Atypical presentations of congenital hemangiomas: Extending the clinical phenotype.

BACKGROUND/OBJECTIVES: Congenital hemangiomas (CH) are a group of benign vascular tumors that are present at birth and exhibit variable involution during infancy. Congenital hemangiomas that do not involute are typically solitary patch or plaque-type tumors that grow proportionally with somatic growth. We report a case series of 9 patients with persistent CH, which exhibited uncommon features including segmental involvement, recurrent or severe pain, or growth via volumetric increase in size or apparent increased extent of anatomic involvement over time. METHODS: Via retrospective chart review, we included patients with persistent CH and atypical presentations. Available data regarding clinical characteristics, natural history, histopathology, imaging, and genetic tests were collected. RESULTS: Data on 9 patients were collected, including 7 noninvoluting CH and 2 partially involuting CH. Three of the 9 cases had segmental distribution, 6 had apparent growth or clinical evolution, and 4 were symptomatic with pain. One also had marked localized intravascular coagulopathy. CONCLUSIONS: Ongoing or recurrent pain and large extent of anatomic involvement can be features of CH, albeit uncommon ones, and can pose both diagnostic and management challenges. Tissue genomic studies can offer a novel tool for CH diagnosis.

Remission of subcutaneous panniculitis-like T-cell lymphoma in a pregnant woman after treatment with oral corticosteroids as monotherapy.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of cutaneous T-cell lymphoma characterized by neoplastic α/ß T cells infiltrating subcutaneous tissues in a lobular pattern. Few data support the optimal treatment regimen for patients, given the rarity of this condition, and even fewer data describe treatment when diagnosed during pregnancy. We describe a case of SPTCL in a pregnant patient who achieved clinical remission after treatment with corticosteroid monotherapy. Our case suggests that corticosteroids should be considered as first-line treatment in pregnant patients with SPTCL.

Adalimumab-Induced Cutaneous Lupus Erythematosus in a 16-Year-Old Girl with Juvenile Idiopathic Arthritis.

Tumor necrosis factor α (TNF-α) antagonists are used in the treatment of numerous autoimmune conditions. Adalimumab is the first monoclonal antibody to TNF-α and is used to treat juvenile idiopathic arthritis. A growing body of literature associates anti-TNF-α therapies with several adverse dermatologic manifestations, including drug-induced lupus erythematosus (LE). We describe a case of cutaneous LE in a 16-year-old girl treated with adalimumab for juvenile idiopathic arthritis. The temporal association between her presenting symptoms and adalimumab initiation and gradual improvement after stopping biologic therapy suggest adalimumab-induced cutaneous LE. With increasing use of anti-TNF therapies in children, the potential for drug-induced LE should not be overlooked.

Vemurafenib-induced neutrophilic panniculitis in a child with a brainstem glioma.

Neutrophilic panniculitis is a rare adverse effect of therapy with selective BRAF inhibitors. We report a case of neutrophilic panniculitis in a 15-year-old girl receiving treatment with vemurafenib for a brainstem glioma. Clinicians should be aware of this rare but important side effect of vemurafenib. This is the first report of neutrophilic panniculitis in a child treated with vemurafenib.

Olanzapine causes a leptin-dependent increase in acetylcholine release in mouse prefrontal cortex.

STUDY OBJECTIVES: The atypical antipsychotic olanzapine is used effectively for treating symptoms of schizophrenia and bipolar disorder. Unwanted effects of olanzapine include slowing of the electroencephalogram (EEG) during wakefulness and increased circulating levels of leptin. The mechanisms underlying the desired and undesired effects of olanzapine are poorly understood. Sleep and wakefulness are modulated by acetylcholine (ACh) in the prefrontal cortex, and leptin alters cholinergic transmission. This study tested the hypothesis that olanzapine interacts with leptin to regulate ACh release in the prefrontal cortex. DESIGN: Within/between subjects. SETTING: University of Michigan. PATIENTS OR PARTICIPANTS: Adult male C57BL/6J (B6) mice (n = 33) and B6.V-Lep(ob) (leptin-deficient) mice (n = 31). INTERVENTIONS: Olanzapine was delivered to the prefrontal cortex by microdialysis. Leptin-replacement in leptin-deficient mice was achieved using subcutaneous micro-osmotic pumps. MEASUREMENTS AND RESULTS: Olanzapine caused a concentration-dependent increase in ACh release in B6 and leptin-deficient mice. Olanzapine was 230-fold more potent in leptin-deficient than in B6 mice for increasing ACh release, yet olanzapine caused a 51% greater ACh increase in B6 than in leptin-deficient mice. Olanzapine had no effect on recovery time from general anesthesia. Olanzapine increased EEG power in the delta (0.5-4 Hz) range. Thus, olanzapine dissociated the normal coupling between increased cortical ACh release, increased behavioral arousal, and EEG activation. Leptin replacement significantly enhanced (75%) the olanzapine-induced increase in ACh release. CONCLUSION: Replacing leptin by systemic administration restored the olanzapine-induced enhancement of ACh release in the prefrontal cortex of leptin-deficient mouse.

