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Biomedical subjects

E N Garabedian

Publications and source records attributed to E N Garabedian.

At least 19 recordsLinked to original sources

[Episodic laryngeal dysfunction].

Episodic laryngeal dysfunction (ELD), also known as paradoxical vocal cord dysfunction or laryngeal dyskinesia, is characterized by abnormal closure of the vocal cords during inspiration (and sometimes at the very start of expiration). It can manifest in different ways depending on the patient's age. In the newborn, it is usually associated with stridor restricted to the inspiratory breath following crying, and the natural history is always one of rapid resolution. Occasionally, a more severe form presents with intense dyspnea and marked inspiratory stridor in a baby that usually has extensive gastroesophageal reflux which has not been treated adequately enough to improve the dyspnea or the associated vasovagal attacks. A tracheotomy may be necessary. It is rare for this disease to present between the ages of 2 and 8 years. Thereafter, it may present as a form of pseudo asthma resistant to bronchodilators and anti-inflammatory drugs. The dyspnea can be very severe and lead the family to seek hospital admission, at least during the initial episodes. There is a female preponderance. The key to making the diagnosis is the complete reversibility of the patient's symptoms when they are distracted. Exertion asthma can be mimicked by forms of ELD that occur only by effort (apart from the profile of the lung function tests). There may be significant gastroesophageal reflux. Medical intervention (hospitalization and tracheotomy) must be avoided, treatment being essentially behavioral. Finally, some cases of paradoxical adduction of the vocal cords have been described with the use of neuroleptics, brain stem compression, cortical lesions and, much more rarely, motoneuron disease. This diagnosis requires a high index of suspicion, particularly in patients with asthma whose presentation or clinical course with treatment is atypical.

Age Factors↗

Audiophonological results after cochlear implantation in 40 congenitally deaf patients: preliminary results.

The aim of this study is to evaluate the prognostic factors of audiophonological results in cochlear implant in congenitally deaf patients. Between 1991 and 1996. 40 congenitally deaf children underwent cochlear implantation in our department, at an average age of 7 years (median: 5 years). The results of speech therapy were evaluated with a mean follow-up of 2 years and were classified according to four criteria: perception of sound, speech perception, speech production and the level of oral language. For each criterion, a score was established ranging from zero to four. These scores were weighted according to age such that the results before and after implantation only reflected the changes related to the implantation. The prognostic factors for good results were: a good level of oral communication before implantation, residual hearing, progressive deafness and implantation at a young age. On the other hand, poor prognostic factors were: the presence of behavioral disorders and poor communication skills prior to implantation. Overall, the major prognostic factor for a good outcome appeared to be the preoperative level of oral language, even if this was rudimentary.

Adolescent↗

Extensive lymphangioma presenting with upper airway obstruction.

OBJECTIVE: To describe the results of an outcome survey of 18 cases of pediatric lymphangioma with dyspnea from encroachment on the tongue base, parapharyngeal space, and/or larynx. DESIGN: Retrospective review of files from January 1983 to September 1998. SETTING: Pediatric otolaryngology departments from 2 referral centers. PATIENTS: Eighteen patients were treated. The average age at initial surgery was 22 weeks (median, 5 weeks). All presented with at least unilateral suprahyoid and infrahyoid cavernous (microcystic) lymphangioma. The tongue base was involved in 11 patients, the parapharyngeal space in 12, and the larynx in 8. INTERVENTIONS: Neck dissection was performed initially in all patients. Tracheotomy was performed in 9 patients (50%). Macroglossia was treated by V glossoplasty. Parapharyngeal extensions were treated by cervicotomy or endoscopy, and larynx and tongue base extensions by carbon dioxide laser photocoagulation. Supraglottic laryngectomy was performed in 2 patients. MAIN OUTCOME MEASURES: Residual disease, decannulation, duration of tracheotomy, and persistent respiratory symptoms. RESULTS: The average follow-up was 4 years postoperatively. One postoperative death occurred. Sixteen (94%) of the remaining 17 patients had residual lymphangioma. Eight (89%) of the 9 patients with tracheotomy underwent decannulation (average duration, 22 months). Ten patients had persistent symptoms, and 6 were asymptomatic. CONCLUSIONS: Involvement of the upper airway seems to be the determining prognostic factor in extensive lymphangioma. Patients with dyspnea by external compression of cervical lymphangioma on the airway responded well to surgery. Aggressive surgical treatment did not seem to significantly improve the prognosis in patients with intrinsic involvement of the upper airway. The natural evolution of untreated massive lymphangioma has not been documented. Less aggressive, symptomatic therapy may be an alternative to avoid mutilating surgery in patients with intrinsic involvement of the airway.

