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1.
Am J Ophthalmol ; 2024 May 25.
Article in English | MEDLINE | ID: mdl-38801873

ABSTRACT

PURPOSE: To compare outcomes of primary lensectomy (PL) versus no lensectomy (NL) during repair of zone I (involving cornea and limbus) and II (up to 5mm posterior to the limbus) ruptured globe injuries (RGIs) with lens involvement. DESIGN: Retrospective clinical cohort study. METHODS: 107 patients with RGIs involving both blunt and penetrating injury to the lens who presented to Wills Eye Hospital between April 1, 2017 and August 31, 2022 were included. Data from presenting visit was collected including demographic information, time from injury to surgery, visual acuity (VA), intraocular pressure (IOP), injury characteristics, and years since residency graduation of surgeon. VA, IOP, retinal detachment (RD) rate, and endophthalmitis incidence were compared between PL and NL groups at postoperative week 1 (POW1) and postoperative month 1 (POM1). VA, peak IOP, need for further surgeries, and types of additional surgery were compared between the two groups at the final visit. RESULTS: 19 (17.8%) patients underwent PL. Age, sex, and initial VA were similar between groups (all p≥0.05). The PL group had surgery later from the time of injury (6.1±14.6 days vs. 1.3±1.9 days; p=0.010), higher IOP at presentation (12.9±11.6 mmHg vs. 7.7±11.3 mmHg; p=0.046), shorter wounds (2.3±1.4mm vs. 4.7±3.2mm; p=0.003), more frequent lens capsule violation (89.5% vs. 50%; p=0.010), increased likelihood of intraocular foreign bodies (52.6% vs. 17.0%; p=0.004), and were more likely to be operated on by surgeons with ≥ 5 years of experience post-residency (68.4% vs. 28.4%; p<0.001). At POW1, the PL group had significantly better logMAR VA (1.2±0.9 vs. 2.0±1.0; p=0.002), and this continued at POM1 (1.0±0.8 vs. 1.6±1.1; p=0.031) and the final visit (0.4±0.7 vs. 1.0±1.1; p=0.010). The PL group had lower IOP at POW1 (12.4±3.0 mmHg vs. 17.3±8.2 mmHg; p=0.005) than the NL group. There was no difference in RD or endophthalmitis rates between the two groups at POW1 or POM1 (p>0.05 for all). The NL group was more likely to require additional surgery by final follow-up (77.3% vs. 47.4%; p<0.001). In the multivariable analysis, PL had better final VA and decreased need for further surgery (both p<0.05). CONCLUSIONS: In the appropriate circumstance, PL during lens-involving anterior RGI repair may lead to improved patient outcomes.

2.
J AAPOS ; 27(5): 276.e1-276.e8, 2023 10.
Article in English | MEDLINE | ID: mdl-37739211

ABSTRACT

PURPOSE: To compare amblyopia treatment outcomes between patients with neurodevelopmental disorders and their typically developing peers. METHODS: Of 2,311 patients diagnosed with amblyopia between 2010 and 2014 at Boston Children's Hospital, 460 met inclusion criteria (age 2-12 with anisometropic, strabismic, or mixed amblyopia [interocular difference (IOD) ≥2 lines]). Treatment and visual outcomes were analyzed according to neurodevelopmental status: neurodevelopmental delay (DD) versus typical development (TD). RESULTS: The DD group (n = 54) and TD group (n = 406) were similar in demographics, amblyogenic risk factors, baseline visual measures, prescribed therapy, and adherence (P ≥ 0.10). Between-visit follow-up time was longer for the DD group (0.65 [0.42- 0.97] years) than for the TD group (0.5 [0.36-0.82] years; P = 0.023). IOD improved similarly in each group by the last visit (DD, -0.15 logMAR [-0.31 to -0.02]; TD, -0.2 logMAR [-0.38 to -0.1]; P = 0.09). Each group reached amblyopia resolution by the last visit at similar frequencies (DD, 23/54 [43%]; TD, 211/406 [52%]; P > 0.2). DD diagnosis did not independently influence amblyopia resolution (HR, 0.77; 95% CI, 0.53-1.12; P = 0.17), but each additional month of interval time between follow-up visits reduced the likelihood of resolution by 2.7% (HR, 0.67; 95% CI, 0.51-0.87; P = 0.003). CONCLUSIONS: Patients with DD and those with TD responded similarly to amblyopia therapy; however, follow-up intervals were longer in patients with DD and correlated with the likelihood of persistent amblyopia, suggesting that greater efforts at assuring follow-up may benefit patients with DD.


