Neurotrophic Keratitis in a Pediatric Patient With Goldenhar Syndrome and Trigeminal Aplasia Successfully Treated by Corneal Neurotization.

Herein, the authors report an unusual case of a 6-year-old boy with right-sided Goldenhar syndrome and trigeminal nerve aplasia who developed neurotrophic keratopathy (NK). Despite the use of therapeutic contact lenses and multiple temporary tarsorrhaphy, NK worsened showing a central corneal scar, neovascularization, and significant stromal thinning, with risk of corneal perforation. Cochet-Bonnet esthesiometry revealed complete corneal anesthesia. To minimize additional corneal complications, the patient underwent indirect corneal neurotization by a sural nerve autograft anastomosed to the contralateral supratrochlear nerve. At 24-month follow up, no epithelial defects, complications, or recurrence were observed. Significant improvements in corneal sensitivity with esthesiometry score of 20 mm and reflex blinking were achieved. This case highlights corneal anesthesia should be suspected among Goldenhar syndrome ophthalmologic abnormalities and monitored before corneal changes become irreversible. Since corneal neurotization can successfully improve corneal sensation, it could be considered as an early therapeutic option to avoid refractory NK.

Oral florid papillomatosis: Topical treatment with 5% imiquimod in orabase.

BACKGROUND: Florid oral papillomatosis is characterized by its tendency to local recurrence that requires multiple treatments, leading to important functional sequelae. METHODS: We present 74-year-old woman with oral florid papillomatosis (OFP) who refused a new surgical treatment, and was treated with imiquimod 5% in orabase on alternate days for 16 weeks. Treatment was complemented with application of hyaluronic acid gel. RESULTS: There were no side effects to the treatment, nor signs of local recurrence, in the treated area at 2 years of follow-up. CONCLUSIONS: After reviewing the literature and according to our knowledge, this is the first published case of oral florid papillomatosis treated topically with imiquimod 5% successfully. Topical treatment with imiquimod 5% in orabase may be a valid alternative for patients with recurrent OFP located in the anterior area of the oral cavity who refuse surgical treatment, although we must closely monitor the patient for the possibility of recurrence or malignant degeneration.

[Minimal important clinical difference in health-related quality of life.] / Diferencia mínima clínica importante en calidad de vida relacionada con la salud.

Una de las limitaciones para el uso de los Cuestionarios de Calidad de Vida Relacionada con la Salud y de los resultados reportados por pacientes es la dificultad de interpretar los valores obtenidos. La diferencia mínima clínica importante es una medida que ayuda a comprender los resultados de estos cuestionarios y valorar la relevancia clínica del efecto conseguido por la intervención realizada. En este trabajo revisamos el concepto de diferencia mínima clínica importante, describimos los métodos utilizados para su obtención y exponemos sus dificultades, limitaciones y aplicabilidad. Dentro de los resultados reportados por pacientes y, particularmente, en los de calidad de vida, la diferencia mínima clínica importante es una herramienta que ayuda a los clínicos a utilizar correctamente las escalas de medida e interpretar el efecto de las intervenciones. Con este artículo esperamos facilitar la implantación y uso de la diferencia mínima clínica importante y los Cuestionarios de Calidad de Vida Relacionada con la Salud en la práctica habitual con nuestros pacientes.

One of the limitations for the use of Health-related Quality of Life Questionnaires and the results reported by patients is the difficulty of interpreting the values obtained. The minimal important clinical difference is a measure that helps to understand the results of these questionnaires and assess the clinical relevance of the effect achieved by the intervention performed. In this paper, we review the concept of minimal important clinical difference, describe the methods used to obtain it, and expose its difficulties, limitations, and applicability. Within the results reported by patients, and particularly in quality of life, the minimal important clinical difference is a tool that helps clinicians to correctly use the measurement scales and interpret the effect of the interventions. With this article, we hope to facilitate the implementation and use of the minimal important clinical difference and the Health-related Quality of Life Questionnaires in routine practice with our patients.

Synchronous occurrence of IgG4-related sialadenitis and ductal carcinoma of the parotid gland: a case report.