Airway Obstruction↗

Head and neck infantile myofibromatosis--a report of three cases.

Infantile myofibromatosis (IM) is a proliferative disorder characterized by the development of single or multiple nodular lesions arising from cutaneous, subcutaneous, muscular, bone or visceral structures. This proliferation may occur at any anatomical site, but in 30% of the cases it involves the head and neck. We report here three cases of head and neck IM occurring in young children and presenting as solitary lesions. The clinical heterogeneity and the misleading histopathological appearances can make the diagnosis difficult. The treatment is surgical but the low rate of recurrence and the possibility of spontaneous tumoral regression may lead to conservative surgery or therapeutic abstention.

Child↗

Congenital tracheoesophageal fistula without esophageal atresia.

The authors report a series of eight cases of isolated tracheoesophageal fistula without esophageal atresia (or an H type fistula), treated in three pediatric ENT departments. This is a rare malformation whose diagnosis requires investigation for associated anomalies. The clinical signs are mainly respiratory but also digestive and the symptomatology can be severe. The diagnosis can be made with a barium swallow combined with cineradiography, but a tracheoesophageal endoscopy remains the investigation of choice. The treatment is surgical. In most cases, the fistula is accessible by a right or left cervicotomy, depending on the surgeon's practice, with a much lower postoperative morbidity as compared to a thoracotomy. The postoperative management was straightforward in most of our cases. We discuss the role of gastro-esophageal reflux with respect to postoperative morbidity as well as systematic treatment for reflux peri-operatively. The pros and cons of the various surgical approaches are also discussed.

Barium Radioisotopes↗

The CHARGE association: the role of tracheotomy.

OBJECTIVES: To evaluate the need for a tracheotomy and its timing during the evolution of an association of malformations, including coloboma, heart defects, choanal atresia, developmental and growth retardation, genitourinary malformation, and ear anomalies (CHARGE association). DESIGN: Retrospective study from January 1988 through December 1997. SETTING: Four academic tertiary care centers. PATIENTS AND METHODS: Forty-five patients with CHARGE association having at least 3 cardinal malformations (growth retardation excluded) and review of the malformations and respiratory manifestations encountered. All the patients underwent endoscopic exploration on several occasions. We reviewed the nature and the timing of therapeutic interventions performed on the airway. RESULTS: Two patients died (one patient of septicemia, the other of unknown causes). Abnormalities of blood gas levels and/or sleep were found in 30 patients (67%), were responsible for cardiorespiratory arrest in 9 (20%), and required admission to the intensive care unit in 21 (47%). Pharyngolaryngeal anomalies leading to dyspnea (discoordinate pharyngolaryngomalacia, glossoptosis, retrognathia, laryngeal paralysis, cleft, stenosis, and difficult intubation) were found in 26 patients (58%). Tracheobronchial anomalies (esophagotracheal fistula, esophageal atresia, and tracheomalacia) were present in 18 patients (40%). Resection of the aryepiglottic folds was attempted 3 times, but without success. Tracheotomy was necessary in 13 patients (29%) at a median age of 2.4 months (mean duration, 25 months). Among these infants, the posterior nasal choanae were patent in 10 patients at the time of tracheotomy. Gastroesophageal reflux was encountered in 36 patients (80%). Prolonged enteral feeding was necessary in 21 patients (47%), with gastrostomy in 16 (of whom 9 needed a tracheotomy). These feeding difficulties and airway problems were highly correlated. CONCLUSIONS: We encountered multiple, complicated airway abnormalities. Resection of aryepiglottic folds was inadequate. Often, a tracheotomy could not be avoided in these patients, regardless of choanal patency. Tracheotomy needs to be performed early to avoid hypoxic events. In some selected patients, ventilation using bilevel positive airway pressure may be an alternative.