Subject(s)
Amblyopia , Neurodevelopmental Disorders , Child , Humans , Child, Preschool , Amblyopia/therapy , Amblyopia/etiology , Visual Acuity , Treatment Outcome , Risk Factors , Neurodevelopmental Disorders/complications , Sensory Deprivation , Follow-Up Studies
3.
Am J Ophthalmol Case Rep ; 30: 101828, 2023 Jun.
Article in English | MEDLINE | ID: mdl-37252050

ABSTRACT

Purpose: To report a patient with conjunctival and buccal neuromas and enlarged corneal nerves without Multiple Endocrine Neoplasia 2B (MEN2B). Observations: A 28-year-old female presented with progressively enlarging bilateral limbal conjunctival growths. Slit lamp examination was notable for enlarged corneal nerves and well-circumscribed gelatinous subepithelial limbal nodules. Systemic examination revealed similar lesions on the tongue. Conjunctival biopsy demonstrated a mucosal neuroma. The patient underwent endocrine workup for MEN2B and genetic testing for the RET-proto oncogene mutations, all of which were negative. Conclusions and Importance: The findings in our patient may be compatible with pure mucosal neuroma syndrome. The pattern of conjunctival neuromas and enlarged corneal nerves should raise concern for MEN2B, a hereditary tumor predisposition syndrome with almost 100% incidence of medullary thyroid cancer, unless prophylactic thyroidectomy is performed. Accurate diagnosis and prompt referral for endocrine and genetic testing is critical. Isolated mucosal neuromas without endocrine manifestations of MEN2B can rarely occur in a "pure mucosal neuroma syndrome," which is a diagnosis of exclusion in a setting of a negative workup.

4.
Brain Inj ; 37(6): 534-540, 2023 05 12.
Article in English | MEDLINE | ID: mdl-36951423

ABSTRACT

OBJECTIVE: The head and intraocular trauma tool (HITT) is a portable, binocular retinal polarization scanner (RPS) that detects ocular fixation with high precision to assess visuomotor function. We conducted a pilot evaluation of a prototype binocular RPS device to evaluate alterations in fixation stability, binocularity (convergence), and saccadic latency after mild traumatic brain injury (mTBI). METHODS: Two groups were studied prospectively: (1) single observation study of mTBI patients in a hospital ER (n = 7) and age-matched controls (n = 43); (2) high-school athletes preseason (n = 28), after sports-related mTBI (n = 3), and at season end (n = 5). Subjects were asked to fixate on an internal target and track randomly presented peripheral and central targets as fixation was assessed using binocular RPS. RESULTS: There were clinically and statistically significant alterations in the hospital ER group after mTBI, including a decrease in fixation stability (54.6% in patents vs 90.2% in controls, p = 0.014) and binocularity (28.7% in patients vs 86.6% in controls; p = 0.004). Similar trends, not statistically significant, were observed in saccadic latency in the hospital ER group as well as in the injured high school athletes. CONCLUSION: The HITT device shows promise as an objective, noninvasive method for assessment of the impact of mTBI on visuomotor function. Additional studies with larger patient populations are required to evaluate efficacy for clinical use.