Immunoglobulin G4-related disease (IgG4-RD) is a rare chronic systemic inflammatory pathology that poses a diagnostic challenge since it can simulate malignancy when it affects a salivary gland as a mass-like lesion. Here, the authors report an unusual clinical case of a 42-year-old man who presented with a painless, slow-growing swelling located in the right parotid gland with a 12-month evolution. Based on imaging tests and open biopsy, a diagnosis of chronic parotitis was presumed and oral methylprednisolone was prescribed. Due to poor response to medication, a total parotidectomy preserving the facial nerve was performed. The final pathology described a unilateral IgG4-related sialadenitis (IgG4-RS) in the parotid gland in combination with a poorly differentiated multifocal ductal carcinoma. The postoperative course was uneventful except for a temporary facial paresis (grade III according to the House-Brackmann classification system) that resolved completely within 5 months. There were no systemic manifestations on the whole-body 18F-FDG PET/CT. Adjuvant radiotherapy was administered without complications. Twenty-four months follow-up after surgery showed no recurrence or evidence of systemic involvement. This clinical report highlights the importance of considering the synchronous occurrence of a carcinoma underlying an isolated parotid gland mass in the context of IgG4-RS, especially if there is no response to prior steroid medication.

Quality of life in long-term oral cancer survivors: a comparison with Spanish general population norms.

PURPOSE: Health-related quality-of-life (HRQOL) data are becoming an important supplement to information pertaining to treatment outcomes for cancer patients. The purpose of this study was to evaluate the HRQOL of patients undergoing primary surgery for oral squamous cell carcinoma > or =5 years after treatment compared with the Spanish general population norms. MATERIALS AND METHODS: A total of 50 oral cancer patients (mean age 55.78 years, 80% male) with cancer-free survival of > or =5 years after surgery were enrolled. HRQOL was assessed with a standardized questionnaire: the Medical Outcomes Study Short Form 36-Item Health Survey. RESULTS: The Medical Outcomes Study Short Form 36-Item Health Survey scores of the oral cancer patients did not differ significantly from those of an age- and gender-matched sample from the Spanish normative population, except for the pain and social functioning domains. The patients had significantly better results compared with the population norms (Wilcoxon test, P < .05) in the physical function, general health, and vitality domains. CONCLUSIONS: These results provide patient-reported evidence that oral cancer survivors have a similar HRQOL compared with the general Spanish population. We also believe that it would be necessary to analyze the quality of life > or =5 years after treatment or from the moment the patient was discharged.

Preoperative ultrasonography for evaluation of clinically N0 neck in oral cavity carcinoma.

The aim of this study was to evaluate preoperative ultrasound criteria to detect lymph node (LN) cervical metastasis in patients with clinically node-negative neck (cN0) oral cavity squamous cell carcinoma (OCSCC). A prospective, single-center, observational study was conducted in 90 patients undergoing cancer excision with or without elective neck dissection (END) between 2005 and 2012. A surgeon and an experienced radiologist performed preoperative cervical ultrasonography in all cases. The primary objective was to obtain an a priori sensitivity of 90% and specificity >50% in cN0 OCSCC staging. The sonographic criteria for LN assessment were as follows: number; neck levels; clusters; aspect; heterogeneity; longitudinal diameter (L); transverse diameter (T); L/T ratio; and combination in series or in parallel of T and L/T ratio. The gold standard for comparison was the LN histological identification of metastasis after END or the occurrence in the follow-up at least 36 months. Statistically significant sonographic criteria in univariate analysis (P < 0.05) were as follows: multilevel lymph nodes, T diameter >6.5 mm, and the combination T > 6.5 mm or L/T < 1.3 ratio; and in multivariate logistic regression analysis were (P < 0.05): combination T > 6.5 mm and L/T < 1.3 ratio, LN in level II, and moderately-poorly differentiated OCSCC. By using selected sonographic criteria, ultrasound can be a valid preoperative diagnostic method to optimize staging cervical metastasis and to help decide about neck dissection.

W43X SDHD mutation in sporadic head and neck paraganglioma.

OBJECTIVE: To analyze the presence of SDHD gene mutations in patients with sporadic head and neck paraganglioma. STUDY DESIGN: The presence of somatic and germline SDHD mutations was investigated in 10 patients by polymerase chain reaction and direct sequencing. RESULTS: Two patients displayed mutations: 259C>T (P87S) in 1 case and 129G>A (W43X) in the other. The first was considered a neutral polymorphism. The second was present in the germline of 1 of her sons, who had an apparently unrelated testicular seminoma and loss of heterozygosity (LOH) in the tumor cells. CONCLUSION: This is the first reported case of an SDHD mutation carrier showing LOH in a testicular seminoma.