Biomedical subjects
Publications and source records attributed to H A Marres.
A 73-year-old man suffered from gustatory sweating after submandibular gland removal. Frey's syndrome was diagnosed and successfully treated with intracutaneous injections with botulinum A toxin.
PURPOSE: The predictive potential of tumor cell kinetic parameters may be improved when they are studied in relation to other microenvironmental parameters. The purpose of this investigation was to quantitatively categorize human tumor samples according to proliferation patterns. Second, it was examined whether these characteristics are retained after xenotransplantation. METHODS AND MATERIALS: Fifty tumor samples from head-and-neck cancer patients were immunohistochemically stained for Ki-67 and vessels. Also, parts of the samples were transplanted into nude mice. Tumors were categorized according to previously described patterns of proliferation. Vascular and proliferation patterns were analyzed using an image processing system. RESULTS: The 50 tumors were categorized into four patterns of proliferation by visual assessment: marginal (6), intermediate (10), random (21), and mixed (12). One tumor could not be classified. These patterns were quantified by calculating the Ki-67 labeling index in distinct zones at increasing distance from vessels yielding good discrimination and significant differences between patterns. The probability of growth after xenotransplantation was significantly higher for tumors with a labeling index and vascular density above the median value compared to tumors with both parameters below the median (82% vs. 35%). Fifty percent of the tumors retained their proliferation patterns after xenotransplantation. CONCLUSION: The categorization by proliferation pattern previously described by others was reproduced quantitatively and spatially related to the vascular network using a computerized image processing system. The combination of quantitative and architectural information of multiple microenvironmental parameters adds a new dimension to the study of treatment resistance mechanisms. Tumor models representative of the various patterns can be used to further investigate the relevance of these architectural patterns.
Two patients, a man and a woman aged 36 and 40 years, respectively, were found to have disturbed blood pressure regulation after medical intervention in the jugular area. The man had undergone radiation therapy of the neck due to regional lymph node involvement of a nasopharyngeal carcinoma. Six years later he developed episodic complaints of headache accompanied by labile hypo- and hypertension. Mental and physical stimuli resulted in excessive sympathetic activation. In the woman, a carotid body tumour was resected bilaterally. There was an immediate onset of hypertension after surgery. In both patients, the clinical syndrome was attributed to afferent baroreflex failure due to carotid sinus denervation.
OBJECTIVE: To analyze the relationship between pure-tone hearing threshold and speech recognition performance in DFNA2/KCNQ4 and DFNA9/COCH, 2 types of high-frequency nonsyndromic hearing impairment. DESIGN: Case series with cross-sectional analysis of phoneme recognition scores related to age and hearing level. SETTING: University hospital. PATIENTS: Forty-five members of 4 separate families, all carrying 1 of 3 different mutations in the KCNQ4 gene at the DFNA2 locus (1p34); 42 members of 7 separate families, all carrying the same Pro51Ser mutation in the COCH gene at the DFNA9 locus (14q12-q13). RESULTS: The deterioration of speech recognition dropped to a 90% score at a higher level of hearing impairment (pure-tone-average at 1, 2, and 4 kHz) in DFNA2-affected patients (65 dB) than in DFNA9-affected patients (46 dB). CONCLUSION: At similar levels of hearing impairment, DFNA2/KCNQ4-affected patients showed better speech recognition performance than DFNA9/COCH-affected patients.
We report on a patient with recovery of activity of the left orbicularis oris and nasalis muscles 3 months after a complete left facial palsy. Stimulation of the affected facial nerve evoked no responses, whereas contralateral facial nerve stimulation showed polyphasic responses with very long latencies in the nasalis and orbicularis oris muscles. Needle electromyography (EMG) revealed abnormal spontaneous activity in the left orbicularis oris muscle. The motor unit action potentials on the left side of the face could be recruited only during marked contraction of the corresponding muscles on the right and were of low voltage and polyphasic ("nascent potentials"). Contralateral reinnervation is probably due to sprouting of terminal branches crossing the midline of the face and innervating bundles of muscle fibers on the affected side. This phenomenon seems unfamiliar to most clinicians. Whether the activity is due to conduction along nerve fibers or muscle fibers crossing the midline is discussed.
