Facial nerve functionality after parotid tumors surgery

The purpose of this study is to show the connection among tumor histology, surgical treatments and facial nerve postoperative functionality. A retrospective review was conducted on 69 patients with benign and malignant parotid tumors: they underwent surgical treatment for benign and malignant parotid tumors at the Maxillofacial Division of Rome University "La Sapienza" from 1988 to 1997. In our series of patients, we performed conservative superficial parotidectomy, conservative total parotidectomy and radical parotidectomy. Tumor enucleation was reserved for those neoplasms with a limited and restricted mass. The surgical approach was based on mass extension and on histopathologic features. Our findings showed that a careful and a timely diagnosis is very important in order to select a conservative surgical treatment for benign and intermediate grade of malignancy tumors with limited dimensions. Most aggressive histological types, and large mass dimensions require a radical treatment with consequent facial nerve postoperative dysfunctions. Our experience demonstrates that this surgical approach provides a very high rate of success in the cure of tumors and a low rate of facial nerve postoperative dysfunctions.     

Combined surgical therapy of temporomandibular joint ankylosis and secondary deformity using intraoral distraction

Temporomandibular joint (TMJ) ankylosis is a pathological process caused by damage of the mandibular condyle. When this event takes place in subjects during the developmental age, it results in an alteration of the entire maxillofacial complex. Therefore, surgical methods able to remove the temporomandibular ankylosis also include necessary operations to correct the secondary maxillofacial deformity. The distraction osteogenesis has induced our center to modify the surgical protocol for the therapy of patients who have developed TMJ ankylosis and secondary maxillomandibular deformity. We have treated four patients with monolateral ankylosis of the TMJ and serious deformities of the maxillomandibular complex secondary to functional limitation. During the same operation, arthroplasty was performed with the removal of the ankylotic block and the interposition of a temporal muscle flap in the new articular space; an intraoral osteodistractor was also positioned to lengthen the mandible. All patients showed recovery of the eurhythmy of the face and good re-establishment of the symmetry. An average 12-month follow-up showed the average opening of the mouth to be at least 35 mm. The combination of TMJ arthroplasty and intraoral osteodistraction provides good functional and aesthetic results in patients affected by ankylosis who have developed secondary maxillofacial deformities.     

Bilateral (opercular and paracentral lobular) polymicrogyria and neurofibromatosis type 1

Anecdotal cases of polymicrogyria (PMG; a malformation of cortical development consisting of an excessive number of small gyri with abnormal lamination) in patients with neurofibromatosis type 1 (NF1) have been described; however, the cases were unilateral and had negative NF1 genetic testing. We describe an 11-year-old girl with NF1 manifesting as a complex epileptic syndrome, including partial seizures secondarily generalized and status epilepticus, who had in association, bilateral, asymmetrical (opercular and paracentral lobular) PMG. She had a 1-bp deletion (c.1862delC) in exon 12b of the NF1 gene. It is notable that, given the key role played by the NF1 gene product, neurofibromin, in normal brain development, and the relatively high frequency of other brain findings in NF1, there are not more NF1 cases with brain malformations manifesting as PMG.     

Fiber tractography assessment in double cortex syndrome

Introduction  

Subcortical band heterotopia (SBH) or double cortex syndrome is a malformation of cortical development that may be related to intractable epilepsy and severe mental retardation or to mild epilepsy and slight mental delay or normal cognitive functions. Several studies have been performed using neuroradiological or neurophysiological techniques, like SPECT, PET, MRS, fMRI, and MEG, in attempt to better characterize this neuronal migration disorder. Recently, also diffusion tensor imaging (DTI) and fiber tracking (FT) have been used to investigate on white matter anomalies in SBH, adding more information about such gray matter anomaly.     

