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Biomedical subjects

Y Manach

Publications and source records attributed to Y Manach.

At least 19 recordsLinked to original sources
PubMed2003

Risk factors for thyroglossal duct remnants after Sistrunk procedure in a pediatric population.

To evaluate recurrence after surgery for thyroglossal duct cyst (TDC) we performed a retrospective chart review. Seventy four patients between 0.5 and 8.5 years of age presenting with a midline neck cyst underwent a Sistrunk procedure for a preoperative diagnosis of TDC. Fifty-seven had histologically confirmed TDC (mean age of the population: 4+/-1.5 years, mean follow-up: 6 years and 8 months). Recurrence occurred in 15% of the cases of histologically confirmed TDC. Four individual risk factors have been identified: number of infection before surgery [more than 2 episodes (P<0.05)]; preliminary surgical procedure (P<0.05); age [less than 2 years (P<0.05)] and multicystic lesion on histopathology (P<0.01). The two first factors being correlated, the risk of relapse might be lowered by a wide excision performed before any infection in children over 2 years.

Child, Preschool↗
PubMed2001

Temporal bone anomaly proposed as a major criteria for diagnosis of CHARGE syndrome.

The acronym CHARGE defines a non-random clustering of congenital malformations of unknown origin. Classical diagnostic criteria include: 1) one major feature namely coloboma/microphthalmia or choanal atresia, and 2) four of the six features designated in the CHARGE acronym. Interestingly, all CHARGE patients hitherto reported had partial or complete semicircular canal hypoplasia on temporal bone CT-scan. We report on semicircular canal agenesis/hypoplasia in three patients with three to four features of the CHARGE syndrome and neither coloboma nor choanal atresia and we propose to include temporal bone malformations as a major criteria for diagnosis of CHARGE syndrome.

Abnormalities, Multiple↗
PubMed2001

Conservative management of Gradenigo syndrome in a child.

Gradenigo syndrome consists of the association of otitis media, facial pain in regions innervated by the first and second division of trigeminal nerve and abducens nerve paralysis. It is caused by osteitis of the petrous apex (PA) and is a very rare complication of otitis media. Its treatment usually consists in mastoidectomy and antibiotics. We report a case of a 6-year-old child, which was managed medically with a positive outcome.

Abducens Nerve Diseases↗
PubMed2001

[Chronic cough related to otorhinolaryngologic disease].

Otorhinolaryngologic etiologies of chronic coughing may be a disturbance of the pharyngo-eosphageal tract or a laryngeal failure such as a palsy or cleft. In children, chronic coughing associated with chronic rhinosinusitis looks much more like a tracheobronchial manifestation of a global respiratory disease than a consequence of upper respiratory tract inflammation.

Adolescent↗
PubMed2001

Pierre Robin sequence: a series of 117 consecutive cases.

A series of 117 cases of Pierre Robin Sequence are classified as isolated (48%), syndromic (35%), and with associated anomalies (17%); the latter group had a poor long-term prognosis. In isolated Pierre Robin Sequence, familial cases and a high incidence of twins were noted. Among syndromic Pierre Robin Sequence, 4 syndromes represent more than 50% of the diagnoses.

Child, Preschool↗
PubMed2001

Hypertelorism-Microtia-Clefting syndrome (Bixler syndrome): report of two unrelated cases.

The association of Hypertelorism, Microtia and Cleft lip and palate (HMC syndrome, MIM 239800) is a rare condition of autosomal recessive inheritance. A total of seven cases of HMC syndrome in five families have been hitherto reported. Here, we report two unrelated cases and put emphasis on the possible normal psychomotor development in this syndrome.

Child, Preschool↗
PubMed1999

Vestibular function in children with the CHARGE association.