Subject(s)
Brain Concussion , Brain Injuries , Sports , Humans , Brain Concussion/complications , Brain Concussion/diagnostic imaging , Athletes , Schools
5.
Am J Ophthalmol ; 251: 156-164, 2023 07.
Article in English | MEDLINE | ID: mdl-36543314

ABSTRACT

PURPOSE: Published studies of amblyopia include only patients with visual acuity (VA) worse than 20/40 in one or both eyes. The purpose of this study is to evaluate patients diagnosed and treated as amblyopic despite not meeting traditional VA criteria for amblyopia. DESIGN: Retrospective clinical cohort study. METHODS: Setting: Institutional practice. PATIENT POPULATION: All patients diagnosed with amblyopia at Boston Children's Hospital between 2010 and 2014. INCLUSION CRITERIA: VA better than 20/40 but not correctable to 20/20 in one or both eyes; age 2 to 12 years. OBSERVATIONS: Demographics, VA, baseline characteristics. OUTCOME MEASURES: Resolution, defined as VA 20/20 in both eyes; stereopsis at the last follow-up. RESULTS: Of 2311 patients reviewed, 464 (20.1%) had subthreshold amblyopia. A majority (61.7%) had an amblyogenic factor, most commonly anisometropia (32.8%). Patients were followed for a median of 3.1 years; nearly all (97.5%) were treated. Of 318 patients who returned for follow-up, 47.8% achieved resolution, including 55.7% of treatment-naïve patients, and 62.5% (5 of 8 patients) offered observation alone. Median stereopsis improved by 0.4 log units in those who achieved resolution, with no change in those with persistent amblyopia. In the multivariate analysis, a longer length of follow-up was significantly associated with resolution of subthreshold amblyopia (odds ratio: 1.38; 95% confidence interval: 1.22-1.57, P < .001). CONCLUSIONS: Patients with subthreshold amblyopia represent a sizeable cohort in real-world amblyopia practice. When offered treatment, half achieved 20/20 vision in both eyes with improved stereopsis as well. Further studies are needed to assess whether observation alone would result in similar outcomes.


Subject(s)
Amblyopia , Anisometropia , Child , Humans , Child, Preschool , Amblyopia/diagnosis , Amblyopia/therapy , Amblyopia/complications , Retrospective Studies , Cohort Studies , Visual Acuity
6.
J AAPOS ; 27(1): 20.e1-20.e5, 2023 02.
Article in English | MEDLINE | ID: mdl-36581151

ABSTRACT

PURPOSE: To demonstrate the use of a novel measure of neighborhood quality, the Child Opportunity Index (COI), for investigating health disparities in pediatric ophthalmology. METHODS: This study included children 2-12 years of age from a registry of patients diagnosed with amblyopia at an urban pediatric hospital between 2010 and 2014. Children previously treated for amblyopia were excluded. Patient demographics, residential addresses, and logMAR visual acuities were collected. The association between visual acuity at presentation and COI was examined using linear mixed-effects models adjusting for individual-level factors, including age, sex, race, ethnicity, and insurance type. RESULTS: This study included 1,050 amblyopic children, of whom 317 (37%) were non-White and 149 (19%) were Hispanic; 461 (44%) had public insurance. Regarding residence, 129 (12%) lived in areas of very low opportunity (COI <20); 489 (47%) in areas of very high opportunity (COI ≥80). Children residing in the lowest opportunity neighborhoods correctly identified approximately two fewer letters at presentation with their better-seeing eye compared with children from the highest opportunity neighborhoods after adjusting for individual-level factors (-0.0090 logMAR per 20 unit increase in COI; 95% CI, -0.0172 to -0.0008; P = 0.031). No difference was appreciated in the worse-seeing eye. CONCLUSIONS: Amblyopic children residing in communities with low neighborhood opportunity had slightly worse visual acuity in the better-seeing eye at presentation. Although statistically significant in the better-seeing eye, the two-letter difference attributable to neighborhood environment may not be clinically significant, and the impact of this disparity on treatment outcomes deserves further investigation.