Elsahy-Waters syndrome: Evidence for autosomal recessive inheritance

Elsahy-Waters or branchioskeletogenital syndrome is a rare MCA/MR syndrome characterized by moderate mental retardation, hypospadias and characteristic craniofacial morphology, which includes brachycephaly, facial asymmetry, exotropia, hypertelorism/telechantus, broad nose, concave nasal ridge, underdeveloped midface, prognathism, and radicular dentin dysplasia. Here we report on a 44-year-old woman and her 45-year-old brother, born to consanguineous parents, who show a striking resemblance to the earlier described patients. The hitherto reported patients were male and in one pedigree parents were consanguineous. The present report of an affected woman and her brother, born to consanguineous parents, supports autosomal recessive inheritance of this condition. We provide a short review of all previously reported patients with Elsahy-Waters syndrome and related entities.     

Pharmacotherapy of spasticity in children with cerebral palsy

Spasticity is one of the most common symptoms presented by neurologic patients. Apart from surgical management, drug therapy is an important treatment of children suffering from spasticity. In this review, recent advances in the pharmacologic armamentarium are reported in detail. In particular, there are oral medications (benzodiazepines, baclofen, dantrolene sodium, alpha 2 adrenergic agonists) and parenteral medications (botulinum toxin type A and B, alcohol). Moreover, there is also baclofen that can be administered intrathecally. There are some reports supporting the use of intramuscular alcohol (45% and/or 5-7% phenol) to reduce spasticity without the loss of voluntary movement or loss of sensation. Among these drugs, intrathecal baclofen is one of the most effective substances that can reduce spasticity significantly in the upper and lower extremities. Finally, the effectiveness of therapy with botulinum toxin type A in the management of spasticity is analyzed. Botulinum toxin type A reduces hypertonia in the injected muscles for a period of 2 to 4 months without important side effects. The purpose of this article is to provide an overview of available oral and parenteral drugs for treatment of spasticity in cerebral palsy and to outline indications and contraindications.     

Mini-invasive lateral oropharyngectomy for T3–T4a oropharyngeal cancer

Mandibular swing is the approach of choice for resection of advanced oropharyngeal carcinomas without bone involvement. This approach requires a mandibulotomy, which is associated with complications. A prospective outcome analysis was performed for 21 patients operated without mandibulotomy for T3–T4a oropharyngeal carcinoma. Tumour size was categorized as T3 in 14 patients (66.7 %) and as T4a (33.3 %) in 7 patients. Twelve patients were N0 (57.1 %), 2 (9.5 %) were N1, and 7 (33.3 %) were N2. Surgical margins were negative in 18 cases (85.7 %), positive in 1 (4.8 %), and close in 2 (9.5 %). Average hospital stay was 14.5 days (range 10–22). Adjuvant treatment (radiotherapy or concurrent chemoradiotherapy) was administered to all but three patients previously irradiated. In all cases radiotherapy started within 42 days of surgery. The 3-year overall survival was 85.7 %, and relapse-free survival was 71.4 %. Oropharyngectomy without mandibulotomy has the same indications as mandibular swing. It provides good access to achieve satisfactory clearance of tumours, sparing patients the morbidity associated with mandibulotomy.     

Surgical techniques in the treatment of pleomorphic adenoma of the parotid gland: our experience and review of literature.

This paper presents a retrospective study carried out on a sample of 100 patients affected by pleomorphic adenoma of the parotid gland and treated at the Department of Maxillofacial Surgery at the University of Rome "La Sapienza" between January 1, 1989 and December 31, 1997. For the diagnosis of this neoformation, cytological tests were performed on material taken from the neoformation using fine needle aspiration and ultrasound scan. In some selected cases, a CT examination of the head and neck with medium contrast or Nuclear Magnetic Resonance (NMR) was carried out. This study sets out to examine the most suitable treatment to be followed for the removal of the pleomorphic adenoma of the parotid gland. In 56 cases the patients underwent a superficial, conservative parotidectomy. Forty one patients had a total parotidectomy with the facial nerve left intact and one patient had a total parotidectomy where the marginal mandibular nerve of the facial nerve was damaged. The remaining two patients involved in the study were suffering from a recurrent pleomorphic adenoma and in these two cases a total parotidectomy was performed where the facial nerve was killed. The removal of the cranial nerve VII in these patients proved necessary because the nerve fibers had adhered to the surrounding scar tissue of the tumor, either after previous surgery or due to repeated chronic phlogosis of the gland.