Single-dose azithromycin prevents trichiasis recurrence following surgery: randomized trial in Ethiopia.

BACKGROUND: Trichiasis recurrence following surgery is a serious problem for trachoma programs. OBJECTIVE: To determine if postoperative treatment with azithromycin compared with topical tetracycline reduces recurrence up to 1 year, and if azithromycin treatment of household members provides additional benefit compared with treating only the surgical patient. DESIGN: A randomized, single-masked, clinical trial was conducted in Ethiopia. A total of 1452 patients with trichiasis were randomized 1:1:1 to the following 3 arms: single-dose (1 g) oral azithromycin alone, single-dose azithromycin for household members (20 mg/kg up to 1 g) plus the patient, or topical tetracycline (twice per day for 6 weeks). MAIN OUTCOME MEASURES: Trichiasis recurrence within 1 year following surgery. RESULTS: The combined azithromycin groups had significantly fewer recurrences, 6.9 of 100 person-years overall, compared with topical tetracycline, 10.3 of 100 person-years (P = .047). There was no additional reduction in the arm that also treated household members, 8.1 of 100 person-years, compared with treating the surgical patients alone, 5.8 of 100 person-years (P = .19). CONCLUSIONS: In trachoma-endemic areas, a single dose of azithromycin reduced postoperative trichiasis recurrence rates by one third compared with topical tetracycline. Application to Clinical Practice In countries where azithromycin is part of the Trachoma Control Program, patients with trachomatous trichiasis should be treated postoperatively to prevent recurrence.

Surgery for Trichiasis, Antibiotics to prevent Recurrence (STAR) Clinical Trial methodology.

Trachoma is the leading infectious cause of blindness worldwide. Surgery is available to correct trichiasis, which results from repeated episodes of infection with C. trachomatis. However, trichiasis recurrence rates post-surgery are very high. Methods for reducing post-surgical trichiasis recurrence need to be explored. This paper outlines the design of the Surgery for Trichiasis, Antibiotics to prevent Recurrence (STAR) Clinical Trial currently underway in Ethiopia. The STAR trial, funded by The National Eye Institute, is a randomized, controlled clinical trial of antibiotic use at time of trichiasis surgery, comparing topical tetracycline to single-dose azithromycin for the surgical patient and single-dose azithromycin for the surgical patient and all household members. The primary outcome is trichiasis recurrence at one-year. Data from this trial will be critical in helping to determine future policy on antibiotic treatment for C. trachomatis following surgery.

The incidence of endophthalmitis after cataract surgery among the U.S. Medicare population increased between 1994 and 2001.

OBJECTIVE: To estimate the annual incidence rate of presumed endophthalmitis after cataract surgery, evaluate any changes in this rate over time, and examine demographic risk factors for endophthalmitis after cataract surgery. DESIGN: Population-based review of Medicare beneficiary claims data. DATA SOURCE: Medicare 5% sample beneficiary data files for inpatient and outpatient claims from 1994 through 2001 were examined to identify all cataract surgeries and subsequent cases of presumed endophthalmitis after cataract surgery. METHODS: All cataract surgery and presumed endophthalmitis cases after cataract surgery were identified based on claims submitted. The annual rate of presumed endophthalmitis after cataract surgery was calculated, and demographic risk factors for endophthalmitis were examined using multivariate models. MAIN OUTCOME MEASURES: Incidence rate of endophthalmitis after cataract surgery and prevalence of demographic risk factors for endophthalmitis over an 8-year period. RESULTS: One thousand twenty-six cases of presumed endophthalmitis occurred after 477,627 cataract surgeries, yielding an incidence rate of 2.15 per 1000 for this 8-year period. Rates of endophthalmitis adjusted for age, gender, and race were significantly higher in 1998 to 2001 than in earlier years (relative risk [RR], 1.41; 95% confidence interval [CI], 1.24-1.60). Older age and black race also were associated with increased risk of endophthalmitis (RR, 1.83; 95% CI, 1.19-2.81; age, > or =90 years, and RR, 1.30; 95% CI, 1.02-1.65, respectively). CONCLUSIONS: Analysis of Medicare claims data suggests that the incidence of endophthalmitis after cataract surgery has been increasing, but does not provide an explanation for this occurrence. An increase in the incidence of endophthalmitis after cataract surgery is of concern, because cataract surgery is the most commonly performed operation in the United States, and the number of cataract surgeries performed annually will likely increase substantially over the coming decades due to the aging of the U.S. population.