Subject(s)
Amblyopia , Child , Humans , Amblyopia/diagnosis , Visual Acuity , Treatment Outcome
7.
Am J Ophthalmol ; 233: 48-56, 2022 01.
Article in English | MEDLINE | ID: mdl-34303687

ABSTRACT

PURPOSE: Many clinicians treat unilateral amblyopia with glasses alone and initiate patching when needed; others start glasses and patching simultaneously. In this study, we reviewed the outcomes of the two approaches at our institution. DESIGN: Retrospective nonrandomized clinical trial. METHODS: Setting: Institutional practice. PATIENT POPULATION: All patients diagnosed with amblyopia at Boston Children's Hospital between 2010 and 2014. INCLUSION CRITERIA: Unilateral amblyopia (visual acuity (VA) 20/40 to 20/200 with interocular difference ≥3 lines,) age 3 to 12 years, with a 6-month follow-up visit. EXCLUSION CRITERIA: Deprivation amblyopia, prior amblyopia treatment, treatment other than patching, surgery. Patients were categorized as "simultaneous treatment" (concurrent glasses and patching therapy at their first visit) or "sequential treatment" (glasses alone at first visit, followed by patching therapy at second visit.) Observation procedures: Patient demographics, VA, and stereopsis were compared. OUTCOME MEASURES: VA and stereopsis at the last visit on treatment. RESULTS: We identified 98 patients who met inclusion criteria: 36 received simultaneous treatment and 62 sequential treatment. Median amblyopic eye VA improved similarly between the simultaneous (∆0.40; interquartile range [IQR], 0.56-0.30 logMAR) and sequential (∆0.40; IQR, 0.52-0.27 logMAR) groups. Patients without stereopsis at first visit had better stereopsis outcomes with sequential treatment (5.12 [IQR, 4.00-7.51] log stereopsis) compared with simultaneous treatment (8.01 [IQR, 5.65-9.21]) log stereopsis, P = 0.046). CONCLUSIONS: VA improved approximately 4 lines regardless of treatment type. For children without stereopsis at first presentation, sequential patching yielded better stereopsis outcomes. These findings require further validation and highlight the importance of evaluating stereopsis in future studies.


Subject(s)
Amblyopia , Amblyopia/therapy , Child , Child, Preschool , Follow-Up Studies , Humans , Retrospective Studies , Sensory Deprivation , Treatment Outcome , Vision, Binocular
8.
Clin J Sport Med ; 32(2): 114-121, 2022 03 01.
Article in English | MEDLINE | ID: mdl-33605602

ABSTRACT

OBJECTIVE: To describe the collaborative findings across a broad array of subspecialties in children and adolescents with postconcussion syndrome (PCS) in a pediatric multidisciplinary concussion clinic (MDCC) setting. DESIGN: Retrospective analysis. SETTING: Multidisciplinary concussion clinic at a pediatric tertiary-level hospital. PATIENTS: Fifty-seven patients seen in MDCC for evaluation and management of PCS between June 2014 and January 2016. INTERVENTIONS: Clinical evaluation by neurology, sports medicine, otolaryngology, optometry, ophthalmology, physical therapy, and psychology. MAIN OUTCOME MEASURES: Specialty-specific clinical findings and specific, treatable diagnoses relevant to PCS symptoms. RESULTS: A wide variety of treatable, specialty-specific diagnoses were identified as potential contributing factors to patients' postconcussion symptoms. The most common treatable diagnoses included binocular vision dysfunction (76%), anxiety, (57.7%), depression (44.2%), new or change in refractive error (21.7%), myofascial pain syndrome (19.2%), and benign paroxysmal positional vertigo (17.5%). CONCLUSIONS: Patients seen in a MDCC setting receive a high number of treatable diagnoses that are potentially related to patients' PCS symptoms. The MDCC approach may (1) increase access to interventions for PCS-related impairments, such as visual rehabilitation, physical therapy, and psychological counseling; (2) provide patients with coordinated medical care across specialties; and (3) hasten recovery from PCS.