Abnormalities, Multiple↗

Myringoplasty in children: predictive factors of outcome.

OBJECTIVES: To assess the results of myringoplasty in children and to determine which factors independently influence the postoperative results. STUDY DESIGN: Retrospective study of the anatomic and functional results of 231 consecutive myringoplasties performed in 188 children between 1988 and 1992. Multivariate analysis of poor prognostic factors by cross-sectional comparison 1 year after surgery. METHODS: Myringoplasties were performed via an endaural approach with a fascia temporalis underlay graft. RESULTS: In 216 of 231 ears (93.5%) the tympanic membrane was closed. A good anatomic outcome was considered to have been achieved in 188 ears (81.6%), although in 18 ears (7.8%) seromucous otitis media occurred, in 8 ears (3.5%) a progressive retraction pocket was encountered, and in 2 ears significant lateralization was present. One hundred thirty-nine (67.5%) of the 206 ears tested in the postoperative period had a postoperative air-bone gap of 10 dB or less. On average, mean bone conduction remained unaltered. The age of the patient and the size and the location of the perforation did not affect the outcome. Three prognostic factors for an abnormal postoperative tympanic membrane were found, with 95% confidence intervals: inflammatory changes in the middle ear mucosa (P < .05), contralateral tympanic perforation (P < .05), and contralateral cholesteatoma (P < .01). CONCLUSIONS: Myringoplasty with underlay grafting of the fascia temporalis in children gives good anatomic and functional results. Inflammatory changes within the middle ear mucosa, contralateral tympanic perforation, and contralateral cholesteatoma independently influence the risk of an abnormal postoperative tympanic membrane. The presence of one of these factors preoperatively should lead to the consideration of alternative, more durable graft material, such as autologous cartilage.

Adolescent↗

Pharyngo-laryngeal histoplasmosis: one case in an immunocompetent child.

We report a very rare case of disseminated pharyngo-laryngeal histoplasmosis with systemic spread in a 10-year-old, immunocompetent child from Guyana. The main signs were a marked deterioration in his general condition, hepato-splenomegaly, multiple lymphadenopathy and ulcerated pharyngo-laryngeal lesions. The diagnosis was made from brushings of the ulcerative lesions, a lymph node biopsy and serological tests performed in the national reference center for histoplasmosis. The initial treatment was with amphotericin B, which was then replaced by oral itraconazole. We report here the main epidemiological, clinical and therapeutic characteristics.

Amphotericin B↗

Results of tympanoplasty in children with primary ciliary dyskinesia.

OBJECTIVE: To assess the results of tympanoplasty in children with primary ciliary dyskinesia complicated by tympanic perforation or cholesteatoma with hearing loss and/or recurrent otorrhea. DESIGN: Retrospective study. Postoperative follow-up of 26.3 months in the type 1 tympanoplasty group and 46 months in the child with cholesteatoma. SETTING: Hospitalized care, referral center. PATIENTS: Seven children with primary ciliary dyskinesia, complicated in 6 children by 9 tympanic perforations (3 bilateral perforations) and in 1 child by an attical cholesteatoma. RESULTS: After 9 type 1 tympanoplasties, the grafts were intact in 9 ears, with no recurrence of otorrhea, but serous otitis media was present in 6 of the 9 ears. Auditory improvement was significant, with an average gain of 17-dB hearing level in speech frequencies. After a canal wall-down tympanoplasty with mastoidectomy for attic cholesteatoma in 1 ear, the cavity that was operated on showed no signs of otorrhea or residual cholesteatoma after a follow-up of 46 months. CONCLUSION: In children with primary ciliary dyskinesia, tympanoplasty has a high probability of graft success and auditory improvement, despite the frequent recurrence of serous otitis media.

