[Unusual presentation forms of Wegener's disease]. / Formas de presentación infrecuente de la enfermedad de Wegener.

Wegener's granulomatosis may appear with the classic granulomatous necrotizing vasculitis affecting both high and low airway and renal glomerule, and rarely small vessels in any other location. In this way we may find generalized and localized forms of the disease. Three atypical cases of the disease are reported. The first one as a limited form with only airway affection. The second one as an unusual case of otitis with progressive affection of I, II, V, VI, VII and VIII cranial nerves. The third one was a generalized case with sinusitis and orbital pseudotumor. Both first and second cases have been controlled for a very long period of time of 22 and 14 years respectively, and none of them has presented any other location of the disease.

Formas de presentación infrecuente de la enfermedad de Wegener / Unusual presentation forms of Wegener's disease

La enfermedad de Wegener es una vasculitis granulomatosa necrotizante que típicamente afecta al tracto respiratorio superior e inferior y al riñón, y con mayor rareza a los pequeños vasos de otras localizaciones, pudiendo existir formas generalizadas y otras localizadas. Presentamos tres casos atípicos. Los dos primeros como formas limitadas de la enfermedad, uno con afectación sólo de la vía aérea y el otro como forma inusual de otitis con afectación progresiva de los pares craneales I,II, V, VI, VII y VIII. El tercero como forma generalizada con sinusitis y pseudotumor orbitario. Durante el largo seguimiento mantenido de los dos primeros casos, de 22 y 14 años respectivamente, no han presentado ninguna otra localización de esta enfermedad (AU)

Wegener's granulomatosis may appear with the classic granulomatous necrotizing vasculitis affecting both high and low airway and renal glomerule, and rarely small vessels in any other location. In this way we may find generalized and localized forms of the disease. Three atypical cases of the disease are reported. The first one as a limited form with only airway affection. The second one as an unusual case of otitis with progressive affection of I, II, V, VI, VII and VIII cranial nerves. The third one was a generalized case with sinusitis and orbital pseudotumor. Both first and second cases have been controlled for a very long period of time of 22 and 14 years respectively, and none of them has presented any other location of the disease (AU)

[Mortality in advanced stage laryngeal cancer]. / Mortalidad en el cáncer de laringe en estadios avanzados.

Patients with advanced laryngeal carcinoma present a high mortality rate due to locoregional recurrence, distant metastases and second cancer. We present a report about the most important prognostic factors in mortality in patients included in III and IV stages. The most important are the presence of metastatic lymph nodes (p = 0.001), extracapsular spread (p = 0.002) and N stage (p = 0.005).

[Distant metastases in patients with advance laryngeal carcinoma]. / Metástasis a distancia en pacientes con carcinoma de laringe avanzado.

Patients with advanced stage cancer larynx have a higher risk of development of distant metastases which means a bad prognosis. In our study we try to identify risk factors with the idea of involve to all of these patients in more aggressive systemic therapy protocols. Data of 57 patients surgically treated, with squamous pharyngolaryngeal carcinoma, stage III and IV, are evaluated. Advanced T stage or more extensive neck disease more often caused distant metastases (p < 0.05), and also the histological evidence of tumor invasion in three or more cervical nodes (p = 0.02), and poorly differentiated tumors (p < 0.05). Tumor site, surface area in cm, presence of ulceration, and loco-regional uncontrol were not associated with distant metastases.

[Melanoma of the hard palate]. / Melanoma de paladar duro.

Oral melanomas are extremely rare and occur most often on the palate and gingiva. Its prognosis is poor. Surgical management remains the preferred treatment, sometimes in combination with chemotherapy. We report the case of a 72 years old woman with a malignant melanoma of the hard palate who developed three years later a gingival nevi and 5 years later metastatic cervical lymph nodes. Our treatment has been only radical surgery all times.

