Definition and Diagnostic Criteria for Pediatric Blepharokeratoconjunctivitis.

Importance: Pediatric blepharokeratoconjunctivitis (PBKC) is a chronic, sight-threatening inflammatory ocular surface disease. Due to the lack of unified terminology and diagnostic criteria, nonspecific symptoms and signs, and the challenge of differentiation from similar ocular surface disorders, PBKC may be frequently unrecognized or diagnosed late. Objective: To establish a consensus on the nomenclature, definition, and diagnostic criteria of PBKC. Design, Setting, and Participants: This quality improvement study used expert panel and agreement applying the non-RAND modified Delphi method and open discussions to identify unified nomenclature, definition, and definitive diagnostic criteria for PBKC. The study was conducted between September 1, 2021, and August 14, 2022. Consensus activities were carried out through electronic surveys via email and online virtual meetings. Results: Of 16 expert international panelists (pediatric ophthalmologists or cornea and external diseases specialists) chosen by specific inclusion criteria, including their contribution to scientific leadership and research in PBKC, 14 (87.5%) participated in the consensus. The name proposed was "pediatric blepharokeratoconjunctivitis," and the agreed-on definition was "Pediatric blepharokeratoconjunctivitis is a frequently underdiagnosed, sight-threatening, chronic, and recurrent inflammatory eyelid margin disease associated with ocular surface involvement affecting children and adolescents. Its clinical spectrum includes chronic blepharitis, meibomitis, conjunctivitis, and corneal involvement ranging from superficial punctate keratitis to corneal infiltrates with vascularization and scarring." The diagnostic criteria included 1 or more suggestive symptoms accompanied by clinical signs from 3 anatomical regions: the eyelid margin, conjunctiva, and cornea. For PBKC suspect, the same criteria were included except for corneal involvement. Conclusions and Relevance: The agreements on the name, definition, and proposed diagnostic criteria of PBKC may help ophthalmologists avoid diagnostic confusion and recognize the disease early to establish adequate therapy and avoid sight-threatening complications. The diagnostic criteria rely on published evidence, analysis of simulated clinical cases, and the expert panel's clinical experience, requiring further validation with real patient data analysis.

Polymyositis and dermatomyositis: ocular manifestations and potential sight-threatening complications.

Dermatomyositis (DM) and polymyositis (PM) are idiopathic inflammatory myopathies characterized by progressive, symmetric, mainly proximal muscle weakness. DM is also characterized by cutaneous involvement. However, other clinical features, systemic involvement, histopathological findings, response to treatment, and prognosis, differ significantly. Although uncommon, ocular manifestations in DM and PM may potentially affect any structure within the eye. Notwithstanding being generally mild, ocular involvement in DM and PM may result in significant morbidity. Left untreated, significant retinal inflammation associated with hemorrhage and detachment may occur, leading to significant vision loss. This review aims to present an up-to-date overview for rheumatologists about the ocular involvement and potential complications of DM and PM and when to refer to the ophthalmologist to avoid sight-threatening complications.

Intradural Extramedullary Primary Central Nervous System Melanoma of the Craniovertebral Junction during Pregnancy: Observations and Outcomes.

BACKGROUND: Primary central nervous system (CNS) melanoma is a rare lesion derived from neural crest precursors. While its management is analogous to metastatic spinal melanoma, the literature does not describe this entity clearly in pregnant patients and the unique implications it presents. Here, we describe the case of a pregnant patient who presented with primary CNS melanoma of the cervical spine. CASE DESCRIPTION: A 27-year-old pregnant patient presented with a 3-month history of neck and interscapular pain. MRI of the cervical spine demonstrated a ventral intradural extramedullary mass adjacent to the C2-C3 vertebral bodies causing severe cord compression. The patient was induced at 31 weeks and shortly thereafter developed quadriparesis and became obtunded. The patient underwent emergent right-sided C1 hemilaminectomy, complete C2-C4 laminectomy, and right-sided intradural division of the dentate ligaments for removal of the ventral intradural mass. Full neurological recovery was achieved before discharge. At follow-up, the infant was found to be negative for transplacental metastasis. We performed fractionated radiotherapy 4 weeks after index surgery. Nine months following index surgery, she presented with severe axial neck pain. Radiographs of the cervical spine demonstrated postlaminectomy kyphosis. The patient later underwent a posterior cervical fusion. She was recurrence-free 9 months follow-up. CONCLUSION: The differential for intradural extramedullary spinal lesions should include schwannoma, neurofibroma, meningioma, metastasis, and melanoma. Physicians caring for pregnant patients with melanoma should be aware of the potential for transplacental metastasis and perform follow-up for fetal complications.