Subject(s)
Athletic Injuries , Brain Concussion , Post-Concussion Syndrome , Adolescent , Athletic Injuries/diagnosis , Benign Paroxysmal Positional Vertigo/therapy , Brain Concussion/complications , Brain Concussion/diagnosis , Brain Concussion/therapy , Child , Humans , Post-Concussion Syndrome/diagnosis , Post-Concussion Syndrome/psychology , Post-Concussion Syndrome/therapy , Retrospective Studies
9.
Am J Ophthalmol ; 230: 200-206, 2021 10.
Article in English | MEDLINE | ID: mdl-33992614

ABSTRACT

PURPOSE: To identify demographic and disease-related characteristics predictive of Lost-to-Follow-Up (LTFU) status in amblyopia treatment and create a risk model for predicting LTFU status. DESIGN: Retrospective cohort study METHODS: Setting: Single-center, ophthalmology department at Boston Children's Hospital (BCH). PATIENTS: 2037 patients treated for amblyopia at BCH between 2010 and 2014. OBSERVATION PROCEDURE: LTFU was defined as patients who did not return after initial visit, excluding those who came for second opinion. Multiple variables were tested for association with LTFU status. OUTCOME MEASURE: Odds ratio of LTFU risk associated with each variable. Multivariate logistic regression was used to create a risk score for predicting LTFU status. RESULTS: A large proportion of patients (23%) were LTFU after first visit. Older age, nonwhite race, lack of insurance, previous glasses or atropine treatment, and longer requested follow-up intervals were independent predictors of LTFU status. A multivariable risk score was created to predict probability of LTFU (area under the curve 0.68). CONCLUSIONS: Our comprehensive amblyopia database allows us to predict which patients are more likely to be LTFU after baseline visit and develop strategies to mitigate these effects. These findings may help with practice efficiency and improve patient outcomes in the future by transitioning these analyses to an electronic medical record that could be programmed to provide continually updated decision support for individual patients based on large data sets.


Subject(s)
Amblyopia , Aged , Amblyopia/diagnosis , Amblyopia/therapy , Child , Follow-Up Studies , Humans , Logistic Models , Lost to Follow-Up , Retrospective Studies
11.
Int J Pediatr Otorhinolaryngol ; 131: 109862, 2020 Apr.
Article in English | MEDLINE | ID: mdl-31927148

ABSTRACT

OBJECTIVES: To evaluate the association between torticollis and enlarged vestibular aqueduct (EVA). METHODS: An online/phone survey was administered to parents of 133 children diagnosed with the following disorders: EVA, GJB2 (Connexin 26) mutations associated congenital hearing loss and epistaxis (control). The survey included questions regarding symptoms of torticollis, vertigo, and hearing loss. RESULTS: Patients with EVA had a 10-fold greater odds of having torticollis than controls (31% vs. 4%; OR = 10.6; 95% CI: 2.9, 39.2). No patients with GJB2 had a reported history of torticollis. Torticollis preceded the diagnosis of hearing loss in most (87%) patients with EVA who had a reported history of torticollis. EVA patients were more likely to have reported motor delay than controls (40% vs. 15%; p = 0.002). EVA patients with prior torticollis (80%; 12/15) were more likely to have balance impairment than EVA patients without prior torticollis (12%; 4/33; p < 0.001). Twelve patients had a reported history of paroxysmal torticollis, all of whom had EVA. CONCLUSION: Torticollis in infants may be a marker of EVA. Infants with torticollis should be monitored closely for hearing loss and motor delay, especially when the torticollis is paroxysmal.


