[Bilateral laryngeal pouch tumor. Localized amyloidosis of the larynx].
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Biomedical subjects
Publications and source records attributed to B Schick.
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Beckwith-Wiedemann syndrome is a rare genetic overgrowth syndrome presenting with organomegaly, abdominal wall defects, macroglossia, and postnatal hypoglycemia. Head and neck manifestations of this abnormality include flame nevus of the forehead and characteristic sulci of the ear lobe. We present a 7-year-old child with Beckwith-Wiedemann syndrome and a rare finding of conductive hearing loss on both sides due to congenital malleus and stapedial fixation. Small fenestra stapedotomy and mobilization of malleus fixation in the epitympanum improved the child's hearing. The bony fixation of the malleus and stapes is explained as atavism of the processus anterior mallei and peripheral lamina stapedialis in embryological development.
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Hemangiopericytomas are mesenchymal tumors and account for about 1% of all CNS tumors. Aggressive growth, tendency to local recurrence and relatively frequent metastases are the clinical features of these tumors. Histological characteristics are marked cellularity, vascularity and a dense net of reticular fibers. This case presents a patient with a local recurrence of a right temporal, atypical meningioma that had been operated on and irradiated elsewhere. After embolization large parts of the tumor were removed palliatively. Histological aspects of the resected tumor were consistent with a diagnosis of an atypical meningioma. Not until hepatic metastases from this tumor were found was the diagnosis re-examined and corrected to a malignant meningeal hemangiopericytoma. Surgical resection of primary tumor with frequent controls and, if needed, postoperative radiation therapy is the treatment of choice. Furthermore metastasizing atypical meningiomas should be examined critically to determine if a hemangiopericytoma is present.
Hemangiomas of the skull base are rare neoplasms and are easily misdiagnosed as acoustic neuromas when occurring in the internal auditory canal. Among these tumors, ossifying hemangiomas are characterized histologically be newly formed bone tissue within their substance. We describe a 26-year old female patient who presented with left-sided sensorineural hearing loss and tinnitus. T2-weighted magnetic resonance imaging demonstrated a bright space-occupying lesion of the internal auditory canal with extension to the geniculate ganglion. Bony erosions of the internal auditory canal were proved by high-resolution computed tomography. A hemangioma was suspected preoperatively and was resected via a middle cranial fossa approach. Histologically, new bone formations were found in a cavernous hemangioma. In general, radiologic findings can suggest a hemangioma of the internal auditory canal and help to differentiate it from acoustic neuroma. Based on the histological findings of intratumoral bone formation, the hemangioma in our patient was classified as an ossifying hemangioma. However, reactive bone formation at the borders of a tumor in the internal auditory canal can also be mistaken as new intratumoral bone formation.
A rare case of an unusual sinus within the styloid process is described. The radiological appearance of this malformation is presented with CT-scans and a hypothesis for its development is presented. The malformation is designated as "Recessus processus styloidei" and this entity is considered to be a dysgenesis of the second branchial arch.
Topical corticosteroids are one of the main pillars in the treatment of nasal polyps. The exact topography of their intranasal deposition has not yet been adequately visualised. The intranasal distribution of a 1% sodium fluorescein solution applied with original Pulmicort Topinasal (budesonide) metered pump bottles was analysed by videoendoscopy. The study group included eight healthy subjects and ten patients who had undergone endonasal sinus surgery. Videoendoscopy was performed in the study group within the first minute after application of the fluorescein solution. Additionally the deposition pattern of Pulmicort Topinasal was analyzed using a nasal model. The examination showed that the majority of the substance is deposited on the anterior portion of the nasal septum and the head of the inferior turbinate. Only a small fraction actually reaches the middle meatus. The distribution is improved by application during the decongested phase of the nasal cycle, after use of vasoconstricting nasal drops and maintaining a spraying angle of 45 degrees upwards. The development of new delivery techniques and systems could improve the efficacy of intranasally administered corticosteroids and reduce the complication rate.
Surgery is the most common treatment for angiofibromas, but the approach is still a major point of discussion. Five cases of angiofibroma with typical localisation were treated surgically by an endonasal approach at the Fulda Academic Teaching Hospital from 1994 to 1997. This article presents an analysis of the clinical findings, computer tomography and magnetic resonance imaging, preoperative embolization, operative technique and complications. Endoscopic and radiologic follow-up ranging from 5 to 39 months excluded any residual tumour or recurrence. The endonasal microendoscopic approach with adequate preoperative embolization should be considered as an useful technique for removing tumours with considerable size without using an external incision.
