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The value of visual evoked potentials in the evaluation of periorbital hemangiomas.

PURPOSE: To present a case of a child with an extensive facial hemangioma in whom the suspicion of intracranial involvement was raised by visual evoked potential (VEP) evaluation. DESIGN: Observational case report. METHODS: VEP analysis was performed using flash and pattern stimuli to determine whether treatment intervention other than occlusion therapy was needed. RESULTS: VEP analysis indicated a marked transoccipital asymmetry in the flash and pattern VEP suggestive of right hemispheric dysfunction. Neuroimaging showed intracranial extension of the hemangioma. CONCLUSION: The VEP evaluation is a noninvasive technique performed in the awake child. This case highlights the usefulness of VEP analysis in the management of extensive periocular capillary hemangiomas and its use as a modality to identify potential intracranial involvement. Large plaquelike facial capillary hemangiomas may have possible underlying dural involvement suggestive of PHACES syndrome.

Amblyopia↗

Orbital exenteration for invasive skin tumours.

Orbital exenteration aims at local control of disease invading the orbit that is potentially fatal or relentlessly progressive. Of all exenterations presenting to ophthalmologists, 40-50% are required for tumours in the eyelid or periocular skin. 99% of these are basal cell carcinomas and 4-6% each are squamous cell carcinomas or sebaceous gland carcinomas. Orbital invasion results in progressive fixation of the tumour to bone and reduced ocular motility. Perineural invasion of branches of the trigeminal nerve leads to numbness or pain, and that the facial nerve, to weakness. Biopsy identifies the cell type and the presence of perineural invasion. CT and MRI scanning help in the assessment of tumour spread within the orbit. Management should be in collaboration with an oncologist. Exenteration may be total-the removal of all orbital contents-or lid-sparing if the tumour is placed posteriorly. The socket may be allowed to heal by granulation or lined with a split skin graft or local flap. Complications may be seen following 20-25% of exenterations and include fistulae, tissue necrosis, exposed bone, and infection. Incomplete clearance of tumours occurs in about 38% of total exenterations and 17% of subtotal. The overall 5-year survival is 55-65%, but significantly worse if there was perineural spread. Facial prostheses may be mounted on glasses or secured with tissue glue or osseointegrated implants. Excellent cosmetic results can be achieved but many patients prefer to wear a patch.

Basal Cell Carcinoma↗

Facial pain due to perineural invasion by basal cell carcinoma.

Perineural invasion by basal cell carcinoma is rare and has been reported only in cases of extensive recurrent carcinoma. A patient with facial pain and progressive fifth cranial nerve symptoms was found to have perineural invasion by basal cell carcinoma in the absence of recurrent disease.

Aged↗

EMOTIONAL REACTIONS TO DISFIGUREMENT FROM CANCER THERAPY.

Disfigurement not only produces current anxieties but reactivates childhood conflicts. The emotional reaction depends upon the disturbance to the patient's major adaptations to life as well as the meaning of the organ to the patient. Fear of isolation and rejection by others may be more terrifying than fear of death. Emotional reactions include regression with marked dependency, anxiety, depression, hostility and, if severe, paranoid states, hypochondriasis, denial, counterphobic behaviour, obsessive-compulsive reactions and schizophrenic reactions. Management basically involves early establishment of a positive doctor-patient relationship. In such a relationship the physican should educate his patient, undercut guilt, accept transient regression and expression of anger, set limits on counterphobic behaviour, either support or gently question denial of reality, and support, without being overly sympathetic, a depressed patient. The nurse, social worker, psychiatrist and the patient's family may be valuable members of the therapy team. Disfigurements of various body areas pose individual problems of management.

Amputation, Surgical↗

Mucinous adenocarcinoma of the face. A case report.

Cutaneous mucinous adenocarcinoma is a rare neoplasm. It occurs most commonly on the facial skin. We present a case of an apparently recurrent primary mucinous adenocarcinoma of the facial skin and highlight the necessary diagnostic work up and management of this tumour.

Adenocarcinoma, Mucinous↗

Tumors arising in accessory parotid tissue.

A summary is presented of our experience with 23 patients who had accessory parotid tumors, comprising 1% of all primary parotid neoplasms seen during a 40 year period. Although more than half of the tumors proved malignant, adequate excision was usually curative. We prefer to approach these tumors through an extended cheek-flap incision. Once the lesion is exposed, either local excision or resection in conjunction with subtotal parotidectomy can be performed, depending on the clinical findings.

Adult↗

Hemifacial infantile hemangioma with intracranial extension: a rare entity.

Intracranial hemangiomas are uncommon, especially in the absence of diffuse hemangiomatosis or the syndrome consisting of posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, eye abnormalities, sternal clefting, and/or supra-umbilical raphe (PHACES). We saw an 8-month-old ex-premature girl with a large left-sided ocular and facial hemangioma that had been growing since early infancy. Examination revealed a 7 x 13 cm violaceous tumor involving the left periocular region and face. Ophthalmologic examination revealed deprivation amblyopia, anisometropia with myopia and astigmatism. Magnetic resonance imaging demonstrated a vascular tumor mass involving the scalp, face, and calvarium with extension into the orbit, infratemporal fossa, nasopharynx, lateral medullary cistern, internal auditory canal, and fourth ventricle. Marked enhancement was seen with contrast, and no posterior fossa malformations were noted. She was treated with prednisolone, which was tapered over 12 months. Follow-up magnetic resonance imaging examination at 25 months showed a marked decrease in the size of all lesions, with residual hemangioma in the periorbital soft tissues and small foci in the orbit and intracranial sites. The brain and ventricular system were normal. Intracranial hemangioma may occur in the setting of a large facial hemangioma (especially segmental) in the absence of the PHACES syndrome or diffuse hemangiomatosis. Radiological imaging should be considered to assess for intracranial hemangioma as well as posterior fossa or arterial anomalies.

Amblyopia↗

[Extensive acrodermatitis chronica atrophicans involving the face].

We report on an 81-year-old woman suffering from extensive acrodermatitis chronica atrophicans with facial involvement. This unusual manifestation may be related to an immunodeficiency state in the course of multiple malignancies. The cutaneous lesions cleared significantly during an aminopenicillin therapy administered orally over 4 weeks.

Acrodermatitis↗

Linear nevus sebaceous syndrome.

The linear nevus sebaceous syndrome is a rare disorder first described in 1962 in two cases by Feuerstein and Mims. As originally described, it consisted of the triad of the characteristic midline facial linear nevus sebaceous of Jadassohn, seizures, and mental retardation. The authors have followed a patient with this syndrome from birth to age 8 and although he does demonstrate several neurologic and ocular abnormalities, he enjoys normal intelligence and has never suffered seizures. The features of this syndrome and several new ocular findings demonstrated in this case are described. Based on our long-term follow-up of this patient and our review of the reported cases to date, the authors suggest that the triad for this disorder should be changed to include the midline facial linear nevus sebaceous of Jadassohn, neurologic abnormalities which may, but not necessarily, include seizures and mental retardation, and ophthalmologic abnormalities.

Arachnoid↗