Laryngoceles and saccular cysts, which are abnormal dilatations of the laryngeal saccule, are uncommon. The etiology is unknown but is probably related to both congenital and acquired factors. These structures are usually asymptomatic and are incidentally discovered through radiographic studies for unrelated symptoms. We describe two patients with upper airway obstruction, one caused by a giant laryngocele and the other by a large saccular cyst. In the former patient, acute tracheotomy had to be performed. The laryngocele and saccular cyst were removed surgically, which relieved patients' symptoms of upper airway obstruction.
UNLABELLED: We performed a retrospective study of all children with Pierre Robin sequence (PRS), admitted to our hospital from 1981-1998 in order to evaluate diagnosis, treatment and prognosis. Patients were divided into two categories: isolated PRS (group 1) and PRS plus, i.e. PRS as part of a more complex syndrome (group 2). A total of 74 patients with PRS were found, 29 (39%) males and 45 (61%) females of whom 47 (63.5%) could be categorised as isolated PRS and 27 (36.8%) as PRS plus. The most frequent diagnoses in patients with PRS plus were Stickler syndrome and the velocardiofacial syndrome. Ophthalmological and fluorescent in situ hybridisation of chromosome 22 investigations should therefore be performed in all patients, as soon as the diagnosis of PRS is established. Some form of airway treatment was necessary in the majority of patients (52 of 74), most could be treated conservatively with prone/lateral positioning and close observation. Endotracheal intubation was necessary in one child from group 1 versus five from group 2. Tracheostomy was performed in three children from group 1 and two from group 2. Feeding problems occurred in about 25% of all PRS patients and stunted growth was seen especially in boys with isolated PRS before the age of 10 months. CONCLUSION: In our series, 33% of patients with Pierre Robin sequence plus had Stickler and velocardiofacial syndromes. Conservative airway management was a sufficient treatment for respiratory problems in the majority of patients. Feeding and growth need special attention in patients with Pierre Robin sequence.
Branchio-oto-renal (BOR) syndrome is characterized by ear malformations, cervical fistulas, hearing loss, and renal anomalies. It is an autosomal dominant disorder with variable clinical manifestations. The most common features of BOR syndrome are branchial, hearing, and renal anomalies. However, many affected subjects have been observed with branchial-cleft anomalies and hearing loss but without renal anomalies, a condition called "branchio-otic" (BO) syndrome. It is logical to question whether the BOR and BO syndromes are allelic or whether they represent distinct genetic entities. We identified a very large extended family whose members had branchial and hearing anomalies associated with commissural lip pits that segregated in an autosomal dominant fashion. Using a genomewide search strategy, we identified genetic linkage, with a maximum LOD score of 4.81 at recombination fraction 0, between the BO phenotype and polymorphic marker D1S2757 in the genetic region of chromosome 1q31. This is the first report of linkage for a second gene associated with BOR syndrome. The findings have important clinical implications and will provide insight into the genetic basis of BOR syndrome.
Tumour oxygenation and vasculature are determinants for radiation treatment outcome and prognosis in patients with squamous cell carcinomas of the head and neck. In this study we visualized and quantified these factors which may provide a predictive tool for new treatments. Twenty-one patients with stage III-IV squamous cell carcinomas of the head and neck were intravenously injected with pimonidazole, a bioreductive hypoxic marker. Tumour biopsies were taken 2 h later. Frozen tissue sections were stained for vessels and hypoxia by fluorescent immunohistochemistry. Twenty-two sections of biopsies of different head and neck sites were scanned and analysed with a computerized image analysis system. The hypoxic fractions varied from 0.02 to 0.29 and were independent from T- and N-classification, localization and differentiation grade. No significant correlation between hypoxic fraction and vascular density was observed. As a first attempt to categorize tumours based on their hypoxic profile, three different hypoxia patterns are described. The first category comprised tumours with large hypoxic, but viable, areas at distances even greater than 200 micrometer from the vessels. The second category showed a typical band-like distribution of hypoxia at an intermediate distance (50-200 micrometer) from the vessels with necrosis at greater distances. The third category demonstrated hypoxia already within 50 micrometer from the vessels, suggestive for acute hypoxia. This method of multiparameter analysis proved to be clinically feasible. The information on architectural patterns and the differences that exist between tumours can improve our understanding of the tumour micro-environment and may in the future be of assistance with the selection of (oxygenation modifying) treatment strategies.