Adolescent↗

First branchial cleft anomalies: a study of 39 cases and a review of the literature.

OBJECTIVES: To identify the clinical and anatomical presentations and to discuss the guidelines for surgical management of anomalies of the first branchial cleft. DESIGN: Retrospective study. SETTING: Three tertiary care centers. PATIENTS: Thirty-nine patients with first branchial cleft anomalies operated on between 1980 and 1996. INTERVENTION: All patients were treated surgically. Complete removal of the lesion required superficial parotidectomy with facial nerve dissection in 36 cases. The relationship of the facial nerve and anomalies is discussed. RESULTS: Anatomically, 3 types of first branchial cleft anomalies are identified: fistulas (n=11), sinuses (n=20), and cysts (n=8). Clinically, 3 types of presentation are noted: chronic purulent drainage from the ear (n=12), periauricular swelling in the parotid area (n=18), and abscess or persistent fistula in the neck located above a horizontal plane passing through the hyoid bone (n=21). A membranous attachment between the floor of the external auditory canal and the tympanic membrane was observed in 10% of cases. The facial nerve was located lateral to the anomaly in 39% of cases. CONCLUSIONS: Before definitive surgery, many patients (n=17) underwent incision and drainage for infection owing to the difficulties in diagnosing this anomaly. Wide exposure is necessary in most cases, and a standard parotidectomy incision allows adequate exposure of the anomaly and preservation of the facial nerve. Complete removal without complications depends on a good understanding of regional embryogenesis, a knowledge of the circumstances surrounding discovery, an awareness of the different anatomical presentations, and a readiness to identify and protect the facial nerve during resection.

Adolescent↗

Second thyroid neoplasms after prophylactic cranial irradiation for acute lymphoblastic leukemia.

An understanding of the pathogenesis of second cancers may help in their prevention. We report on two children who were treated for acute lymphoblastic leukemia (ALL), with an exclusively cranial prophylactic irradiation (18 Gy) and who presented with a thyroid carcinoma (TC) 12 and 13 years later. From a thorough review of the literature of TC after ALL and of radiation-induced TC, a strong case can be made that these tumors are caused by late effects of scattered radiation. The risk is at its highest in small children. After cranial irradiation, patients require clinical monitoring of the thyroid and cervical area for nodules, continued indefinitely. We suggest that, in most cases, an alternative form of neuromeningeal prophylaxis should be offered in small children with ALL.

Adolescent↗

Posterior laryngeal clefts: preliminary report of a new surgical procedure using tibial periosteum as an interposition graft.

OBJECTIVE: To present the preliminary results of a new surgical procedure for posterior laryngeal cleft repair. DESIGN: Retrospective study in an academic tertiary care center. METHOD: The study included three male patients (age at surgery, 2, 13, and 14 mo). One presented with severe aspiration and cyanotic attacks, the two others with aspiration and recurrent chest infections. The types of laryngeal clefts included complete cleft of the cricoid with varying degrees of tracheal involvement but not further than the first six tracheal rings. Associated malformations included one VATER syndrome, one esophageal atresia, and one tracheoesophageal fistula. Surgery was performed under general anesthesia with nasotracheal intubation. A vertical anterior laryngofissure was performed. The mucosal margins of the clefts were incised and then repaired in two layers with polyglactin sutures. The original feature of this procedure was the interposition of a small piece of tibial periosteum between the two layers. This fascia graft is known to be strong and resistant in cleft palate surgery. MAIN OUTCOME MEASURE: Clinical and endoscopic follow-up was used for evaluation of results. RESULTS: The three patients had successful laryngeal repair at a mean follow-up of 6 months (range, 4-14 mo). CONCLUSION: The anterior laryngofissure provides a good surgical access to the cleft. The interposition of tibial periosteum allows durability of the cleft repair. A longer follow-up is needed to confirm these preliminary results. A computed tomography scan study and a study on the rabbit are planned in order to evaluate the outcome of these periosteal grafts.

