Ptosis and macroglossia in a woman with systemic light-chain amyloidosis.

BACKGROUND: Amyloidosis is a rare and variable disease, characterized by extracellular deposits of amyloid protein in different tissues and organs. Patients may present with a range of symptoms, depending on the extent of involvement. Rapid, accurate diagnosis is still challenging in clinical practice. CASE REPORT: A 72-y-old woman presented with a 1-y history of droopy upper left eyelid, resulting in decreased visual acuity, and progressive tongue swelling, resulting in dysarthria, dysphagia, and sleep apnea. Physical examination revealed puffy eyes, moderate swelling up to 1 cm of the upper left eyelid, swollen submental region, and protrusion of the tongue, causing an inability to close the mouth. An abnormal serum free light chain ratio implied the presence of monoclonal gammopathies, and Congo red staining revealed amyloid deposits in specimens from both the tongue and left eyelid. Therefore, a diagnosis of systemic light-chain (AL) amyloidosis was confirmed. The patient then received oral melphalan therapy and surgical intervention for macroglossia. Clinical symptoms including dysarthria, dysphagia, and sleep apnea were under control at 6-month follow-up. CONCLUSIONS: We report an uncommon case presenting initially with both ptosis and macroglossia, for which a final diagnosis of systemic AL amyloidosis was made. Detailed history and laboratory investigation must be implemented on suspicion of amyloidosis, because early recognition of amyloid-associated diseases and appropriate treatment can improve clinical outcomes.

Grommets for otitis media with effusion in children with cleft palate: a systematic review.

BACKGROUND AND OBJECTIVE: No consensus has yet been reached with regard to the link between otitis media with effusion (OME), hearing loss, and language development in children with cleft palate. The objective of this study was to address the effectiveness of ventilation tube insertion (VTI) for OME in children with cleft palate. METHODS: A dual review process was used to assess eligible studies drawn from PubMed, Medline via Ovid, Cumulative Index to Nursing and Allied Health Literature, Cochrane Library, and reference lists between 1948 and November 2013. Potentially relevant papers were selected according to the full text of the articles. Relevant data were extracted onto a data extraction sheet. RESULTS: Nine high- or moderate-quality cohort studies were included in this study. VTI was administered in 38% to 53% of the OME cases, and more severe cases appeared more likely to undergo VTI. Compared with conservative forms of management (eg, watchful waiting), VTI has been shown to be beneficial to the recovery of hearing in children with cleft palate and OME. A growing body of evidence demonstrates the benefits of VTI in the development of speech and language in children with cleft palate and OME. These children face a higher risk of complications than those undergoing conservative treatments, the most common of which are eardrum retraction and tympanosclerosis, with an incidence of ∼ 11% to 37%. CONCLUSIONS: This review provides evidence-based information related to the selection of treatment for OME in children with cleft palate. Additional randomized controlled trials are required to obtain bias-resistant evidence capable of reliably guiding treatment decisions. The conclusions in this review are based on underpowered cohort studies and very-low-strength evidence.

Influences of patient age on deep neck infection: clinical etiology and treatment outcome.

OBJECTIVES: To analyze the differences in clinical features, etiology, treatment modalities, and microbiology of the deep neck infections between the elderly and the adults. STUDY DESIGN: Cases series. SETTING: Single hospital. SUBJECTS AND METHODS: A retrospective review was performed on patients older than 18 years of age with diagnosis of deep neck infection from January 2008 to December 2012. A total of 148 patients were enrolled in this study, including 32 elderly (older than 65 years of age) and 116 adults who aged between 18 and 65. Data collected included age, gender, clinical presentations, etiology, treatment modalities, and microbiology. RESULTS: Odontogenic and salivary origin were the most common sources of infection for the elderly group, whereas the odontogenic and tonsillar origin were the most common sources of deep neck infections in the adult group. Compared to the adult group, the elderly group had significantly higher ratio with multiple spaces involved (53.1% vs 30.2%, P = .016), complication (15.6% vs 4.3%, P = .024), and surgical interventions (75.0% vs 38.8%, P < .001), in addition to longer hospital stay (11.1 ± 7.2 days vs 8.2 ± 4.5 days, P = .029). CONCLUSION: Compared to the adult group, the elderly patients with deep neck infection had more cases with multiple spaces involvement, complications, surgical interventions, and longer hospital stay. However, the outcome of the elderly group was the same as the adult group. Therefore, the benefits of aggressive management for deep neck infection should not be withheld from patients simply because of the old age.