Sinonasal mucosal melanomas: defining profiles for better survival outcomes.

BACKGROUND: Sinonasal mucosal melanoma is an aggressive malignancy with a 5-year survival rate ranging from 20% to 39%. Despite the evolving surgical and radiotherapy techniques, and introduction of immune-checkpoint inhibitor therapy, overall survival rates remain poor. METHODOLOGY: A retrospective cohort study was conducted at the Hospital Clinic de Barcelona and the Hospital de la Santa Creu i Sant Pau between 1984 and 2020; primary outcome measures were 3 and 5-year melanoma-specific survival (MSS). Kaplan-Meier survival analysis and Cox proportional hazards model were performed to identify predictors of survival. RESULTS: Fifty patients were included, the mean age was 70.4, MSS at 3 and 5 years was 51.2%, and 29.5%, respectively. The median follow-up was 39.6 months during which 46% presented locoregional recurrence and 36%, metastasis. The univariate and multivariate analyses found as survival predictors the N category, the treatment received, the surgical margins and the mitotic index. CONCLUSIONS: We found an overall 5-year MSS of 29.5%. Those patients with intention-to-cure (stages III and IVa) treated by surgery that were N0 at diagnosis, with < 10 mitoses per HPF showed a 5-year MSS rate of 74.1%. More studies will be needed to adequately define the patients' profiles that will benefit from a better survival outcome.

Spontaneous cerebrospinal fluid fistula in the clivus.

INTRODUCTION: Spontaneous cerebrospinal fluid (CSF) fistulas are infrequent and only 10 cases in the literature have been located in the clivus. We describe two new cases of CSF fistulas in this site and review the literature. CASE REPORT: The first patient was a 52-year-old woman referred to our centre for intermittent rhinorrhea that had been diagnosed after an episode of meningitis. The second case was a 69-year-old man who was visited for rhinorrhea of one-year duration; he also developed meningitis during the preoperative study. In both cases, the spontaneous CSF fistula was diagnosed by beta-2-transferrin testing, CT scan and MRI. We performed an endonasal endoscopic transsphenoidal approach and used free grafts and vascularized flaps to close the clival defect. Treatment was successful in both cases. DISCUSSION: The physiopathology of spontaneous CSF fistulas remains unknown. Possible explanations given to date in this location are pulsatility of the basilar artery, repeated Valsalva maneuvers and Marfan's disease, the two latter also related to CSF fistulas in other locations. Closure of a CSF leak towards the nasal cavity is mandatory due to potential complications. Our results support the endoscopic transsphenoidal approach using free grafts and/or pediculated flaps as a good alternative to open surgery.

La estandarización en el trasplante endotelial de membrana de Descemet: resultados de las primeras 450 cirugías / Standardization of the Descemet membrane endothelial keratoplasty technique: Outcomes of the first 450 consecutive cases

OBJETIVOS: Analizar los resultados de 450 casos con diferentes patologías endoteliales intervenidos mediante trasplante endotelial con la técnica de recambio de la membrana de Descemet (DMEK, por sus siglas en inglés: Descemet membrane endothelial keratoplasty) y evaluar las consecuencias de la estandarización de esta técnica. MATERIAL Y MÉTODOS: Se compararon 3 subgrupos consecutivos: el subgrupo I (casos 1-125) representaba la extensión de la curva de aprendizaje; el subgrupo II (casos 126-250) la transición a la estandarización de la técnica y el subgrupo III (casos 251-450) la estandarización propiamente dicha. Se registraron los resultados de agudeza visual mejor corregida pre- y postoperatorios, la densidad de células endoteliales, la paquimetría y las complicaciones. RESULTADOS: A los 6 meses de la cirugía, el 79% de los pacientes alcanzaron una agudeza visual mejor corregida ≥ 0,8 y el 43% ≥ 1. La densidad de células endoteliales media preoperatoria fue 2.530 ± 220 células/mm2, y 1.613 ± 495 células/mm2 al sexto mes poscirugía. La paquimetría era 668 ± 92 μm y 526 ± 46 μm pre- y postoperatoria a los 6 meses, respectivamente. No se encontraron diferencias en cuanto a la agudeza visual mejor corregida, la densidad de células endoteliales o la paquimetría entre los subgrupos (p > 0,05). El desprendimiento del injerto se observó en el 17,3% de los ojos. La tasa de desprendimientos disminuyó del 24 al 12%, y el número de reintervenciones, del 9,6 al 3,5%, del subgrupo I al III respectivamente. CONCLUSIONES: Los resultados visuales y la densidad de células endoteliales tras DMEK son independientes de la estandarización de la técnica quirúrgica. Sin embargo, la estandarización de la técnica podría haber contribuido con un descenso en el número de desprendimientos y con un relativamente bajo número de intervenciones secundarias. A la vista de estos resultados, DMEK podría convertirse en el tratamiento de elección para las enfermedades del endotelio corneal