Comparison of Dosing Schemes for Thyroid Hormone Replacement Therapy After Thyroidectomy.

BACKGROUND: Current thyroid hormone replacement therapy (THRT) is built on weight-based standard calculation of dose. A novel Poisson regression model, which accounts for seven clinical variables, was recently proposed to improve accuracy of THRT. We aimed to compare the accuracy of estimated THRT dose to reach euthyroid and the difference in predicted dose between the Poisson (scheme A) and the weight-based standard (scheme B) in patients following total thyroidectomy for benign disease. METHODS: We retrospectively reviewed medical record of patients who underwent total or completion thyroidectomy for benign disease at a single institution between 2011 and 2019. The THRT dose was calculated using both schemes. We compared the difference between calculated THRT and prediction rates for optimal THRT dosing needed to achieve a euthyroid state between dosing schemes. Patients were evaluated for achieving euthyroid state, defined as TSH 0.45-4.5 mIU/L. We also compared dosing error rates (> 25 mcg over- and underdosing) between schemes. Prediction rates were compared by BMI tertiles to account for the effect of BMI extremes in achieving euthyroid state. The difference in predicted dose between schemes was calculated in both the total sample size and patients that met euthyroid. A measure of agreement, Kappa, was used to estimate agreement between dosing schemes. RESULTS: A total of 406 patients underwent total thyroidectomy for benign disease, with 184 having sufficient follow up data confirming euthyroid state. Of the 184 patients, 85.9% (n = 158) were women, 81% (n = 149) were Hispanic, and 56.5% (n = 104) were obese with a median BMI of 30.8 kg/m2. Scheme A resulted in a higher, but not statistically significant, accuracy rate (A: 60.3%, n = 111 versus B: 53.8%, n = 99; P = 0.21). Overdosing errors were lower with Scheme A (A:17.9% versus B: 32.1%; P = 0.0025) and less extreme > 25 µg (A: 17.9% versus B: 26.1%; P = 0.08). A trend in improved accuracy in patients with a BMI > 35 kg/m2 was noted (A: 46.9% versus B: 34.4%; P = 0.20). Scheme A also resulted in less overdosing errors in obese patients compared to Scheme B (A: 19.2% versus 45.2%; P = 0.0006). The average difference in predicted dose between schemes was an entire dose difference, mean of 16.0 µg and 15.8 µg for the total and euthyroid samples respectively. Furthermore, for the majority of patients the predicted dose did not match between the two dosing schemes for total and euthyroid samples, 76% (n = 311) and 76% (n = 141) respectively. In patients that achieved euthyroid, agreement between dosing schemes was low to moderate (Kappa = 0.360). CONCLUSIONS: Lower rates of overdosing were found for scheme A, particularly with obese patients. No statistically significant differences in predicted THRT dose was observed between schemes. The difference in predicted dose between schemes was on average 15 ug, correlating with an entire dose. The consideration of clinical variables other than weight (scheme A) when determining optimal THRT dosing may be of importance to prevent overdoses, with particular clinical relevance in patients with higher BMIs.

Isolated Central Nervous System Metastasis From Neuroendocrine Carcinoma of the Cervix Without Pulmonary Metastasis.

