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.

A Retrospective Analysis of Multiple Affected Salivary Gland Diseases: Diagnostic and Therapeutic Benefits of Interventional Sialendoscopy.

OBJECTIVES: Multiple affected salivary gland diseases are rare compared to single gland diseases and represent a major diagnostic challenge. These cases are commonly neglected in the scientific literature, despite the considerable suffering of these patients. The aim of this retrospective study was to report disease characteristics, diagnostic pathways, and therapeutic options, including sialendoscopic treatment of multiple affected salivary gland disorders. METHODS: Patients experiencing multiple affected salivary gland diseases treated between 2013 and 2020 were consecutively identified. Data regarding demographics, clinical presentation, disease characteristics, treatment, complications, and follow-up were analyzed retrospectively. RESULTS: In total, 71 patients were identified with these diseases and included obstructive disease without sialolithiasis (n = 22), inflammation (n = 15), immune disease (n = 19), radioiodine-induced sialadenitis (RAI) (n = 5), sialadenosis (n = 2), and juvenile recurrent parotitis (JRP) (n = 8). Diagnostic and therapeutic sialendoscopy was performed on 113 salivary glands in 42 patients, leading to completely (n = 27, 64.3%) or partially (n = 11, 26.2%) improved symptoms in most cases. In total, 4 patients did not improve after interventional therapy. CONCLUSIONS: Multiple affected salivary gland diseases are rare and diagnostically challenging. Interventional sialendoscopy offers an effective and safe therapeutic option and should be considered in this specific cohort.

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.

Lemierre syndrome in a 5-month-old male infant: Case report and review of the pediatric literature.

OBJECTIVES: To report, to the best of our knowledge, the youngest patient with Lemierre syndrome. DESIGN: Descriptive case report with review of the pediatric literature. SETTING: Pediatric intensive care unit in a tertiary referral hospital. DATA SOURCE: Systemic review of the literature, including PubMed (English-only journals) and major textbooks. PATIENT: We report a 5-month-old boy who presented with fever and a perforated left-sided otitis media. He developed left-sided complicated otitis media with retroauricular fluid collection, mastoiditis, and temporomandibular joint effusion. The clinical picture was complicated by a left internal jugular vein and left lateral sinus thrombosis. Fusobacterium necrophorum grew in the pus culture. INTERVENTIONS: Low molecular weight heparin. MEASUREMENTS AND MAIN RESULTS: No immunodeficiency and no thrombophilia were identified as predisposing conditions for Lemierre syndrome. Surgical drainage, early and adequate antibiotic treatment, and anticoagulation were followed by complete recovery. CONCLUSIONS: This case report illustrates that Lemierre syndrome can occur in infants without underlying risk factors for severe infections or thrombotic complications.