Our experience with surgical treatment of tympanojugular pragangliomas.

Tympanojugular paragangliomas are benign and slow growing lesions of the lateral skull base. Due to their localization and vascularity, they represent a surgical challenge. Treatment modalities include preoperative embolization of feeding vessels and tumour itself, surgical removal and irradiation. In our group, 16 patients with large tympanojugular paragangliomas have been operated in the period of 10 years. Surgical radicality has been achieved in 62.5%, in the rest of patients the tumour remnants were either irradiated with a Leksell gamma knife, or left without treatment in a wait-end-rescan approach. According to the benign character of the tumour, radicality is not always the highest goal, since a radical removal may be compromised by a high postoperative morbidity.

Complicated decisionmaking in indications for auditory brainstem implant (ABI) in a patient with neurofibromatosis 2.

A case of an accidental finding of neurofibromatosis 2 in a practically asymptomatic patient is described. Various therapeutic modalities, including restoration of hearing after vestibular schwannoma surgery with an auditory brainstem implant (ABI), are considered.

[Bilateral ankylosing paraarticular ossifications of the hip joint with involvement of the sciatic nerve]. / Oboustranné ankylozující paraartikulární osifikace kycelních kloubu s plegií n. ischiadicus.

In December 1997 a 48 year-old man was treated by resection of the bilateral paraarticular ankylosing ossifications that developed after 6 weeks of unconsciousness after necrotic pancreatitis. The ossifications on the posterior part of the hip joint involved a highly separated sciatic nerve. There was a bilateral osseous ankylosis of the hip joint and total denervation of sciatic nerve. In April 1999 the ossifications on the right side were removed. The splitted sciatic nerve was in two osseous channels. The head of the femur was vital and immediately following the operation, flexion of the hip joint was possible to 90 degrees. The same procedure was performed on the left hip joint in October 1999. In April 2000, a re-occurance of the paraarticular ossification on the ventral part of the right hip joint was removed from an ilioinquinal approach. In June 2002 the patient was able to stand and walk without crutches. ROM of the knee joints improved bilaterally to 120 degrees of flexion. The flexion of the right hip joint is 70 degrees and in the left hip is 80 degrees. 3 years after decompression of both sciatic nerves involved in heterotopic bone established significant reinervation. The motor function of the proximal femoral muscles and the function of the tibial nerve was renewed. Complete motor denervation syndrom persists in common peroneal nerve. Sensitive inervation was renewed as in n. tibialis as in common peroneal nerve except acral parts of the foot a toes. Concerning function of the sciatic nerves, the overall results can be assessed as partially succesfull because of partial denervation syndrome of sciatic nerves. In definite clinical improvement the recovery of the neurologic status is very important besides the mechanical improvement of range of motion of the hip joint.

Our surgical experience with large vestibular schwannomas.

Our aim is to remove large vestibular schwannomas (VS) radically with minimal morbidity. Usually, these tumours cannot not be treated by irradiation. In the years 1997-2003, 69 VS were operated in the Department of Otorhinolaryngology, Head and Neck Surgery of the First Medical Faculty in Prague, Czech Republic. Prevailing majority of these tumours were of the 4th grade (House classification), compressing the brainstem. Six patients in the group suffered from neurofibromatosis 2, in five cases the patients were indicated for neurosurgery due to rapid tumour growth after previous irradiation. All tumours were radically removed using a retromastoid osteoplastic and translab approach with an intraoperative nerve monitoring. Good function of the facial nerve was achieved in 90%. The nerve had to be resutured in 4 cases with consequent satisfactory results, cross anatomosis was not performed. Hearing function was preserved in 8% of patients only. In 6 patients with neurofibromatosis 2, the auditory brainstem implant (ABI) was used to preserve hearing. This study demonstrates that a radical removal of large vestibular schwannomas is possible using a minimally invasive surgical technique and peroperative nerve monitoring with a good impact on quality of life. Auditory brainstem implants bring a new chance of hearing after tumour removal in patients with NF2.

[Use of the auditory brainstem neuroprosthesis in the Czech Republic]. / Sluchová kmenová neuroprotéza v Ceské republice.

BACKGROUND: Auditory brainstem implant (ABI) is an electroprosthetic device enabling sound sensations in deaf persons with a bilateral lesion of auditory nerves. Stimulation of auditory nuclei in the floor of the IVth ventricle is realized by an electrode array introduced during surgery in the lateral recess of the IVth ventricle. METHODS AND RESULTS: The main indication group for ABI is represented by patients with neurofibromatosis 2 (NF2) suffering from bilateral vestibular schwannomas. During surgery aimed at tumour removal, auditory nerve function and integrity are almost always destroyed, therefore, an ABI can be introduced as an one stage procedure. Implantees use the device mainly as the aid in lipreading, only very rarely they can comprehend speech without visual cues. Auditory brainstem implant programme has been introduced in the Czech Republic in the year 1999. It was the very first ABI surgery in the Central Europe. Since that time, 5 patients had received the auditory brainstem implant, from which the first four use the device for a longer time. CONCLUSIONS: The last operated patient has not been activated yet. It may be said, that ABI represents a benefit to all our patients, in one implanted this benefit is significant, since he can understand speech without lipreading, the other implantees use the device as an aid in lipreading. In one female patient, the device benefit is severely limited by a motoric handicap after partial cerebellar resection during surgery. Nevertheless, she uses the implant on a daily basis, but contact with her is limited and difficult.

First auditory brainstem implant in the Czech Republic.

