To analyze the cosmetic and functional results of a large series of patients with unilateral congenital ptosis without spontaneous compensatory ipsilateral frontalis hyperaction who underwent supramaximal levator resection (SMLPSr). A multicenter retrospective review of 35 children (14 girls and 21 boys) of mean age 5.5±3.6 SD years with unilateral congenital ptosis who underwent surgery in 3 different countries: Italy, n=8; Argentina, n=11; and Brazil, n=16. Preoperative evaluation included measurements of upper eyelid margin reflex distance (MRD1) and levator palpebrae superioris muscle excursion, assessment of frontalis hyperaction, and ocular motility examination. At least 6 months postoperatively, photographs were used to measure the upper eyelid contour of OU. The spontaneous blinking amplitude and downward eyelid saccades of OU were quantified in a subset of 14 patients. Quantitative comparison among the 3 centers was performed with nonparametric 1-way analysis of variance (Kruskal-Wallis). Paired t tests were used to compare the pre- and postoperative measurements, and p value<0.05 was statistically significant. The mean preoperative MRD1 of the operated eyes increased from 0.5±1.1 SD mm to 3.4±0.84 SD mm (t=15.9; p<0.000001), consequently the eyelid positional asymmetry decreased from 3.1±1.21 mm to 0.1±0.86 SD mm (t=16.5; p≤0.000001). Twenty-nine percent of eyelids had mild contour abnormalities and 31.4% showed some degree of lash ptosis. Spontaneous blinks were abnormal in 93% of the cases (eyelids). The amplitude of the abnormal blinks ranged from 12.9% to 65.4% (mean=37.1%) of the contralateral eyelids. Downward eyelid saccades were reduced in 79% of the eyelids. The amplitudes the saccades ranged from 2.2% to 84.6% (mean=54.8%). In unilateral congenital ptosis, SMLPSr effectively reduces the positional asymmetry between eyelids. Mild contour abnormalities and lash ptosis are the main complications of the surgery. Postoperatively, spontaneous blinks and downward saccades were reduced in most eyelids. The reduced postoperative eyelid kinetics indicates that only patients with normal upward Bell signs are good candidates for this procedure.
Background: Sjögren Syndrome (SS) is a systemic autoimmune disease with a wide spectrum of manifestations that can lead to misdiagnosis. This study describes and compares demographic, clinical, serological, and histopathological data from subjects with SS and non-Sjögren Syndrome (NSS). It also details specific features within the primary SS (pSS) and secondary SS (sSS) groups identifying sub-groups.Methods: The sample included individuals referred to an academic medical center in Brazil for investigation of SS from 2012 to 2020. Patients were retrospectively classified as primary SS (pSS), secondary SS (sSS), or NSS, based on the American-European Consensus Group criteria (AECG-2002), after multi-professional clinical and laboratory evaluation.Results: A total of 676 individuals were screened and 510 (75.4%) completed the assessments; 198 patients were classified as pSS, 149 as sSS, and 163 as NSS. Symptoms and glandular dysfunction tests were similar in the groups. Concerning pSS, extraglandular manifestations were present in 59% of patients; the elderly had more dry symptoms and peripheral neurological disorders; and 2.5% developed non-Hodgkin lymphoma. In sSS, each overlap promoted distinct clinical and laboratory variants. Several alternative diagnoses were identified as a cause of sicca complex in NSS group.Conclusions: The diagnosis of SS remains a challenge behind dryness. Up to 31% of the suspected cases had other conditions associated to the symptoms. Histopathological analysis of LSG and SSa determined the diagnostic. Aging in pSS and overlap disease in sSS were responsible for distinct phenotypes and characteristic sub-groups in SS.