BACKGROUND: Histopathological examinations and computed tomographic scans of the temporal bone in patients with the CHARGE association (a malformative syndrome that includes coloboma, heart disease, choanal atresia, retarded development, genital hypoplasia, and ear anomalies, including hypoplasia of the external ear and hearing loss) have shown an absence of semicircular canals and a Mondini form of cochlear dysplasia. Until recently, no information was available concerning a possible loss of vestibular function, which could be a factor in retarded posturomotor development. To our knowledge, this is the first report of otolith tests done on patients with the CHARGE association. OBJECTIVE: To test residual vestibular function in patients with the CHARGE association. STUDY DESIGN: In 7 patients with the CHARGE association, we made electro-oculographic recordings of vestibulo-ocular responses to earth-vertical and off-vertical axis rotations to evaluate the function of the canal and the otolith-vestibular systems. RESULTS: None of the 7 patients had semicircular canals in the computed tomographic scan, and none had canal vestibulo-ocular responses to earth-vertical axis rotation, but all had normal otolith vestibulo-ocular responses to the off-vertical axis rotation test. CONCLUSIONS: These results support the hypothesis of a residual functional otolith organ in the hypoplastic posterior labyrinth of children with the CHARGE association. The severe delays in psychomotor development presented by these children are more likely a consequence of multiple factors: canal vestibular deficit, visual impairment, and environmental conditions (long hospital stays and breathing and feeding problems). The remaining sensitivity of the otolith system to gravity and linear acceleration forces in these children could be exploited in early education programs to improve their posturomotor development.

Abnormalities, Multiple↗
PubMed1998

Use of the subgaleal fascia to construct the auditory canal in microtia associated with aural atresia. A preliminary report.

In this paper we introduce the use of a subgaleal flap to construct the auditory canal in cases of microtia associated with aural atresia. The anatomy, the vascularisation, and the nomenclature of the different planes that constitute the temporal region are wellknown. The subgaleal fascia, also referred to as the loose areolar fascia has been until now the less surgically exploited of these planes. Nevertheless, it has a structure and a vascularisation that enables it to be dissected and used surgically. The subgaleal flap used to line the neoauditory canal drilled into the bone provides a vascular bed that greatly improves the quality of the skin graft and supports the tympanoplasty. The procedure is done while the ear is being raised, which constitutes the second stage of the two-stage ear reconstruction. This new approach has been used on 22 ears. A preliminary analysis of the results shows that construction of the auditory canal has been improved.

Adolescent↗
PubMed1997

[Functional surgery of major aplasia of the ear. Current status].

One of the challenges of functional surgery for microtia is to prevent the postoperative otorrhea. The subgaleal flap or the galeal flap, which are the two components of the superficial temporalis flap as it is currently used in plastic surgery, are, each of them, convenient for covering the ear canal and give a good support for the skin grafting. The dry ear canal can fit an air conduction hearing aid.

Ear↗
PubMed1997

[Neuroglial heterotopia. Apropos of 8 cases with non-nasofrontal sites].

Neuroglial heterotopia, glioma, is an uncommon congenital nervous tissue tumor, usually found in a nasofrontal localization. About 60 cases with an atypical localization, usually in the pterygomaxillary fossa have been reported. We present 8 cases. Symptomatology followed CT-scan or MRI identified localization and was helpful in orienting diagnosis before biopsy. Exeresis was difficult, particularly in deep infratemporal localizations reaching the base of the skull. The main risk is recurrence. Different surgical approaches have been discussed and should be adapted to each case.

Choristoma↗
PubMed1997

[Endoscopic endonasal ethmoidectomy in severe sinusitis of cystic fibrosis. Mid-term results in 12 patients].