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We describe an unusual occult transclival cerebrospinal fluid (CSF) fistula to the sphenoid sinus demonstrated by MRI. CT was performed because of a posterior cerebral infarct caused by cardiac arrhythmia. Axial sections showed fluid in the sphenoid sinus. High-resolution scans revealed a bony defect 3 mm in diameter of the posterior wall of the sphenoid sinus, and MRI showed a transclival CSF fistula. This occult lesion was confirmed by surgery and duraplasty was successfully performed via an endonasal approach.
The case of a 50-year-old man with an unusual sinus of the styloid process is presented. This anomaly caused recurrent otorrhea and repeated neck inflammation. The sinus was identified in the upper part of the styloid process and was resected completely as definitive treatment. Since the styloid process is formed by mesenchymal tissue of the second branchial arch, lack of chondrification and ossification was presumed to be the cause of the sinus found in the patient. In case of direct communication between the styloid process cavity and the middle ear space the term "styloid process recess" is suggested. This recess can act as a possible source for recurrent middle ear infections and neck inflammations.
Angiofibromas are usually characterized by typical location with involvement of the posterior nasal cavity and nasopharynx. They are most commonly found in adolescent males. Despite these facts, a case of an angiofibroma presented in a 15-month-old boy at an atypical site anterior and medial to the lacrimal sac. The tumor was resected via an endonasal, micro-endoscopic approach avoiding an external incision.
Clarithromycin is a relatively new macrolide antibiotic with an action spectrum similar to that of erythromycin. Its main indications for use are for upper and lower respiratory and skin and soft tissue infections. Little is known about its safety in pregnancy, although animal reproductive studies found an increased rate of cardiovascular anomalies, cleft palate, and embryonic loss. Human data, limited to case reports and one small uncontrolled study, cannot allow evidence based counseling of pregnant women who were exposed to the drug before finding out they were pregnant. Pregnant women who had been counseled on the use of clarithromycin by five centers, were matched for age, smoking, and alcohol use with a control group of pregnant women who were exposed to nonteratogenic antibiotics. A total of 157 women were followed up. Of these, 122 were exposed to the drug in the first trimester. There were no significant differences found between the two groups in the rates of major and minor malformations; 2.3 versus 1.4% for major (p = 0.86) and 5.4 versus 4.9% for minor (p = 0.96). Spontaneous abortion rates in the exposed group was significantly different, higher (14%) than in the control group (7%) (p = 0.04). This first prospective controlled study of exposure to clarithromycin in pregnancy suggests that this agent does not increase the rate of major malformations above the baseline risk of 1-3%. The higher rate of reported spontaneous abortions, although still within the expected baseline rate, may warrant further study.
BACKGROUND: Meningitis or cerebrospinal fluid rhinorrhea can occur years or even decades after trauma and can be the first indication of a previously unidentified dural lesion. In spite of being difficult, precise localization of an occult traumatic dural lesion is a necessary prerequisite for duraplasty. PATIENTS AND METHODS: In a retrospective study covering a period of 17 years, we identified 27 patients with occult traumatic dural lesions. The dural lesions were localized by high-resolution CT, MRI, fluorescein nasal endoscopy, and CT cisternography. In all cases the expected dural lesion was demonstrated and treated surgically. RESULTS: In 23 out of 27 patients (85%), the rhinobasal fistula was detected as a bony defect by CT. Furthermore MRI examination in 100% (5/5), a preoperative fluorescein nasal endoscopy in 50% (10/20) and CT cisternography in 56% (5/9) were able to localize the dural lesion. CONCLUSION: We recommend high-resolution CT and MRI as diagnostic tools of choice in searching for occult dural lesions. Fluorescein nasal endoscopy and CT cisternography are justified as invasive techniques if CT and MRI are inconclusive but clinically CSF leakage is still suggested. The patient will remain at risk of potentially fatal meningitis until the lesion is appropriately repaired by duraplasty. Therefore modern clinical and radiological diagnostic methods should be used to search for an unknown dural lesion.
