent and effective airway management. Membership of dedicated airway, aero digestive or craniofacial teams are desirable but not required. I expect this clinical brief to help many brilliant clinicians in their pursuit of perfection.

A 14-year-old boy with extranodal natural killer cell lymphoma of the nose, nasopharynx, larynx, and trachea in remission 6years after primary diagnosis. A longitudinal case report.

Nasal type extranodal natural killer/T-cell lymphoma (ENKTL) is a rare lymphoma in the USA and Europe but endemic in East Asia and in areas of South and Central America. Clinically natural killer cell lymphomas are divided into three categories; nasal, non-nasal and aggressive lymphoma/leukemia subtypes. ENKTL, nasal type occurs in the nose and can extend to the upper aero-digestive tract as reported in this longitudinal case study. This is a longitudinal report of progress of a 14-year-old boy with ENKTL originating in the nasal cavity with subsequent extension and recurrence in the contralateral nose, nasopharynx, larynx and trachea presenting with varying degrees of respiratory problems and eventually, respiratory distress. Caregiver refusal of stem cell transplantation prompted an alternative diagnostic and therapeutic approach. Clinical course with recurrences, extensions and remissions over 6years with tailored endoscopic surgical treatment and radiochemotherapy is documented to present a guide in the multidisciplinary management of this rare disease.

Non tuberculous mycobacterial lesion of the parotid gland and facial skin in a 4year old girl: A proposed treatment strategy.

OBJECTIVE: We report a case of a parotid-facial caseating granulomatous infection caused by atypical mycobacteria (Mycobacterium avium) in an immuno-competent child. The size and depth of the lesion and its proximity to the facial nerve present a challenge for a purely surgical treatment strategy. An alternative treatment strategy is developed to avoid severe disfigurement. STUDY DESIGN/SUBJECT: Atypical mycobacterial infection of the parotid region in a 5 year old girl: timeline and definition of a planned combined treatment strategy with antibiotics and surgical excision. RESULTS/CONCLUSION: Cervicofacial infections caused by non-tuberculous mycobacteria (NTM) may present surgical challenges due to the size and depth of the lesion and its proximity to the facial nerve and major vascular structures. Even minor scars are highly visible and poorly tolerated. Close clinical monitoring combined with judicious treatment strategies is necessary for successful treatment and good cosmesis. Recent literature provides insufficient guidance in formulating the best treatment strategy for the individual patient. Comparisons of antibiotic therapy with variations of surgical excision are abundant but poorly formulated. Our case presented with a lesion involving skin, superficial and deep lobe of the parotid gland. Lesion was in immediate proximity to the distribution of the facial nerve through the parotid gland. The risk of surgical damage to the facial nerve in the acute phase of the inflammation and the required extent of skin excision were significant. We decided to start treatment with combination antimycobacterial antibiotics in close cooperation with the pediatric infectious disease specialists. We observed and documented the regress and executed a delayed surgical excision when the lesion was reduced to skin only. In our opinion this was the best treatment strategy that helped us avoid extensive dissection in the vicinity of the facial nerve as well as a parotidectomy. Excision of the involved skin with the deep portion was performed 6.5 months after initial diagnosis.

Evaluation of growth curves in children after supraglottoplasty.

OBJECTIVES: To determine if monitoring weight growth curves is a sensitive objective parameter for evaluating operative outcomes after supraglottoplasty. STUDY DESIGN: Retrospective chart review. METHODS: An IRB approved retrospective review of patients who underwent supraglottoplasty from 2/28/2012 to 10/20/2014 by the otolaryngology department at a single institution was performed. Variables collected included age, race, sex, preoperative weight percentiles, and weight percentiles at 3 month, 12 month, and 3 year followup intervals. RESULTS: 20 patients met inclusion criteria. 15 (75%) patients were male and 5 (25%) were female. 9 (45%) patients were African American, 8 (40%) were Caucasian, and 3 (15%) were other. Average weight for age at surgery was 29.8 percentile. 6 (30%) had failure to thrive by weight. By 3 months postop average weight had increased by 7.67 percentile (p=0.09, 95% CI -1.62 to 17.0), by 12 months there was an observed increase of 19.1 percentile (p=0.06, 95% CI 0.47-37.8), and by 3 years the average weight had increased by 26.53 percentile (p=0.03, 95% CI 4.47-48.59). By three years postop the average weight had normalized (64.5 percentile). Among those who met preoperative failure to thrive criteria (average 0.11 percentile), weight gain was still dramatic with average weight percentile of 37.5 by 3 years postop. CONCLUSION: Patients undergoing supraglottoplasty are typically underweight for age. Statistically significant weight gain occurs in children after going supraglottoplasty. This intervention can normalize their growth chart growth patterns by 3 years postoperatively, even in children with failure to thrive.

Treatment Options for Pediatric Obstructive Sleep Apnea.

There are a variety of therapies available for the treatment of pediatric obstructive sleep apnea syndrome (OSAS). In children with enlarged adenoids or tonsils, adenotonsillectomy (AT) is the preferred treatment, but other surgical options include partial tonsillectomy and lingual tonsillectomy. In specific populations, craniofacial or bariatric surgery may be indicated, and tracheostomy should be reserved for cases where there is no other therapeutic option. Positive airway pressure (PAP) is the most effective non-surgical therapy for OSAS as it can be successfully used in even cases of severe OSAS. Nasal steroids and leukotriene receptor antagonists may be used in the treatment of mild or moderate OSAS. Rapid maxillary expansion and dental appliances may be effective in select populations with dental problems. Other non-surgical therapies, such as positional therapy, supplemental oxygen, and weight loss have not been shown to be effective in most pediatric populations.

Maxillofacial extramedullary hematopoiesis in a child with sickle cell presenting as bilateral periorbital cellulitis.

Review of a case of paraosseous extramedullary hematopoiesis (EMH) affecting the maxillary sinuses and retro-orbital spaces imitating bilateral orbital cellulitis. Maxillofacial EMH causes diagnostic/therapeutic challenges. This case report describes a 4-year-old African American male with sickle cell disease (HbSS) who presented with bilateral orbitofacial swelling. Diagnosis was made with imaging and confirmed with tissue sampling. Partial exchange transfusion was utilized to stop the progression of maxillofacial EMH and to treat the patient's chronic anemia. Follow-up MRI showed regression of orbital and retro-orbital involvement. Early treatment with conservative modalities and close observation may prevent need for more invasive treatments.

Balloon dilatation for congenital nasal piriform aperture stenosis (CNPAS): a novel conservative technique.

Congenital nasal piriform aperture stenosis (CNPAS) is an uncommon but important cause of nasal obstruction in infants. When it is severe, nasal decongestants or temporary nasal airway devices cannot address the problem adequately. The most common surgical technique used to treat the bony piriform aperture stenosis is the sublabial approach and drill-out of the nasal aperture followed by nasal stenting. This paper describes an infant with bilateral CNPAS who underwent a single successful balloon dilatation and short-term stenting of the nasal piriform apertures without the need for additional procedures.