Pattern of recurrence of trachomatous trichiasis after surgery surgical technique as an explanation.

OBJECTIVE: To evaluate the pattern of recurrence of trachomatous trichiasis (TT) after bilamellar tarsal rotation surgery for trachoma. DESIGN: Observational cohort. PARTICIPANTS: Three hundred eighty-four participants having at least 1 eye that had undergone a single TT surgery a minimum of 18 months before June 2001. The 630 study eyes were divided equally between left (311) and right (319) eyes. METHODS: Patients living in Central Tanzania were identified from surgical lists. Participants were screened for recurrence of TT, including evidence of epilation, after surgery. MAIN OUTCOME MEASURES: Detailed information on the location of recurrence was collected, including number of lashes touching the globe and location of trichiatic lashes (nasal, central, or temporal). RESULTS: One hundred seventy-six eyes had evidence of TT recurrence (28%), including 23 eyes having recently undergone epilation. In eyes without epilation, left eyes had a higher rate of recurrence than right eyes (32% vs. 25%; P = 0.05). Among eyes with recurrence originating from 1 location, recurrence was highest centrally (40%). Right eyes had nasal recurrence more often than temporal recurrence (33% vs. 20%). Left eyes had temporal recurrence more often than nasal recurrence (41% vs. 24%). CONCLUSIONS: Recurrence of TT after surgery is more common in the left eye and on the left side of the eyelid. The surgical procedure is more difficult to perform on the right side of the eyelid by a right-handed surgeon. This difficulty may lead to an unintentional change in surgical technique on the right, which may result in lower recurrence on that side.

Risk factors for postsurgical trichiasis recurrence in a trachoma-endemic area.

PURPOSE: The World Health Organization recommends trichiasis surgery to prevent blindness caused by trachoma; however, recurrence is common. Risk factors for recurrence have not been widely studied, particularly in trachoma hyperendemic areas. METHODS: Three hundred ninety-four persons with trichiasis in Tanzania were examined. Participants had undergone surgery >18 months before the study. Trichiasis recurrence and active trachoma at study visit were assessed. Ocular swabs were collected and tested for Chlamydia trachomatis. Household members were examined for active trachoma. RESULTS: Of the surgical eyes, 28% had recurrence; 40% of patients had recurrence in one or both eyes. Rates did not vary by time since surgery. Eye-level recurrence rates varied significantly across districts, ranging from 16% to 38%. Current chlamydial infection among surgical cases was low (6%) and was not associated with recurrence. Recurrence was associated with tarsal conjunctival inflammation (OR: 2.4; 95% confidence interval [CI]: 1.6-3.8) and residence in the Kongwa district (OR: 2.3; 95% CI: 1.2-4.6). CONCLUSIONS: Recurrence after trichiasis surgery is high, suggesting that vigilant follow-up in surgical cases is needed to reduce blindness. Recurrence is associated with evidence of inflammation in the tarsal conjunctiva, although it is not clear whether the inflammation contributes to recurrence, or is a result of the recurrence. Longitudinal studies of trichiasis patients after surgery are needed.

Mass treatment and the effect on the load of Chlamydia trachomatis infection in a trachoma-hyperendemic community.

PURPOSE: Trachoma remains a leading cause of blindness. Determining the most effective antibiotic treatment strategy is essential for the success of country-based trachoma control programs. METHODS: Baseline and 2-month follow-up examinations were performed in a trachoma-hyperendemic village. All residents were offered azithromycin for trachoma after baseline was determined. Infection with Chlamydia trachomatis and chlamydial load were determined by PCR. Clinical trachoma status was evaluated. A high chlamydial load was defined as a higher than median chlamydial load among those with infection. Risk factors were examined in multiple logistic regression models. Associations are presented as odds ratios and 95% confidence intervals. RESULTS: At baseline, 57% of participants were infected with C. trachomatis. Although clinical trachoma correlated with infection, 23% of participants with high chlamydial loads showed no clinical signs. Adults represented only 10% of the population with high loads. Treatment significantly decreased the proportion positive in the community and the load in the community. However, 27% of individuals with high loads at baseline who received treatment also were infected at 2 months. Of those, 93% with high loads at 2 months were aged < or =10 years. CONCLUSIONS: Although most of the chlamydial load in this community resided in children, 10% of the high load resided in adults, most of whom did not have follicular trachoma and in whom the infection would be missed under treatment strategies that focus on clinical disease or children. These data support a mass treatment strategy for hyperendemic communities, at least as a first approach. In addition, treatment of children age < or =2 years should be reexamined, as >30% with high loads at baseline remained infected at 2 months, despite monitored treatment according to weight.