[Salivaly gland tumors in childhood: importance of an early diagnosis and treatment]. / Tumores de glándulas salivales en la infancia. Importancia de un diagnóstico y tratamiento precoz.

OBJECTIVE: Review of our series of salivaly gland tumors at pediatric age, given its low frequency and their higher rate of malignancy compared with the adult population. MATERIAL AND METHODS: Retrospective study of the medical records of all pediatric patients with salivaly gland tumors treated in our center between 1998 and 2013. RESULTS: We identified 12 patients. The most common site of tumor location was the parotid gland in 11 patients followed by the sublingual gland in 1 patient. Three patients had previous cancer and radiotherapy history. The diagnosis (histological or radiological) were: 4 hemangiomas, 2 pleomorphic adenomas, 4 mucoepidermoid carcinomas, one acinar cell carcinoma and 1 primitive neuroectodermal tumor (PNET). No patient had metastasis at diagnosis. Preoperative fine needle aspiration (FNA) was performed in 6 patients with solid masses, being diagnostic in all cases. Eight patients underwent surgery. In two cases of total parotidectomy initial neuroapraxia appeared but none had permanent facial paralysis. There were no cases of Frey syndrome. The aesthetic results were good. There was only one tumor recurrence and all patients, except one who died of leukemia relapse, survive today. CONCLUSIONS: Salivaly gland tumors are uncommon in pediatric age but their high rate of malignancy makes it necessary to establish a rapid diagnosis. In our experience, FNA showed a high sensitivity. For malignant tumors, definitive treatment is surgical excision, preserving the facial nerve whenever possible. For vascular tumors, surgery is reserved for cases refractory to medical treatment.

OBJETIVO: Revisión de nuestra serie de neoplasias de glándulas salivales en pediatría, dada su baja frecuencia y mayor tasa de malignidad en comparación con la población adulta. MATERIAL Y METODOS: Estudio retrospectivo de las historias clínicas de los casos pediátricos de tumores de glándulas salivales tratados en nuestro centro entre los años 1998 y 2013. RESULTADOS: Se estudian 12 pacientes. Los tumores afectaban a la glándula parótida en 11 casos y en uno a la glándula sublingual. Tres pacientes presentaban historia oncológica previa y antecedentes de radioterapia. El diagnóstico (histológico o radiológico) fue de: 4 hemangiomas, 2 adenomas pleomorfos, 4 carcinomas mucoepidermoides, 1 carcinoma de células acinares y 1 tumor primitivo neuroectodérmico (PNET). Ningún paciente presentaba metástasis al diagnóstico. Se realizó PAAF preoperatoria en 6 pacientes que presentaban masas sólidas, siendo diagnóstica en todos ellos. Ocho pacientes fueron intervenidos. En dos casos de parotidectomía total apareció neuroapraxia inicial pero ninguno tuvo parálisis facial definitiva. No hubo ningún caso de síndrome de Frey. Los resultados estéticos fueron buenos. Un solo caso presentó recidiva tumoral y todos los pacientes, excepto uno que falleció por recaída de leucemia, sobreviven en la actualidad. CONCLUSIONES: Los tumores de glándula salival son raros en pediatría pero su alta tasa de malignidad hace necesario establecer un diagnóstico rápido. En nuestra experiencia, la PAAF presentó elevada sensibilidad. En los tumores malignos el tratamiento es la exéresis, preservando el nervio facial siempre que sea posible. En los tumores vasculares la cirugía se reserva para casos refractarios al tratamiento médico.

[Cervical cystic lymphangioma: still a challenge]. / Linfangioma quístico cervical: todavía un reto.

INTRODUCTION: Cervical lymphangiomas can not only cause severe sequelae (aesthetic, phonatory or deglutory) but can also be life thereatening due to airway compresion or massive bleeding. This paper analyzes our surgical results, the value of prenatal diagnosis and the use of new techniques such as the EXIT procedure for airway control in sereve cases. PATIENTS AND METHODS: We retrospectively reviewed the medical record of patients with cervical lymphangiomas treated in our center between 1986 and 2009, according to our Clinical Documentation Database. Data referred to prenatal diagnosis, intrapartum airway management, surgical procedures and morbidity, sclerosing substance infiltration and long term sequelae was analyzed. RESULTS: Thirteen cases were identified. 53.8% of the patients were diagnosed by prenatal ultrasound, and MRI was performed in 46.1%. The tongue was affected in 30.7%, parotid glands in 38.4% and airway in 38.4%. Four EXIT procedures were carried out (nasotracheal intubation) and one emergency tracheotomy was needed. Five patients required more than one surgical prodedures, including partial glossectomy, with severe intraoperatory complications in 23% (bleeding, pharyngeal damage). In eight patients primary or adyuvant sclerotherapy was used. Three children with giant masses died, two due to intracystic bleeding and one from sepsis. Among the survivors, 50% have no sequelae. CONCLUSIONS: Cervical lymphangiomas are a very sereve condition, not only due to possible airway compresion or massive bleeding but also becose of the severe secualaes they may cause. Tongue or parotid gland infiltration are difficult to treat. In severe cases diagnosed prenatally a close follow up in selected centers, with multidisciplinary teams consisting of obstetricians and pediatric surgeons, trained in the EXIT procedure is warranted. Despite therapeutic efforts the prognosis of large masses is still poor.