Mortalidad en el cáncer de laringe en estadios avanzados / Mortality in advanced laryngeal carcinoma

Los pacientes con carcinoma de laringe avanzado presentan una mortalidad elevada, dadas las altas tasas de recidiva locorregional, segundos cánceres y metástasis a distancia. Realizamos un estudio sobre los factores que más influyen en la mortalidad en los estadios III y IV encontrando como más importante la presencia de adenopatías cervicales metastásicas (p=0,001), la extensión extracapsular (p=0,002) y el estadio N (p=0,005) (AU)

Patients with advanced laryngeal carcinoma present a high mortality rate due to locoregional recurrence, distant metastases and second cancer. We present a report about the most important prognostic factors in mortality in patients included in III and IV stages. The most important are the presence of metastatic lymph nodes (p = 0.001), extracapsular spread (p = 0.002) and N stage (p = 0.005) (AU)

Melanoma de paladar duro / Melanome of the palate

Los melanomas de cavidad oral son extremadamente raros y aparecen de preferencia en paladar y encías. Su pronóstico es malo. El tratamiento de elección es quirúrgico, combinado según los casos con quimioterapia. Presentamos el caso de una mujer de 72 años, con un melanoma de paladar duro, que desarrolló 3 años más tarde un nevus de mucosa gingival y a los 5 años una adenopatía cervical metastásica. Nuestra forma de tratamiento ha sido exclusivamente quirúrquico en las tres ocasiones (AU)

Oral melanomas are extremely rare and occur most often on the palate and gingiva. Its prognosis is poor. Surgical management remains the preferred treatment, sometimes in combination with chemotherapy. We report the case of a 72 years old woman with a malignant melanoma of the hard palate who developed three years later a gingival nevi and 5 years later metastatic cervical lymph nodes. Our treatment has been only radical surgery all times (AU)

Metástasis a distancia en pacientes con carcinoma de laringe avanzado / Distant metastases in patients with advance laringeal carcinoma

Los pacientes con carcinoma de laringe en estadios avanzados tienen un alto riesgo de desarrollar metástasis a distancia, lo cual ensombrece considerablemente el pronóstico. En nuestro estudio tratamos de identificar cuáles son los factores de riesgo y planteamos someter a este grupo de pacientes a un tratamiento sistémico más agresivo. Analizamos los datos de 57 pacientes con carcinoma escamoso faringolaríngeo en estadios III y IV, tratados quirúrgicamente. Encontramos que los estadios T avanzados y la afectación cervical ganglionar causan más metástasis (p<0,05), así como la invasión tumoral de 3 o más ganglios (p=0,002), y los tumores pobremente diferenciados (p<0,05). La localización tumoral, su extensión en cm, la presencia de ulceración y la falta de control locoregional no se asociaron con una mayor incidencia de metástasis (AU)

Patients with advanced stage cancer larynx have a higher risk of development of distant metastases which means a bad prognosis. In our study we try to identify risk factors with the idea of involve to all of these patients in more aggressive systemic therapy protocols. Data of 57 patients surgically treated, with squamous pharyngolaryngeal carcinoma, stage III and IV, are evaluated. Advanced T stage or more extensive neck disease more often caused distant metastases (p < 0.05), and also the histological evidence of tumor invasion in three or more cervical nodes (p = 0.02), and poorly differentiated tumors (p < 0.05). Tumor site, surface area in cm, presence of ulceration, and loco-regional uncontrol were not associated with distant metastases (AU)

[Pharyngoesophageal foreign bodies]. / Cuerpos extraños faringoesofágicos.

A review was made of 77 rigid esophagoscopies to remove a foreign body from the upper digestive tract. Mean age of patients was 59 years. Meat bones were the most frequent impacted foreign body and the cervical esophagus was the most frequent site of impactation. No foreign body was found in 22% of esophagoscopies, a high rate that could have been due to our lack of confidence in radiographic studies, which yield false positive and false negative information. Esophagoscopy is performed whenever patient-referred symptoms are clear. The only complications were three cases of major esophageal erosions without perforation and one pneumothorax. A case of intramural esophageal pseudodiverticulosis was found.

[Congenital absence of major salivary glands]. / Ausencia congénita de glándulas salivares mayores.

Congenital absence of major salivary glands is infrequent, with only a few cases in the world medical literature. We found 30 documented cases in a bibliographic search. We report a case of total agenesia of the parotid gland diagnosed in the course of a study motivated by symptoms of facial pain and the temporomandibular joint. Agenesia of a major salivary gland (specially the partial ones) usually develops with no manifestation that motivates the patient to seek treatment. Parotid aplasia can be an isolated finding or associated with the absence of other glands or with abnormalities such as aplasia of the lacrimal gland, mandibulofacial dysostosis, and ectodermic dysplasia.