Subject(s)
Hearing Loss, Sensorineural/complications , Hearing Loss/congenital , Torticollis/complications , Vestibular Aqueduct/abnormalities , Case-Control Studies , Connexin 26 , Connexins/genetics , Female , Hearing Loss/complications , Humans , Infant , Male , Mutation , Retrospective Studies , Vertigo/etiology
12.
Cureus ; 12(11): e11774, 2020 Nov 29.
Article in English | MEDLINE | ID: mdl-33409021

ABSTRACT

Coronavirus disease 2019 (COVID-19) was designated as a global pandemic by the World Health Organization (WHO) on March 11, 2020. The Cochrane Database of Systematic Reviews documents that COVID-19 has a wide range of common symptoms, which have made it difficult to characterize the disease. To date, the neurological symptoms of stuttering and word-finding difficulties have not been reported in confirmed COVID-19 cases. This case report describes the clinical course of a 53-year-old female that presented to the emergency department (ED) twice with varying symptoms consistent with COVID-19. At the second ED visit, she complained of new-onset stuttering and word-finding difficulties and tested positive for COVID-19 using the polymerase chain reaction (PCR) nasopharynx test. When contacted, the patient stated that her speech issues persisted at least seven days after discharge from her second ED visit. As a result, the virus may cause symptoms of an acute neurological event and should be taken into diagnostic consideration. These neurological findings may be explained by the recent discovery of the COVID-19 spike protein's ability to destabilize the blood-brain barrier (BBB) and enter the central nervous system (CNS). Increased classification of unrecognized COVID-19 symptoms and complications may aid in the characterization, surveillance, and prevention of the disease.

13.
J Vitreoretin Dis ; 4(6): 459-466, 2020.
Article in English | MEDLINE | ID: mdl-37007658

ABSTRACT

Purpose: This work assesses the educational use of a 3-dimensional (3D) heads-up visualization system for vitreoretinal surgery. Methods: A cross-sectional survey was performed among 18 medical students, 18 residents, 7 VR fellows, 6 nurses, 4 anesthesiologists, and 3 surgeons on their experience with the Ngenuity 3D Visualization System (TrueVision and Alcon) compared with the standard microscope. Results: Most medical students (88%) reported better identification of surgical landmarks; 63% ranked the Ngenuity as their preferred method of learning (vs microscope or 2-dimensional display). Residents reported a superior understanding of anatomy (69%), an increased ability to ask questions (63%), and improved ergonomics (94%). Epiretinal membrane peel, internal limiting membrane peel, and core vitrectomy were most enhanced (69% to 77%) for residents. Fellows' experience with trocar placement, vitrectomy, and oil/gas insertion was the same as before (67% to 100%), whereas endolaser and closing were worse (67% to 100%). Regarding autonomy, 83% of fellows reported no change, whereas 17% reported increased levels. Nurses were better able to track case progress (67%) and anticipate which tools were needed (50%). All anesthesiologists reported the same or worse engagement in cases, noting it was difficult to switch between the 3D screen and the anesthesia equipment. All surgeons reported a better awareness of trainees in the room and an improved ability to point out landmarks; 67% reported being more comfortable with resident and fellow autonomy. Conclusions: The Ngenuity is most helpful to students and residents. Integrating 3D displays into the surgical curriculum is beneficial, with care to ensure nurse and anesthesiologist satisfaction.