OBJECTIVES: To evaluate the clinical outcome of the first 450 consecutive cases after Descemet membrane endothelial keratoplasty (DMEK), as well as the effect of standardization of the technique. MATERIAL AND METHODS: Comparison between 3 groups: Group I: (cases 1-125), as the extended learning curve; Group II: (cases 126-250), transition to technique standardization; Group III: (cases 251-450), surgery with standardized technique. Best corrected visual acuity, endothelial cell density, pachymetry and intra- and postoperative complications were evaluated before, and 1, 3 and 6 months after DMEK. RESULTS: At 6 months after surgery, 79% of eyes reached a best corrected visual acuity of≥0.8 and 43%≥1.0. Mean preoperative endothelial cell density was 2,530±220 cells/mm2 and 1,613±495 at 6 months after surgery. Mean pachymetry measured 668±92 μm and 526±46 μm pre- and (6 months) postoperatively, respectively. There were no significant differences in best corrected visual acuity, endothelial cell density and pachymetry between the 3 groups (P > .05). Graft detachment presented in 17.3% of the eyes. The detachment rate declined from 24% to 12%, and the rate of secondary surgeries from 9.6% to 3.5%, from group I to III respectively. CONCLUSIONS: Visual outcomes and endothelial cell density after DMEK are independent of the technique standardization. However, technique standardization may have contributed to a lower graft detachment rate and a relatively low number of secondary interventions required. As such, DMEK may become the first choice of treatment in corneal endothelial disease

Standardization of the Descemet membrane endothelial keratoplasty technique: Outcomes of the first 450 consecutive cases.

OBJECTIVES: To evaluate the clinical outcome of the first 450 consecutive cases after Descemet membrane endothelial keratoplasty (DMEK), as well as the effect of standardization of the technique. MATERIAL AND METHODS: Comparison between 3 groups: Group I: (cases 1-125), as the extended learning curve; Group II: (cases 126-250), transition to technique standardization; Group III: (cases 251-450), surgery with standardized technique. Best corrected visual acuity, endothelial cell density, pachymetry and intra- and postoperative complications were evaluated before, and 1, 3 and 6 months after DMEK. RESULTS: At 6 months after surgery, 79% of eyes reached a best corrected visual acuity of≥0.8 and 43%≥1.0. Mean preoperative endothelial cell density was 2,530±220 cells/mm2 and 1,613±495 at 6 months after surgery. Mean pachymetry measured 668±92 µm and 526±46 µm pre- and (6 months) postoperatively, respectively. There were no significant differences in best corrected visual acuity, endothelial cell density and pachymetry between the 3 groups (P > .05). Graft detachment presented in 17.3% of the eyes. The detachment rate declined from 24% to 12%, and the rate of secondary surgeries from 9.6% to 3.5%, from group I to III respectively. CONCLUSIONS: Visual outcomes and endothelial cell density after DMEK are independent of the technique standardization. However, technique standardization may have contributed to a lower graft detachment rate and a relatively low number of secondary interventions required. As such, DMEK may become the first choice of treatment in corneal endothelial disease.

Encefalopatía por hiperamoniemia en el adolescente / Encephalopathy by hyperammonemia in the teenager

Fundamento. La hiperamoniemia origina múltiples alteraciones,principalmente en el sistema nervioso central.Si el fallo hepático no es su causa, deberán investigarseotras etiologías menos frecuentes intentandollegar a un diagnóstico definitivo.Caso clínico. Presentamos el caso de un paciente de16 años que ingresó por encefalopatía aguda e hiperamoniemia.Tras realización de analítica, TAC cerebral,ecografía y Doppler abdominal, se inició tratamientoempírico de hiperamoniemia secundaria a un errorinnato del ciclo de la urea. Se trató el edema cerebraly se tomaron medidas para eliminación del amoniacosin resultado favorable, falleciendo a los cuatro días delingreso.Conclusiones. El complejo manejo de la hiperamoniemiay la alta morbi-mortalidad que conlleva requiere unmanejo multidisciplinar. La instauración de tratamientoprecoz e identificación de la causa son claves para mejorarlos resultados(AU)