BACKGROUND: Haemangiopericytomas are rare vascular neoplasms which show either slow local tumour growth or aggressive progression in size with a high tendency of recurrence and metastasis. Manifestations of haemangiopericytomas in the area of the nose and sinuses are supposed to have a relatively benign course. To date clinical and histological parameters for prognosis are uncertain. Therefore in a quite large number of cases only the clinical course allows to evaluate the dignity of a haemangiopericytoma. PATIENTS AND RESULTS: We present our experience with five haemanglopericytomas in contact with the skull base out of a total number of 457 skull base tumours. The site of origin of these five haemangiopericytomas were: nasal septum, pterygopalatine fossa, oronasopharynx, temporal bone, and parotid gland. All neoplasms showed primary or recurrent tumor in contact with the skull base and an intracranial extension was observed twice. One patient died postoperatively of a cerebral infarction. Three patients showed one or more recurrences which were treated surgically. In one case 5 recurrences occurred. Despite of 4 surgical procedures within a period of 15 months, the tumour could be controlled only for a short period of time, and the patient died 16 months after the first operation. CONCLUSION: One has to consider that haemangiopericytomas of the head and neck show a potential malignant course. The vascular tumor may recur decades later. Adequate therapy and life-long follow-up are therefore mandatory in haemangiopericytomas. Complete surgical resection of the tumour is usually the treatment of choice, in spite of the high degree of surgical skill required, especially in advanced tumour and in case of recurrence.
BACKGROUND: The operative treatment and radiation therapy of jugulotympanic paragangliomas (JTP) are still a matter of controversial discussion. In spite of various improvements during the last 50 years, selecting the appropriate treatment modality (surgery, radiation, or observation) is still a challenge. PATIENTS: During a 16-year period, 44 patients with 45 JTP (10 at level A/B and 35 at level C/D according to Fisch) were seen at the ENT-department in Fulda. Forty-one cases were treated surgically. RESULTS: Complete resection was possible for level A/B in 100% of the patients (n = 10). Residual tumor was demonstrated for level C in 23% of the patients (5/22) and for level D in 40% (4/10) with a median follow-up time of 69 months. In two cases residual tumor was treated by radiation. Six patients with residual paraganglioma tissue were maintained under observation without any evidence of tumor progression (median follow-up time 39 months). We report one death after the attempt to resect a large residual paraganglioma that had already caused brain stem compression. A sufficient duraplasty could not be achieved following radiation therapy. CONCLUSIONS: Complete tumor resection of jugulotympanic paragangliomas of levels A and B is often possible without injury to the cranial nerves. Extensive tumors present difficulties in complete tumor resection and increase the risk of cranial nerve injuries. Advanced paragangliomas therefore require an individualized therapeutic regime including surgery, radiation therapy, and observation of tumor growth.
BACKGROUND: Calvarial bone graft is often used in reconstructive cranio-facial surgery. As most common three different forms can be distinguished: outer-table bone, full thickness grafts and composite flaps (bone with a periostal or muscular pedicle). PATIENT AND METHOD: An extensive fibrous dysplasia of the frontal region was removed in a 26 years old patient. Reconstruction was carried out with alloplastic material achieving a good esthetic result. Recurrent seroma and occurrence of a fistula demanded removal of the alloplastic material and en-bloc reconstruction of the forehead region was accomplished with a parietal outer-table graft. Within a follow-up time of one year a good esthetic and stable reconstruction has been achieved. CONCLUSION: Split-thickness calvarial bone is still a versatile graft in reconstruction of the forehead region. Although a low rate of side effects in harvesting calvarial bone grafts are in general expected, one has to be aware of dural lesions occuring in the donor site during craniotomy.
BACKGROUND: The loss of facial nerve activity reduces the quality of life in regard to functional disturbances and to the appearance of the patient. As far as possible a reconstruction of the facial nerve should be advocated. Only this procedure allows the natural variety of differentiated facial expression. If reconstruction of the facial nerve is not indicated or turns out to be unsatisfactory, a dynamic muscle transposition should be considered. PATIENTS AND METHODS: The patient records of the ENT-Departments in Fulda for the period from 1988 to 1995 and in Mönchengladbach from 1993 to 1995 were reviewed to survey the experience of secondary plastic rehabilitation of the paralysed face. Our special interest focussed on the oral region with dynamic muscle transfer. The techniques leading to rehabilitation of the lid region were included in this study. RESULTS: Transfer of the temporalis muscle was performed 12 times and a masseter muscle flap 4 times to rehabilitate the oral region. Static rehabilitation (canthoplasty, tarsorrhaphy) of the lid was performed 8 times, implantation of a gold weight in the upper lid 5 times and dynamic rehabilitation of the lid region with a temporalis muscle transposition once. CONCLUSIONS: Rehabilitation of the oral region is best managed with a temporalis muscle flap because of its favourable vector of tension and its broad, flat form. Masseter muscle transposition is particularly indicated if a temporalis muscle transfer is not possible or if resection of the facial nerve because of malignancy of the parotid gland has to be performed and nerve reconstruction is not advisable.