Perineural spread of head and neck cancer is an uncommon cause of cranial neuropathy. We studied five patients with cranial neuropathy resulting from perineural spread of head and neck carcinomas. Trigeminal neuropathy with facial pain or paresthesias was the most common clinical manifestation. MRI in the coronal plane under gadolinium enhancement established the diagnosis by visualization of the lower divisions of the trigeminal nerve. Perineural tumor spread can cause headaches in patients with head and neck cancer.
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OBJECTIVES: To verify the initial results of a new anterograde replacement method of the second-generation indwelling Provox voice prosthesis, Provox 2 (Atos Medical AB, Hörby, Sweden), and to determine its device life. DESIGN: Nonrandomized, multi-institutional, controlled clinical trial. SETTING: Four academic hospitals and/or comprehensive cancer centers in The Netherlands. PATIENTS: Two hundred thirty-nine consecutive patients who had undergone laryngectomy and were visiting the outpatient clinic for replacement of their voice prosthesis. INTERVENTION: Anterograde replacement of the Provox 2 voice prosthesis. MAIN OUTCOME MEASURES: Evaluation of ease of use by the medical professional and appreciation by the patients, by means of structured questionnaires; comparison of device life between the original Provox and the new Provox 2 voice prosthesis in a subset of patients. RESULTS: Voice prostheses replaced were Provox (n = 188), Groningen (Medin, Groningen, The Netherlands) (n = 47), and Nijdam (Medin) (n = 4). Anterograde replacement of Provox 2 was always possible. The new anterograde method was preferred by the medical professionals in 97.1% of cases and by 93.7% of the patients, who reported significantly reduced discomfort (P<.001). There was no significant difference in device life between Provox and Provox 2 (median, 125.5 and 104 days, respectively). In 57.5% of patients, the Provox 2 device life was shorter and in 42.5% it was longer (sign test, P = .09). CONCLUSIONS: The results of the initial study concerning ease of use for the medical professionals and decreased discomfort for the patients of the new anterograde replacement procedure of the Provox 2 prosthesis were confirmed. The device life of Provox and that of Provox 2 were comparable, despite the alterations needed to optimize the Provox 2 prosthesis for the anterograde procedure.
UNLABELLED: BACKGROUND. An anterior pharyngeal pouch is a common anatomic variation after total laryngectomy. The exact part the pouch has to play in postlaryngectomy swallowing disorders is unknown but might be bigger than expected. METHODS: Nine patients with a symptomatic pouch were treated with CO2 laser and questioned about pre- and postoperative complaints. RESULTS: Eight out of nine patients noticed an obvious improvement of their swallowing complaints after laser surgery. CONCLUSION: Based on the results, it is concluded that CO2-laser treatment with division of the bar of tissue posterior to the pouch is seen as a safe and simple way to treat patients for symptoms that can be wrongly attributed to a total laryngectomy.
BACKGROUND: Prospective evaluation of the percutaneous tracheostomy by the guide wire dilating forceps (GWDF) technique. METHODS: In 50 selected patients percutaneous tracheostomy with fiberscopic control was performed and evaluated. RESULTS: Most percutaneous tracheostomies were performed without any adverse effect. No life-threatening complications or deaths were related to the procedure. The procedure was successful in 49 of 50 patients (98%). In 1 patient the procedure was converted to an open tracheostomy because significant bleeding occurred. Five perioperative complications, including this significant bleeding and four minor complications, occurred in 50 patients (10%). Early complications occurred in 6 of 48 patients (13%), including one significant bleeding and five minor complications. A subglottic stenosis occurred in 2 of 36 successfully decannulated patients (6%). In one case this was certainly due to prolonged endotracheal intubation. CONCLUSIONS: The GWDF technique is a safe and efficient bedside alternative to open tracheostomy. Fiberscopic control is recommended to increase the safety of the procedure. Although studies of late complications are necessary, it appears to be justifiable to consider percutaneous tracheostomy for patients who require tracheostomy.