Background. Hyperammonemia causes several alterations,mainly in the central nervous system. If hepaticfailure is not its etiology, other less frequent causesmust be investigated in the search for a definitive diagnosis.Clinical case. We report the case of a 16 year old patientadmitted for acute encephalopathy and hyperammonemia.After analysis, brain CT, ultrasound and abdominalDoppler, we began empirical treatment of hyperammoniemiasecondary to disorders of the urea cycle. Wetreated the brain edema and eliminated ammonia butwe did not obtain favourable results and the patientdied four days later.Conclusions. The complex management of hyperammonemiaand the high morbidity and mortality involvedrequire a multidisciplinary approach. Only earlytreatment and identification of the hyperammonemia`setiology can avoid high morbidity and mortality in thesepatients(AU)

Treatment results for ethmoid sinus carcinoma.

OBJECTIVE: The aim of this study was to describe the results of treatment in patients with ethmoid sinus carcinoma. MATERIALS AND METHOD: We performed a retrospective study of 34 patients with carcinoma of the ethmoid sinus, and collected the following data: age, sex, employment, tobacco and alcohol consumption, tumour-node-metastasis stage, treatment, and survival. RESULTS: The mean patient age was 64 years. Seventy-six per cent of patients were men and 24 per cent women. Squamous cell carcinoma was the most frequent histological tumour type (44 per cent). Eleven patients were classified as T(2), six as T(3), six as T(4a) and 11 as T(4b). Two patients (6 per cent) had nodal metastasis at the time of diagnosis. The anterior skull base was involved in 17 patients (50 per cent) and the anterior orbital contents were affected in seven patients (21 per cent). The five-year actuarial observed survival rate for all patients was 44 per cent. CONCLUSIONS: Combined treatment with surgery and post-operative radiotherapy permitted good local control in patients with ethmoid sinus carcinoma. We do not recommend prophylactic neck treatment for ethmoid sinus carcinoma.

[Encephalopathy by hyperammonemia in teenagers]. / Encefalopatía por hiperamoniemia en el adolescente.

BACKGROUND: Hyperammonemia causes several alterations, mainly in the central nervous system. If hepatic failure is not its etiology, other less frequent causes must be investigated in the search for a definitive diagnosis. CLINICAL CASE: We report the case of a 16 year old patient admitted for acute encephalopathy and hyperammonemia. After analysis, brain CT, ultrasound and abdominal Doppler, we began empirical treatment of hyperammonemia secondary to disorders of the urea cycle. We treated the brain edema and eliminated ammonia but we did not obtain favourable results and the patient died four days later. CONCLUSIONS: The complex management of hyperammonemia and the high morbidity and mortality involved require a multidisciplinary approach. Only early treatment and identification of the hyperammonemia's etiology can avoid high morbidity and mortality in these patients.

[Epidemiologic analysis of 72 carcinomas of the nasal cavity and paranasal sinuses]. / Análisis epidemiológico de 72 carcinomas de fosas y senos paranasales.

OBJECTIVE: The aim of this study is to define the epidemiological aspects of carcinoma of the nasal cavity and paranasal sinuses. MATERIAL AND METHODS: We performed a retrospective study of 72 carcinomas of the nasal cavity and paranasal sinuses. Various sites, age and sex distribution, drug consumption, TNM stage grouping and treatment were reported. RESULTS: The average age was 63. Seventy- five percent of patients (54/72) were male and 25% (18/72) female. The site of origin was paranasal sinuses in 46 patients (64%), 30 in ethmoid sinus, 15 in maxillary sinus and 1 in sphenoid sinus. Twenty-six patients (36%) were located in nasal cavity. Squamous cell carcinoma was the most frequent histological type in both localizations. The 5-year adjusted survival rate for all patients was 60% (IC: 54-66), 36% (IC: 28-44) for paranasal sinus carcinoma and 86% (IC: 79-93) for nasal cavity carcinoma. The 5-year adjusted survival rate according to the T distribution in 46 carcinomas paranasal sinus was 80% T2, 71% T3, 19% T4a and 6% T4b.(p = 0.0002). CONCLUSIONS: Carcinoma of nasal cavity and paranasal sinuses represent a group of tumors that differ from the rest of carcinomas of the head and neck.