Adolescent↗

Management of acute otitis media caused by resistant pneumococci in infants.

OBJECTIVES: To assess the clinical outcome and risk of failure after oral vs. intravenous treatment in otitis media caused by penicillin-resistant pneumococci. To determine the possible correlations between pneumococcal minimal inhibitory concentration (MIC) to penicillin and clinical outcome. DESIGN: Retrospective study of 156 cases collected between 1993 and 1995. Mean follow-up: 5 months. Setting. Two tertiary academic medical centers in Paris, France. PATIENTS AND METHODS: Pneumococcus was isolated from 191 of 570 ear samples obtained from children with otitis media and shown to be penicillin-resistant in 156. Medical history, antibiotic therapy during the previous 3 months and day-care center attendance were reviewed. For the current episode microbiologic characteristics of the isolated strains, type of treatment, therapy efficacy and clinical outcome were analyzed. Patients were predominantly young (76.3% were <1 year old) and bacteriologic samples were taken mainly because of previous treatment failure. RESULTS: Among 156 children with pneumococcal penicillin-resistant otitis media, 72.2% attended day-care centers, 71.8% had been previously treated with aminopenicillin and 52.5% with cephalosporins. Failure of previous empirical oral therapy was noted in 84% (one-third of these had been receiving amoxicillin-clavulanate). Patients treated intravenously had had a more protracted otitis but no greater number of previous episodes of acute otitis media than those receiving oral therapy. Acute mastoiditis occurred in 4 infants resulting in mastoidectomy. Oral treatment (mainly with high dose amoxicillin,120 to 150 mg/kg/day) and intravenous therapy (cephalosporin or glycopeptide) had been used in 59 and 41%, respectively. Mean duration of therapy was 10.7 days. Three failures (1.9%) and 10 recurrences (6.4%, average 28 days) occurred. No statistical difference was found between intravenous and oral therapy with respect to risk of recurrence. A high penicillin MIC value was correlated with previous antibiotic treatment but not with clinical outcome. CONCLUSIONS: Oral therapy appears to be as effective as intravenous therapy for the treatment of penicillin-resistant pneumococcal otitis media. Intravenous treatment should not necessarily be dictated by the penicillin susceptibility value but should be considered in cases of failure to thrive, persistent otitis or other complications.

Acute Disease↗

Discoordinate pharyngolaryngomalacia.

In cases of severe laryngomalacia, laser division of the aryepiglottic folds (AEFs) or endoscopic supraglottoplasty may be an ineffective solution. Failure of this technique is rare and the reasons for failure are not well established. The purpose of this study was to describe those cases of laryngomalacia in which endoscopic treatment did not reverse the clinical situation. We introduce the concept of discoordinate pharyngolaryngomalacia (DPLM). DPLM was defined as severe laryngomalacia with complete supraglottic collapse during inspiration, without shortened AEFs or redundant mucosa, and with associated pharyngomalacia. Twenty-seven of 82 children with severe laryngomalacia presented a DPLM. Endoscopic treatment was performed in 16 children and the surgical procedure was inadequate to reverse the clinical problem in these patients. In 10 children correction of additional sites of obstruction was required (uvulopharyngopalatoplasty, surgery of choanal atresia, aortopexy). Tracheostomy was necessary in 13 children. Bi-level positive airway pressure (BiPAP) was used successfully in 2 children and tracheotomy was avoided. Treatment still needs to be better defined.

Endoscopy↗