14.
Am J Ophthalmol ; 211: 87-93, 2020 03.
Article in English | MEDLINE | ID: mdl-31712066

ABSTRACT

PURPOSE: Many bilateral amblyopia patients have asymmetric visual acuity (VA). There is no standard treatment for these patients, and outcomes have not been well described. Our goal is to compare VA outcomes in this group based on timing of occlusion therapy. DESIGN: Retrospective interventional comparative case series. METHODS: Setting: Institutional practice. PatientPopulation: Patients diagnosed with amblyopia at Boston Children's Hospital between 2010 and 2014. InclusionCriteria: VA ≥ 0.3 logMAR bilaterally by objective optotype-based measures, interocular difference (IOD) ≥ 0.18 logMAR, age 2-12 years. ExclusionCriteria: Loss to follow-up, managed surgically, deprivation amblyopia. Patients had either primary or secondary occlusion (primary = initiated when VA ≥ 0.3 logMAR bilaterally; secondary = initiated to correct residual IOD once VA improved to ≤0.18 logMAR in the stronger eye). ObservationProcedure: Patient demographics, VA, IOD, and stereopsis were compared between groups. OutcomeMeasures: VA improvement at 12-18 months and at last visits. RESULTS: Of 2,200 patients reviewed, 167 (7.6%) had asymmetric, bilateral amblyopia; 98 met inclusion and exclusion criteria. Patients were equally divided between primary (n = 50) and secondary (n = 48) occlusion groups. There were no differences in demographics, baseline VA, or IOD between groups (P ≥ .22), although the primary occlusion group had a higher proportion of strabismic amblyopia (P = .007). VA in both eyes, IOD, and stereopsis improved similarly between groups, even after stratifying by amblyopia subtype (P ≥ .48). The secondary occlusion group was more likely to achieve 20/30 bilaterally and IOD ≤ 1 line at 12-18 months (P ≤ .4), although this equalized by the last visit. CONCLUSION: In patients with asymmetric, bilateral amblyopia, VA improved by 4 lines in the weaker eye and 2 lines in the stronger eye, while IOD improved by 2 lines, irrespective of occlusion status. Primary occlusion thus provided no further benefit over spectacle correction alone.


Subject(s)
Amblyopia/therapy , Sensory Deprivation , Therapeutic Occlusion , Visual Acuity/physiology , Amblyopia/physiopathology , Child , Child, Preschool , Depth Perception/physiology , Eyeglasses , Female , Follow-Up Studies , Humans , Male , Retrospective Studies , Vision, Binocular/physiology
15.
J AAPOS ; 23(6): 325.e1-325.e6, 2019 12.
Article in English | MEDLINE | ID: mdl-31689500

ABSTRACT

PURPOSE: To evaluate the finding of anomalous superior oblique muscles in congenital fibrosis of the extraocular muscles (CFEOM), a feature not previously emphasized in this condition. METHODS: The medical records of all patients clinically or genetically diagnosed with CFEOM at Boston Children's Hospital between 2010 and 2018 were reviewed retrospectively. Those who underwent strabismus surgery during the study period were included in the analysis. Baseline patient characteristics, type of CFEOM, results of genetic testing, and intraoperative features of the superior oblique muscle or tendon were recorded. RESULTS: Of 24 patients identified (age range, 1 month to 62 years), 10 (42%) had genetically confirmed CFEOM, and 22 underwent strabismus surgery, 14 (64%) involving the superior oblique muscle. Of these, 7 (50%) had anomalously inserted tendons (most commonly attached nasal to the superior rectus muscle), whereas 7 (50%) had increased superior oblique muscle tension. CONCLUSIONS: Half of CFEOM patients who underwent superior oblique surgery had abnormally inserted superior oblique tendons, and 50% had tight muscles or abnormally thin tendons, findings that have not been well-characterized in this condition. The findings suggest that abnormal insertion of the superior oblique muscles and tendons are additional features of the disease process in CFEOM that have not been described previously. These features may contribute to the severe upgaze limitation in CFEOM and highlight the importance of superior oblique tenotomy in surgical management.


Subject(s)
Eye Movements/physiology , Fibrosis/diagnosis , Oculomotor Muscles/physiopathology , Ophthalmologic Surgical Procedures/methods , Ophthalmoplegia/diagnosis , Tendons/surgery , Tenotomy/methods , Adolescent , Adult , Child , Child, Preschool , Female , Fibrosis/physiopathology , Fibrosis/surgery , Follow-Up Studies , Humans , Infant , Infant, Newborn , Male , Middle Aged , Oculomotor Muscles/surgery , Ophthalmoplegia/physiopathology , Ophthalmoplegia/surgery , Retrospective Studies , Young Adult
16.
Otolaryngol Head Neck Surg ; 159(2): 365-370, 2018 08.
Article in English | MEDLINE | ID: mdl-29685082

