Evaluation of Cheek Edema in an Infant Reveals Langerhans Cell Histiocytosis.

BACKGROUND: Langerhans cell histiocytosis is a rare hematological disorder. Skin rash is the typical early feature, and bony involvement is the second most common presentation. METHODS: We present a case of a 5-month-old female infant with left hemifacial swelling, initially treated for infection with antibiotics. However, due to persistence of swelling and new onset fever, further evaluation with ultrasonography, CT scan, FDG PET/CT and eventually biopsy was performed. RESULTS: Imaging methods revealed mandibular osteolysis indicative of either osteomyelitis or histiocytosis X. Tissue biopsy was diagnostic for Langerhans cell histiocytosis. CONCLUSION: Langerhans cell histiocytosis may present in infancy with a variety of symptoms, included an isolated bony lesion. Langerhans cell histiocytosis, despite its rarity, should be included in the differentiated diagnosis, when bone osteolysis is found.

Petrous bone cholesteatoma removal with hearing preservation.

OBJECTIVE: Clinical and radiologic outcome analysis after petrous bone cholesteatoma (PBC) removal with simultaneous functional reconstruction. STUDY DESIGN: Retrospective case review over an observation period of 16 years (1992-2007). SETTING: Tertiary referral center. PATIENTS: A total of 21 patients (17 adults and 4 children) with PBCs were included, accounting for 3.4% of all cholesteatoma cases (n = 621). The average follow-up duration was 7 years (range, 2-15 yr). In all patients, a modified subtotal petrosectomy was performed with simultaneous tympanoplasty, musculoplasty, and meatoplasty in 90%. In 2 patients (10%), the cavity was obliterated. Two others (10%) needed a simultaneous temporal craniotomy. MAIN OUTCOME MEASURES: Preoperative, intraoperative, and postoperative findings including signs, classifications, recurrences, hearing, and facial nerve outcomes. RESULTS: In the majority of patients (67%), a supralabyrinthine PBC was found. Complete macroscopic resection was achieved in all patients. During the follow-up period, 4 patients (19%) were identified as having recurrent disease. Hearing was postoperatively preserved in 14 of 17 patients with preoperative hearing (82%; 4 preoperative dead ears), of whom, 13 had functional hearing, with a mean pure-tone average of 37 dB (range, 15-55 dB). Preoperative facial nerve dysfunction completely resolved in 4 of 6 patients, and no new dysfunctions occurred postoperatively. All cavities became self-cleaning and dry. CONCLUSION: Hearing preservation was feasible in the majority of patients with PBCs. The functional surgical concept and the high number of supralabyrinthine lesions seemed to be important prerequisites for the good functional outcomes observed.

Cholesterol granuloma of the petrous apex: benefit of computer-aided surgery.

The following is an analysis of the role of computer aided surgery by infralabyrinthine-subcochlear approach to the petrous apex for cholesterol granulomas with hearing preservation. In a retrospective case review from 1996 to 2008 six patients were analysed in our tertiary referral centre, otorhinolaryngology outpatient clinic. Excellent intraoperative localisation of the carotid artery, facial nerve and the entrance into the cholesterol cyst of the bone by means of the navigation system was seen. Additionally, the operation time decreased from an initial 4 h down to 2 h. The application of computer-aided surgery allows intraoperative monitoring of the position of the tip of the microsurgical instruments in case of a rare disease and in the delicate area of the petrous apex giving a high security level.