In the Czech Republic, the first implantation of a stimulation electrode into the brainstem was performed on 11 January 1999 in the Department of ORL, Head and Neck Surgery, The First Medical Faculty, Charles University in Prague, University Hospital Motol. The selected patient was a 40-year-old woman with neurofibromatosis type 2 (NF2) who had previously undergone bilateral vestibular schwannoma surgery. Both tumours had been radically removed, the left-sided tumour in 1987, the right-sided one in 1988. She had been completely deaf since the last operation, i.e., for 11 years. The surgery was realized by the international cooperation of three teams. Placement of the electrode pad of the Nucleus CI21 + 1M system on the ventral and dorsal cochlear nuclei was performed. Electrically evoked auditory brainstem responses (EABRs) proved the correct position of the electrode array. The post-operative course was uneventful. Six weeks after the surgery the patient received her speech processor. Since that time, the patient already absolved several sessions of a speech processor tune-up. She uses the device as an aid in lip-reading. No adverse or pathological side effects have been observed. The patient was the 45th person in Europe to receive an ABI and the first in the Czech Republic.

1H NMR ganglioside ceramide resonance region on the differential diagnosis of low and high malignancy of brain gliomas.

1. The high-resolution 1H NMR (MRS) spectra of human brain tumor homogenates revealed a broad resonance at 5.3-5.4 ppm in glioblastoma multiforme (N = 16) and brain metastases (N = 3). The broad resonance was identified as ceramide, a sphingosine-fatty acid combination portion of ganglioside, indicating an elevated abundance of monounsaturated fatty acids. GLC analysis of gangliosides in the highly malignant glioblastoma multiforme revealed that the elevated monounsaturated fatty acid is oleic acid (C18:1). The resonance at 5.3-5.4 ppm region was not detectable in normal human brain (N = 2), in meningiomas (N = 2), or in low-grade astrocytomas (N = 12). In normal human brain the abundance of monounsaturated fatty acid is minimal. 2. This investigation was made possible because the method of producing homogenate resulted in (i) no loss of lipids during the process and (ii) a well-homogenised sample, with (iii) no loss in chemical integrity. 3. The properties of tumor gangliosides include antigenic specificity and immunosuppressive activity and the ceramide, a sphingosine-fatty acid combination, noticeably influences the ganglioside immunosuppressive activity. 4. The observation of 1H NMR ceramide resonance in high-malignant brain tumors emphasizes the dramatic role of aberant gangliosides and ceramide precursors on the grade of malignancy and invasiveness. 5. Further insight into the specific nature of the ceramide portion of gangliosides in grading the malignancy of brain tumors should be investigated further.

[Surgical treatment of injuries of the recurrent laryngeal nerve]. / Chirurgická lécba poranení n. laryngeus recurrens.

The authors give an account of the results of suture and decompression of the n. laryngeus recurrence in 15 patients with a mean follow up period of 17,6 months. A total of 12 nerves were sutured, one of them by means of an autograft from the n. suralis. A total of 11 nerves were decompressed. One patients after suture of the nerve and contralateral decompression did not attend a check-up examination. Of 11 sutured and checked nerves the results were very good in ten instances when a remarkable improvement of the voice was recorded as well as of the adduction movement and phonation closure of the vocal cords. In one instance the suture of the nerve failed. After decompression of the nerve in seven instances recovery of the function of the affected vocal cord occurred. After decompression of another two nerves the adduction movement of the vocal cord was restored, the voice became normal and phonation closure occurred. In one instance the nerve decompression failed. EMG examinations were made in three instances after suture of the nerve and in one instance after decompression of the nerve. The EMG examination correlated with the clinical finding. With regard to these results it is possible to recommend after injuries of the n. laryngeus recurrence its revision. The results of suture of the nerve or its decompression within five days after injury are very satisfactory in the group of patients treated by the authors.

[Teamwork in saving the life and lower extremities of a child]. / Záchrana zivota a dolní koncetiny u dítete týmovou spoluprací.

The authors describe the case of a successfully cured severe multiple injury in a seven-year-old boy, who after falling from his bicycle under a lorry suffered a brain injury, injury of the small intestine and of the neurovascular bundle in the retroperitoneal and inguinal area. The author emphasizes that in multiple injuries the life can be saved and best possible function recovered only by a correct algorithm of different surgical procedures in therapeutic care focused first on saving the patient's life and subsequently on restoration of all important functions and application of the maximum of contemporary therapeutic possibilities. The authors describe the life saving revision of the abdominal cavity and arrest of massive haemorrhage, the urgent reconstruction of blood vessels of the left inguinal area made within six hours after the accident and the delayed reconstruction of the femoral nerve made within four months after the injury. The resultant condition is evaluated after a six-year period. The general condition of the injured boy is normal, the functional condition of the left lower extremity can be evaluated clinically and on the basis of EMG as excellent.

The management of extensive tumours of the scalp.

Though tumours of the scalp and skull are not very common certain severe and extensive forms are associated frequently with intracranial propagation and therefore their reconstruction represents often a major problem requiring the use of a series of surgical methods. Their treatment requires the use of expanders, local flaps, pedicled cutaneous and musculocutaneous flaps, as well as free flaps. This report deals with 10 patients with localized extensive tumours larger than 120 cm2 within the region of the scalp and skull subjected to various reconstruction procedures. The applied surgical techniques are discussed and attention is devoted also to the involvement of bone in the above mentioned localization as well as to the problems associated with these lesions.