ABSTRACT

Objective To review peripheral vestibular disorders in pediatric patients with dizziness following concussion. Study Design Case series with chart review. Setting Pediatric vestibular clinic and pediatric multidisciplinary concussion clinic at a tertiary level pediatric hospital. Subjects and Methods We retrospectively reviewed 109 patients seen for dizziness following a concussion between September 2012 and July 2015. Patients were ≤20 years of age at the time of concussion. Incidences of specific peripheral vestibular disorders were assessed along with timing of diagnosis relative to the date of injury, diagnostic test findings, and treatment interventions associated with those diagnoses. Results Twenty-eight patients (25.7%) were diagnosed with peripheral vestibular disorders. None of these disorders were diagnosed prior to evaluation in our pediatric vestibular clinic or our multidisciplinary concussion clinic, which occurred a mean of 133 days (95% confidence interval, 89.2-177.3) after injury. Benign paroxysmal positioning vertigo was diagnosed in 19 patients, all of whom underwent successful canalith repositioning maneuvers. Other diagnoses included temporal bone fracture (n = 3), labyrinthine concussion (n = 2), perilymphatic fistula (n = 2), and superior semicircular canal dehiscence (n = 2). Both patients with perilymphatic fistula and 1 patient with superior semicircular canal dehiscence underwent successful surgical management, while 1 patient with superior semicircular canal dehiscence was managed nonsurgically. Conclusion Peripheral vestibular disorders may occur in pediatric patients with dizziness following concussion, but these disorders may not be recognized until symptoms have persisted for several weeks. An algorithm is proposed to guide the diagnosis and management of peripheral vestibular disorders in pediatric patients with concussion.


Subject(s)
Brain Concussion/complications , Vestibular Diseases/etiology , Adolescent , Athletic Injuries/complications , Dizziness/diagnosis , Dizziness/etiology , Female , Humans , Male , Retrospective Studies , Vestibular Diseases/diagnosis , Vestibular Function Tests
17.
Eur J Paediatr Neurol ; 22(4): 667-673, 2018 Jul.
Article in English | MEDLINE | ID: mdl-29656928

ABSTRACT

INTRODUCTION: Migraine variant disorders of childhood include benign paroxysmal torticollis of infancy (BPTI) and benign paroxysmal vertigo of childhood (BPVC). This study aimed to review our experience with BPTI and BPVC and determine the incidence of children transitioning between each of these disorders and to vestibular migraine (VM). METHODS: We retrospectively reviewed the medical records of patients seen at the Balance and Vestibular Program at Boston Children's Hospital between January 2012 and December 2016 who were diagnosed with BPTI, BPVC, and/or VM. RESULTS: Fourteen patients were diagnosed with BPTI, 39 with BPVC, and 100 with VM. Abnormal rotary chair testing was associated with progression from BPTI to BPVC (n = 8, p = 0.045). Eight (57.1%) patients with BPTI and 11 (28.2%) with BPVC had motor delay. Eleven (78.6%) patients with BPTI and 21 (53.8%) with BPVC had balance impairment. Six BPTI patients developed BPVC (42.9%), six BPVC patients developed VM (15.4%), and two patients progressed through all three disorders (2%). One BPTI patient progressed directly to VM. DISCUSSION: Most patients with BPTI will experience complete resolution in early childhood, but some will progress to BPVC, and similarly many patients with BPVC will progress to VM. Parents of children with these disorders should be made aware of this phenomenon, which we refer to as "the vestibular march." Children with BPTI and BPVC should also be screened for hearing loss, otitis media, and motor delay.


Subject(s)
Benign Paroxysmal Positional Vertigo/epidemiology , Migraine Disorders/epidemiology , Torticollis/epidemiology , Child , Child, Preschool , Disease Progression , Female , Humans , Male , Migraine Disorders/complications , Retrospective Studies , Torticollis/complications
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