[Auricular alloplastic reconstruction with osteointegrated implants: a new therapy option in microtia]. / Reconstrucción aloplástica auricular mediante implantes osteointegrados: una nueva opción terapéutica en la microtia.

Microtia has been treated over the years with autogenous tissues, although the results continue to improve, it is technically a very demanding procedure, even in the hands of a well trained surgeon. Osseointegrated implant-supported ear prosthesis, is less demanding, more straightforward, and offers a better cosmetic result. Since 1996 we have treated 8 patients with microtia types III and IV, with titanium osseointegrated implant-supported ear prosthesis in the bone of the mastoid process. The surgical procedure consists in two stages. First stage: when the three titanium screws are drill in the mastoid process of the temporal bone, with a previous CT scan of the area, that is used to measured the bone thickness. Second stage is done 3 to 6 months later. Ear remnants are removed at the beginning of the procedure, three titanium couplings are then secured to the integrated fixtures, a plastic cap is attached to each of the abutments, until healing takes place; three to four weeks later the auricular prosthesis made out of silicone over a metallic structure, will we plug in the titanium screws. Cosmetic results and patients acceptability have been good in all 8 cases, with few postoperative complications. This procedure has become and option for the patients with ear malformations.

Maxillectomy in childhood.

A maxillectomy in the upper part of the maxilla is an uncommon operation in childhood. The authors comment on the clinical evolution, the related pathologies and the surgical techniques employed in the treatment of eight patients.

[Juvenile nasopharyngeal angiofibroma]. / Angiofibroma nasofaríngeo juvenil.

Juvenile Nasopharyngeal Angiofibroma is a rare benign neoplasm occurring almost exclusively in adolescent males. Surgery is the only curative treatment, but it is very difficult to be performed, because of the nasopharyngeal location of the tumour. We studied a case and its treatment with arterial embolization before the intervention. Surgery is started using the transfacial route, with total resection and avoiding irreparable mutilations.

[Our experience in the treatment of cervico-facial adenitis caused by non-tuberculous mycobacteria (atypical mycobacteria)]. / Nuestra experiencia en el tratamiento de las adenitis cervicofaciales por micobacterias no tuberculosas (micobacterias atípicas).

The authors believe that the greater part of chronic cervicofacial adenitis actually observed in our hospitals, are not caused by M. tuberculosis or M. bovis but by scrofulaceum mycobacterium, M. avium, M. fortuitum and M. Kansasii, and above all, by the first two of these. They present their experience with 16 cases of cervico-facial adenitis due to atypical mycobacterium (CAAM) treated in our centre during the last years, in which period no case of cervical tuberculosis (CT) was observed. It is important to establish an early differential diagnosis between both etiologies, seeing as treatment is different. Whilst tuberculostatics can solve the phymic infection, surgical extirpation is the only solution for CAAM. The diagnosis of these types of infection is achieved by means of a very characteristic clinical procedure and by cutaneous tests specific for each bacteria. Faced with the clinical suspicion, the total extirpation should be effected of the adenopathic block affected. The exact diagnosis can only be made by the culture of the operatory mass.

[Fistula of the pyriform sinus. Report of 2 cases]. / Fístula del sinus piriforme. A propósito de dos casos.

The authors present two patients of 8 and 11 years of age that presented a left sided piriform sinus fistula diagnosed by esophagram and surgically treated in our centre. One had been operated previously with a diagnosis of relapsing thyroglossal quist on five occasions. The piriform sinus fistula is a recently recognised entity as cause of thyroiditis and suppurative cervical infections in the pediatric age. Communicated for the first time in the literature in 1957 by Japanese authors, it was not until 1973 that Tucker published for the first time in English a case of recurrent cervical abscess caused by a fistula initiated in the piriform sinus. Until now very few cases have been published. The majority of authors consider it to be an embryological residue of the third or fourth pharyngeal pouch. The key to diagnosis is to bear it in mind whenever a patient presents suppurative thyroiditis or repetitive cervical infections, principally of the left side. An esophagram is necessary to demonstrate the course of the fistula. In cases of thyroiditis a thyroidal gammagraphy will allow vision of a cold zone in the upper third of the affected lobe. Treatment consists of total extirpation of the fistula and affected portion of the thyroidal lobe, once the infection has been solutioned. Before intervention it is important to collocate a tube in the fistula's course by means of a direct microlaryngoscopy which will serve as guide and allow the injection of methylene blue.