Neuroendocrine carcinoma of the cervix (NECC) accounts for 2% of all cervical cancers. Brain metastasis is rare, with few cases described in the literature, and is usually associated with preceding pulmonary metastasis. We describe an additional case of isolated brain metastasis without pulmonary metastasis from NECC and reflect on unique management. A 37-year-old woman with a history of NECC presented with severe headache post-total hysterectomy with pelvic lymph node dissection. The computed tomography (CT) scan demonstrated obstructive hydrocephalus with several intra-axial lesions located in the pineal region, left cerebellar hemisphere, and left frontal operculum. A right frontal ventriculostomy was initially placed to relieve the hydrocephalus. CSF was sent for cytology but was unrevealing. Due to the degree of brainstem compression and the need to obtain a pathologic diagnosis, a posterior fossa craniotomy for the removal of the lesion was performed. Histopathology demonstrated small blue cell tumors positive for neuroendocrine markers consistent with neuroendocrine carcinoma of the cervix. Resection of additional metastasis was not recommended. An endoscopic third ventriculostomy (ETV) was then performed in order to remove the ventriculostomy with success. The patient was then referred to radiation oncology and received whole-brain radiotherapy (WBRT) for a total of 30 Grays (3000 cGy) over 10 fractions. Interval imaging demonstrated complete resolution of the pineal and left frontal lesions. The patient was symptom-free for approximately three months. She then presented with paraplegia consistent with follow-up imaging of her neuraxis, demonstrating drop metastasis in her cervical, thoracic, and lumbar spine. Spinal radiation was given with partial recovery in upper extremity function, however, lower extremity function did not recover. The patient was then transferred to palliative care. There are no guidelines on NECC brain metastasis management. Brain metastasis is associated with reduced longevity. NECC has a propensity for early dissemination and treatment failure. ETV is preferred over ventriculoperitoneal shunting in cases with obstructive hydrocephalus, as it may reduce the risk of tumor seeding. Retrospectively, our patient may have benefitted from upfront craniospinal radiation.

Underrepresented in Surgery: (Lack of) Diversity in Academic Surgery Faculty.

BACKGROUND: A call to increase diversity among academic surgery faculty (ASF) was made in 2008, after recognizing the lack of surgeons considered underrepresented in medicine (URM). We aimed to quantify and assess trends among URM ASF in the interval since that call to action. METHODS: Publicly available data on ASF were reviewed. We calculated the percentage of ASF in 2018 by URM group, then stratified by academic rank of assistant professor, associate professor, and full professor. We compared 2005-2018 ASF of Hispanic or Latino (HL) and African American (AA) background; 2005 data were unavailable for other URM groups. RESULTS: In 2018, URM surgeons accounted for 7.06% (n = 1013/14,340) of ASF (AA: n = 492, 3.43%; HL: n = 485, 3.38%; American Indian or Alaskan Native: n = 23, 0.16%; and Native Hawaiian/Pacific Islander: n = 13, 0.09%). When comparing 2005-2018, AA ASF remained stable across ranks (total: n = 298, 3.12% versus n = 492, 3.43%; P = 0.09), whereas HL ASF decreased across ranks (total: n = 415, 4.35% versus n = 485, 3.38%; P = 0.00007). CONCLUSIONS: Surgeons from URM backgrounds account for 7% of ASF. No increase in AA and a decrease in HL ASF occurred from 2005 to 2018. There is a paucity of data for other URM groups. Active strategies to increase diversity and inclusion in academic surgery are necessary.

Comorbid depressive symptoms and self-esteem improve after either cognitive-behavioural therapy or family-based treatment for adolescent bulimia nervosa.

This study examined the effect of family-based treatment for bulimia nervosa (FBT-BN) and cognitive behavioral therapy for adolescents (CBT-A) on depressive symptoms and self-esteem in adolescents with BN. Data were collected from 110 adolescents, ages 12-18, who met Diagnostic and Statistical Manual of Mental Disorders-Fourth Edition, text revision criteria for BN or partial BN. Participants were randomly assigned to FBT-BN or CBT-A and completed measures of depressive symptoms and self-esteem before and after treatment and at 6- and 12-month follow-up assessments. Depressive symptoms and self-esteem significantly improved in both treatments, and neither treatment appeared superior on these clinical outcomes. Parents often worry whether FBT-BN addresses comorbid depressive symptoms and low self-esteem. Our findings address this concern, as they demonstrate that FBT-BN does not differ from CBT-A in improving depressive symptoms and self-esteem, and both treatments result in symptom improvement. These findings can help clinicians guide families to choose a treatment that addresses BN and depressive symptoms and low self-esteem.