BACKGROUND AND PURPOSE: A better understanding of the vascular architecture and the microenvironmental parameters (VAMP) will allow the identification of tumours that can be more effectively treated by intensified fractionated radiotherapy or modifiers of blood flow and oxygenation or combinations of these approaches. MATERIALS AND METHODS: Proliferation (BrdUrd), vascular architecture (endothelial marker), perfusion (Hoechst 33342) and oxygenation (NITP) were studied in two human laryngeal squamous cell carcinoma tumour lines grown as xenografts in nude mice. The effects of carbogen and nicotinamide on these parameters were evaluated. RESULTS: Carbogen treatment resulted in a decrease of the number of perfused blood vessels from 66% to 55% in one of the two tumour lines. In this tumour line nicotinamide prevented this reduction of tumour blood flow by carbogen. In both tumour lines the labelling index (LI) decreased after treatment with carbogen for 1 h, from 11-13% to 5-7%. Both tumour lines showed a drastic reduction of hypoxia by carbogen alone or by carbogen plus nicotinamide. CONCLUSIONS: In both laryngeal squamous cell carcinoma xenograft tumour lines carbogen was very effective in reducing diffusion limited hypoxia. Only in one of the two tested tumour lines carbogen also caused a reduction of tumour blood perfusion, which could be compensated for by nicotinamide. In addition, carbogen reduced tumour cell proliferation. The fact that differences in response to nicotinamide and carbogen were observed and that they can be studied in vivo provides a basis for further development of a 'predictive profile' which will guide the clinician to select the optimal treatment for individual patients or groups of patients.
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We present a full-term newborn infant who suffered from immediate postpartum severe respiratory distress. The infant had an inspiratory stridor as a result of a swelling of the soft palate, extending from the roof of the nasopharynx. Transoral endotracheal intubation resulted in normal saturation levels. Histologic examination after an open biopsy showed mature neuroglial tissue. Radiology demonstrated the presence of a right parapharyngeal process obstructing the nasopharynx and oropharynx and extending to the right middle and posterior fossa, via the foramen ovale. After transoral debulking, the infant was extubated successfully. After an uneventful period of 5 months, the patient was readmitted at our hospital for treatment of meningitis. Subsequently, the inspiratory stridor recurred, and staged surgery was performed. First, a transcranial approach was used to remove a large intradural part of the process and close the defect at Meckel's cave. Two weeks later the retro- and parapharyngeal part of the process were removed transorally. Given the site of the defect of the skull base and the intradural location of the process, the diagnosis is a transalar sphenoidal encephalocele. This is a rare type of basal encephalocele, and has never been reported in an infant nor known to present with respiratory distress. The pathogenesis, clinical presentation, pathology, and therapeutic implications of basal encephaloceles are discussed.
The manifestations of branchio-oto-renal syndrome (BOR), Treacher Collins syndrome, tricho-rhino-phalangeal syndrome, van der Woude syndrome, and Langer-Giedion syndrome are well-defined; these conditions represent clinically and genetically separate syndromes. Autosomal-dominant branchio-oto-renal syndrome comprises preauricular pits, branchial fistulas, hearing loss, and renal anomalies. However, several families have been described without one or more of these clinical findings. In some families, the phenotypic expression is limited to branchial anomalies, preauricular pits, and hearing loss, with no renal dysplasia (branchio-otic or BO syndrome). In other families, branchial and renal anomalies occur without hearing impairment. It is not known whether the variable clinical manifestations are due to the effect of a single gene or whether these represent different syndromes. We investigated BO syndrome in a large family to determine whether BOR and BO syndromes are allelic to each other. The genetic linkage analysis provides evidence that BO syndrome is not allelic to the BOR gene at 8q13.