Langerhans cell histiocytosis: an uncommon disease commonly manifesting in the craniofacial skeleton.

Langerhans cell histiocytosis is an infrequent and enigmatic proliferative disorder that commonly presents in the head and neck region. This is an analysis of 77 patients with Langerhans cell histiocytosis treated at Children's Hospital and Dana Farber Cancer Institute from 1974 through 1993. The study focused on clinical findings, anatomic location and extent of disease, therapy, and outcome. The patients were, on average, under 5 years of age at initial presentation. Over 62 percent of the patients had signs and symptoms referred to the craniofacial skeleton. Osteolytic lesions of the cranium were the most common, followed, in frequency, by scalp rash, osteolytic mandibular tumor(s), enlarged nodes, and gingival swelling or ulceration. Single bony lesions usually were treated with curettage or radiotherapy. Chemotherapy was used commonly for advanced disease with multifocal or disseminated presentation. Initial therapy included moderate doses of single agents; other agents were added if no response was achieved. The natural history of Langerhans cell histiocytosis varied from an acute fulminant course, a waxing and waning chronic disease, to spontaneous regression. Young age at presentation and organ dysfunction predicted a poor prognosis. Statistical analysis showed that there was no significant relationship between outcome and extent of skeletal involvement when controlling for age or organ dysfunction.

Dramatic dilatation of the upper airway secondary to a Valsalva manoeuvre in a lateral cephalometric teleradiograph of a child.

Radiographs are routinely used by orthodontists for the planning of treatment for their patients and they can, in some cases, play a decisive role in the early diagnosis of some unexpected medical or surgical disorders. This report presents the case of a substantial dilatation of the upper airway in a 10-year-old girl, which was attributed to a forced Valsalva manoeuvre. The diagnosis was confirmed upon repetition of the teleradiograph with the mouth open.

Preparing a hemimandibulectomy patient for delayed reconstructive surgery.

A rehabilitation device was designed and used before a reconstructive orthognathic procedure in an adult patient with a severe facial asymmetry. The asymmetry developed as a result of a left hemimandibulectomy in childhood. With this prototype device, the remaining hemimandible was gradually brought into its natural position and dynamics in order to accommodate a graft. The goals were to improve esthetics and function, reduce the chance of the remaining hemimandible not adapting to the graft, and reduce the risk of secondary TMJ pathosis after the mandibular reconstruction.