Nonosseous Periocular Manifestations of Langerhans Cell Histiocytosis: A Case Report and Systematic Review.

PURPOSE: Langerhans cell histiocytosis (LCH) is a disorder of dendritic cell proliferation that typically involves bone. It can be diagnostically challenging when LCH presents without bony involvement, leading to delays in diagnosis and treatment. In this study, the periocular manifestations of LCH in cases where the underlying orbital bones are not involved are described through a systematic review. METHODS: A systematic review of the literature was performed to capture all cases of LCH that involved the periocular region but not the underlying orbital bones. These included LCH cases that involved the periocular skin, the ocular surface, and the orbital tissue. The authors also highlight an additional case where LCH presented with periocular edema and multifocal, nodular conjunctival lesions. RESULT: This review illustrates that LCH rarely presents with periocular infiltration without orbital bone involvement. In these atypical cases, LCH can present as an eyelid mass, a chalazion-like lesion, generalized periocular swelling, ocular surface lesions, or infiltration of any orbital structure. Ocular surface LCH has a higher rate of recurrence than other periocular LCH. Orbital LCH can involve any tissue including extraocular muscles, the lacrimal gland, or indistinct areas within the orbit. CONCLUSIONS: LCH is a clinicopathologic diagnosis. Although most cases involve the bone, any soft tissue can be involved. Biopsy is required to confirm the diagnosis of this heterogeneous disease.

The relationship between transorbital ultrasound measurement of the optic nerve sheath diameter (ONSD) and invasively measured ICP in children. : Part II: age-related ONSD cut-off values and patency of the anterior fontanelle.

PURPOSE: To analyse the diagnostic accuracy of age-related optic nerve sheath diameter (ONSD) cut-off values in children for detecting raised intracranial pressure (ICP) and to assess the benefit of using patency of the anterior fontanelle in describing a different set of cut-off values. METHODS: The ONSD measurement was performed prior to invasive ICP measurement in children under general anesthesia. The diagnostic accuracy of the ONSD measurement was compared to ICP at thresholds of 20, 15, 10, and 5 mmHg. This was further analysed in children above and below the age of 1 year, with a subgroup analysis of age at 4 years, and assessment of the anterior fontanelle (AF) as a reliable physiological marker in part II of this study. RESULTS: Data from 174 children were analysed. In children ≤1 year old, the ONSD measurement with the best diagnostic accuracy for detecting ICP ≥ 20 mmHg was 5.16 mm, compared to 5.75 mm in children >1 year old (p < 0.001). In addition, patency of the anterior fontanelle (AF) was found to be a useful clinical marker for defining different ONSD cut-off values at ICP thresholds of 20, 15, 10 and 5 mmHg. CONCLUSION: Transorbital ultrasound measurement of the ONSD is a reliable non-invasive marker of ICP particularly at higher thresholds of 20 and 15 mmHg. Patency of the AF is a useful clinical marker for defining different ONSD cut-off values in children.

Ophthalmic complications of otitis media in children.

PURPOSE: To evaluate the outcome of ophthalmic complications in children with otitis media. METHODS: The records of children with ophthalmic complications arising from otitis media who presented to the British Columbia Children's Hospital between August 2006 and March 2008 were reviewed retrospectively. RESULTS: Of 1,400 patients presenting to the emergency department for otitis media during the study period, 7 with ophthalmic complications were identified (age range, 1-11 years). All patients had abducens nerve palsy on presentation. Other notable ophthalmic complications included papilledema, Horner syndrome, and proptosis. Extracranial and intracranial complications included mastoiditis, petrositis, parapharyngeal abscess, hydrocephalus, epidural abscess, and cerebral venous thrombosis, including cavernous sinus thrombosis in 2. Of the 7 patients, 6 were treated with surgery, including myringotomy and tube placement (6 patients) and mastoidectomy (3 patients). All patients were initially anticoagulated and received intravenous antibiotics. Satisfactory final visual outcomes and stereopsis ranging from 40 to 100 seconds were achieved in all patients. CONCLUSIONS: Ophthalmic complications of otitis media in children are likely to include abducens palsy. All patients in our series required anticoagulation and intravenous antibiotics. Most required otolaryngologic surgery, but none required strabismus surgery, and all patients had satisfactory visual and ocular motility outcomes.