Congenital plate-like osteoma cutis of the forehead: an atypical presentation form.

Cutaneous ossifications or osteoma cutis can be found in many syndromes. Primary osteoma cutis, present since birth or the first months of life, in the absence of metabolic disorders or trauma, is found in congenital plate-like osteoma cutis and progressive osseous heteroplasia, coexisting in the latter with deep connective tissue ossifications. This report documents the case of a 7-year-old female with a plate-like cutaneous ossification of the forehead causing aesthetic deformities. Other dermal ossifications in the inguinal and preauricular region, as well as the left hand and a small focus in the extraocular muscles of the left orbit, were also present. These lesions did not show progression, and most of them were present since birth. The lesion of forehead was treated surgically. The lack of progression and the fact that the orbital lesion was solitary still fits the criteria for the diagnosis of plate-like osteoma cutis. Plate-like osteoma cutis and progressive osseous heteroplasia may represent varieties of the same disorder.

[Tumors of the parotid gland]. / Tumeurs de la glande parotide.

Tumors of the parotid are the most frequently encountered salivary gland tumors. Knowledge of the histology and anatomy of the salivary gland is important when considering the histiogenesis of salivary gland tumors, requiring close cooperation between the pathologist and the surgeon. Most tumors are benign epithelial formations. Pleomorphous adenomas predominate. Superficial lobectomy is adequate treatment. When the tumor involves a deep lobe, total parotidectomy is indicated. Treatment of malignant tumors depends on the histology, its TNM stage and other factors. Total parotidectomy with lymphadectomy and radiotherapy are needed in case of high grade malignancy. In children, vascular neoplasias are the most frequent, followed by malignant tumors. Their histological features and treatment are the same as for adults.

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.

[Goldenhar syndrome]. / El sindrome de Goldenhar.

We present a report on 9 patients with Goldenhar's Syndrome, and the criteria used for the diagnosis. Familiar data and clinical characteristics. Are described and discussed with that of other authors. The medical literature about this syndrome shows a lack of uniformity on the criteria necessary to diagnose the syndrome, as well as the role of the pathogenic factors. The authors propose the adoption of uniform criteria on the basis of their own experience and the report of Feingold and Baum. They can help to better delineate the differences between Hemifacial Microsomia and Goldenhar's Syndrome.

[Primary cervical tumors of the sympathetic nervous system. Report of 8 cases]. / Tumores cervicales primitivos del sistema nervioso simpático. A propósito de 8 casos.

The authors present his experience in the treatment of 8 patients with primary tumors of cervical sympathetic ganglions. The presence of a hard, painless, adherent and slow evolution latero-cervical mass is the first sign in 7 cases. The other one, a neuroblastoma, had a Horner's syndrome. The authors expose the methods of diagnostic and staging, some characteristics of surgical technique, chemotherapy treatment and follow-up methods (TAC, Gammagraphy with i-MIBG and Tumoral Markers). This imply a survival in the 100% of cases.

Haemangiomas of the parotid gland in children.

The authors present the case histories of 40 intraparotid haemangiomas, in 11 of which they achieved healing after radical surgical treatment (parotidectomy). Since 1971, the authors have adopted a conservative approach (not surgical), seeing that most of the cases involute spontaneously up to complete healing. None of the 29 cases treated conservatively has undergone exacerbations of the process, after the age of 6-9 months. No emergency surgical operations were required.