Cuerpos extraños faringoesofágicos / Pharynogesopnageal foreign bodies

Hemos realizado una revisión de 77 esofagoscopias rígidas para extraer cuerpos extraños del tracto digestivo superior. La edad media de los pacientes fue de 59 años, el cuerpo extraño más frecuente los huesos de carne y el esófago cervical el lugar más común de impactación. Un 22 por ciento de las esofagoscopias fueron negativas, una alta tasa que nosotros atribuimos a nuestra falta de confianza en los estudios radiológicos dada la existencia de falsos positivos y negativos, lo que nos lleva a realizar la esofagoscopia siempre que la clínica del paciente es clara. Como complicaciones sólo encontramos tres casos de erosiones esofágicas importantes sin ninguna perforación y un neumotórax. También encontramos un caso de pseudodivertículo esofágico intramural (AU)

A review was made of 77 rigid esophagoscopies to remove a foreign body from the upper digestive tract. Mean age of patients was 59 years. Meat bones were the most frequent impacted foreign body and the cervical esophagus was the most frequent site of impactation. No foreign body was found in 22% of esophagoscopies, a high rate that could have been due to our lack of confidence in radiographic studies, which yield false positive and false negative information. Esophagoscopy is performed whenever patient-referred symptoms are clear. The only complications were three cases of major esophageal erosions without perforation and one pneumothorax. A case of intramural esophageal pseudodiverticulosis was found (AU)

Ausencia congénita de glándulas salivares mayores / Congenital absence of major salivary glands

La ausencia congénita de las glándulas salivares mayores, es un hallazgo infrecuente, con pocos casos comunicados en la literatura médica mundial: en la revisión bibliográfica realizada, hemos encontrado 30 casos documentados. Aportamos un nuevo caso de agenesia total de glándula parótida, diagnosticado mediante estudios de imagen, al estudiar un cuadro de dolor facial y de la articulación temporomandibular, pues la agenesia de una glándula salival mayor (especialmente las agenesias parciales), suele cursar sin manifestaciones propias que hagan consultar al paciente. Puede ser un hallazgo aislado o asociado a otras anormalidades, como la ausencia de la glándula lacrimal, disostosis mandibulofacial y displasias ectodérmicas (AU)

Congenital absence of major salivary glands is infrequent, with only a few cases in the world medical literature. We found 30 documented cases in a bibliographic search. We report a case of total agenesia of the parotid gland diagnosed in the course of a study motivated by symptoms of facial pain and the temporomandibular joint. Agenesia of a major salivary gland (specially the partial ones) usually develops with no manifestation that motivates the patient to seek treatment. Parotid aplasia can be an isolated finding or associated with the absence of other glands or with abnormalities such as aplasia of the lacrimal gland, mandibulofacial dysostosis, and ectodermic dysplasia (AU)

[Malignant melanoma of the middle ear, a rare site]. / Melanoma maligno de oído medio, una rara localización.

Melanomas of upper airway tract constitute around 1% of the total melanomas of head and neck. It's for this reason we cannot forget their location in middle ear, although it is truly rare, and we have only found three cases published in literature. We report a patient of 75 years old, who went to our hospital with otorrhea of two months of evolution, with subjective sensation of deafness. In the exploration ENT a bleeding neoformation was observed by otoscopy, which occupied all external auditory canal. The biopsy of this neoformation was informed as malignant neoplasm. These cells show a little cytoplasmic melanotic pigment and were positive to the tinction with Masson Fontana, as well as to the immunohistochemical antibodies S-100 and HMB-45. These antibodies are specific of malignant melanoma. We had a low opinion of surgical treatment because the affectation of the temporal bone and the size of melanoma. Because of badly prognosis, our patient died six months after the diagnosis.

[Effectiveness of tonsillectomy, evaluated by acoustic rhinometry]. / Eficacia de la adenoidectomía evaluada mediante rinometría acústica.

Hypertrophy adenoid commonest cause of nasal obstruction in paediatrics. The morbility can be try alteration in the respiratory physiology, as well as in the face development and function of the middle ear. We used the acoustic rhinometry to evaluate the surgical results of the adenoidectomy in children with hypertrophy adenoid, compiling the data on their clinic, and correlating them as well with the rhinometric results. The acoustic rhinometry is a relatively new method of exploration of the nasal cavity. We obtain space measures that are going to allow to us to prove it. These measures are the cross-sectional areas and volumes registered in the nasal cavity when this cavity is divided longitudinally in two zones, the previous one until 32 mm measured from the narina, and the later one to 64 mm from the same point. For this study we have taken 45 patients with hypertrophied adenoids. We have made measurements pre and post surgery. Our results show a clear gain in areas as in volumes after surgery and also show the utility of the acoustic rhinometry like method of exploration at the time of quantifying the morphometric alterations of the nasal cavity.

[Olfactory esthesioneuroblastoma. Review of seven cases]. / Estesioneuroblastoma olfatorio. Revisión de siete casos.

Esthesioneuroblastomas are fairly rare malignant tumors: only 20 cases have been reported in Spanish records. It is locally aggressive and produces late recurrences. There is no such thing as a universally accepted treatment model. We describe our experience with 7 cases seen in our hospital from 1980 to 1996. A or B stages can be treated with irradiation, surgery or a combination of both. C stages with the most aggressive tendencies may require the combined use of preoperative irradiation, surgery and chemotherapy. We analyzed the forms of presentation, diagnosis, differential diagnosis, treatment, prognosis, and survival. The 5-year survival rate was 75, 60 and 41% for stages A, B and C, respectively.

[ENT outpatient surgery]. / Cirugía ambulatoria en ORL.

The number of patients who undergo outpatient surgery is constantly growing in Spain. Outpatient surgery is interesting not only because of its economy, but also the social benefits that derive from patients returning home sooner and the rapid reduction of the waiting list. We discuss our experience in the last year with 40 patients who underwent surgery. We made a comparative study of patients with the same pathology and anesthetic risk who were treated by outpatient surgery and those treated by inpatient surgery with a traditional technique who were admitted 24 hours before surgery. Our rate of complications (19%) was close to the traditional rate. However, 3, 927, 000 pesetas were saved and the patients were very satisfied, which encouraged us to continue. Indications and complications are discussed.

[Invasive giant prolactinoma]. / Prolactinoma invasivo gigante.

Large prolactin-secreting tumors are rare and their endocrinological and surgical management may be complex. We report the case of a patient with a prolonged history of unilateral tinnitus and sensation of a stopped-up ear who had a very large, invasive and aggressive tumor of the sphenoidal region with bone destruction, invasion of structures in every direction from the sellar region and extracranial extension to the ethmoid and nasopharynx. Serum prolactin level at the time of diagnosis was 16,860 ng/ml (normal: 3-17 ng/ml). Medical treatment with bromocriptine reduced the prolactin level to 31 ng/ml and reduced the size of the tumor, although less than expected. The literature is reviewed and the rarity of such large, invasive prolactinomas is highlighted, as well as the absence of symptoms and signs suggesting the presence of such a large tumor of the skull base.

[Middle ear carcinoma]. / Carcinoma de oído medio.

Middle-ear cancer represents 5 to 10% of all ear neoplasms. Risk factors include chronic suppurative pathology of the middle ear and prior irradiation. Most malignant tumors are squamous-cell carcinomas. We report the case of a 51-year-old woman with this process. The ideal treatment is mastoidectomy or pterosectomy followed by radiotherapy. We made a bibliographic review of the clinical manifestations, diagnosis, treatment, and prognosis of middle-ear cancer. Five-year survival rates range from 25 to 50% in patients who undergo surgery and radiotherapy.

[Sinus manifestations of the acquired immunodeficiency syndrome]. / Manifestaciones sinusales del síndrome de inmunodeficiencia adquirida.

OBJECTIVE: We decided to review the different disorders which may affect the paranasal sinuses in the course of the acquired immuno-deficiency syndrome, and the most suitable treatment for them. DEVELOPMENT: Involvement of the paranasal sinuses is not particularly common in the acquired immuno-deficiency syndrome. The commonest disorder is sinusitis. Also seen are lymphomas no-Hodgkin, small cell, Burkitt's or no Burkitt and immunoblastic. Cases of Kaposi's sarcoma or malignant tumors such as a schwannoma may appear. In our hospital, an HIV+ patient presented with a frontal cerebral abscess which had developed from sinusitis of the whole left paranasal sphere. We have therefore reviewed 150 histories of HIV+ patients and the relevant literature available. CONCLUSIONS: Only in 3.3% of the HIV+ patients reviewed did sinusitis develop during the course of their illness. In one of these, there was intracranial spread of infection with the appearance of cerebral abscess following infection by Streptococcus milleri. We emphasize the review of the literature dealing with sinus pathology in these patients. On many occasions diagnosis was not made since it was masked by other infections. Cure is difficult and relapses are frequent in spite of suitable treatment and are associated with decline in immunocompetence.