UNLABELLED: Sinusitis, with sometimes severe symptoms, are frequent in cystic fibrosis (CF). The aim of this mild-term retrospective study was to evaluate the functional and anatomical results of functional endoscopic ethmoidectomy (FEE) in 12 patients with CF. Nasal polyposis (9 cases) and medialization of the nasomaxillary wall (3 cases) were detected during endoscopic examination. Bilateral and multifocal opacities of the paranasal sinuses were present on the CT-scan images in all cases. A FEE was performed in 11 cases and in 1 case, only the anterior ethmoid was opened. Symptoms improved or disappeared in 9/12 cases between 1 and 3 years of follow-up and in 5/7 cases after 3 years of follow-up. A good or mild anatomical result was recorded in 6/12 cases between 1 and 3 years of follow-up and in 5/7 cases after 3 years of follow-up. During follow-up, a new surgical procedure (limited in 8 patients and complete in 3 patients) was often necessary. CONCLUSION: It is reasonable to propose FEE in CF patients with chronic sinusitis presenting severe symptoms with the aim of a substantial functional but not anatomical improvement.

Adolescent↗
PubMed1995

[Preoperative X-ray computed tomographic evaluation of major aplasia of the ear in children].

PURPOSE: To describe computed tomography (CT) findings in congenital aural atresia (CAA) and to illustrate the impact of these findings in the preoperative evaluation. MATERIALS AND METHODS: Sixty-seven congenital aural atresia (bilateral: 10) in 57 children were studied using high resolution CT. Sections 1.5 mm thick were removed from the coronal and the axial plans without sedation (mean age: 9, 6 years). RESULTS AND DISCUSSION: A narrow bony external auditory canal (EAC) was present in 24% of the cases. In one of these cases, the EAC contained a cholesteatoma and was consequently a clear indication of surgery. An hyperpneumatized mastoïd (22%), a posterior position of the temporo mandibular joint (16%), and a bulging medial temporal fossa (12%) made the operation much more difficult. The tympanic cavity was small in 68% of the cases, normal in 28% and absent in 4% of the cases without any detectable ossicular chain. Ossicular chain anomalies were present in 91% of the cases. This consisted most frequently of a fusion of the malleus and the incus (76%) with or without fusion to the tympanic wall (33%). Lateral and anterior displacement of the descending portion of the facial nerve was present in 62%. Oval and round windows were normal in 86% of the cases. A soft tissue opacity (33%) in the tympanic cavity made it difficult to evaluate the stapes, the incudo stapedial articulation, and the facial nerve. Simultaneous abnormalities of inner ear were exceptional (1 case). CONCLUSION: High resolution CT is the best method in CAA evaluation and for guiding the planning of the surgical correction.

Child↗
PubMed1995

Endoscopic and CT-scan evaluation of rhinosinusitis in cystic fibrosis.

In order to obtain an accurate evaluation of nasal and paranasal sinus disease in cystic fibrosis patients, 75 patients with a proven cystic fibrosis diagnosis have been investigated using the following standard techniques: questionnaire, ENT examination, endoscopical examination, sinus X-rays, and in 31% of the cases a CT scan. The analysis of results shows that nasal obstruction is the most frequent symptom (32%) and that nasal polyps are present in 43% of the cases. Endoscopic examination seems to be of great interest in giving a more accurate picture of nasal and paranasal sinus disease. Analysis of CT scan images leads the authors to describe a new and specific entity in nasal and paranasal sinus disease in cystic fibrosis patients: the pseudomucocele. In this report, the authors discuss the various aspects of pseudomucocele, attaching particular importance to the CT scan results.

Adolescent↗
PubMed1994

Apparent genetic homogeneity of the Treacher Collins-Franceschetti syndrome.

The Treacher Collins-Franceschetti syndrome (TCOF) or mandibulofacial dysostosis (MFD) is an autosomal dominant disorder characterized by craniofacial abnormalities and hearing loss. A refined genetic linkage map of the TCOF locus was established in 8 independent families, using 12 microsatellite DNA markers of the distal 5q. Positive lod score values were obtained for all markers with a maximum at the D5S413 locus (Zmax = 3.79 at theta = 0%). Multipoint linkage analysis and haplotype analysis supported the location of the gene between loci D5S434 and D5S412. These results are consistent with previous linkage analyses [Dixon et al.: Am J Hum Genet 49:17-22, 1991, Am J Hum Genet 52:907-914, 1993; Jabs et al.: Genomics 11:193-198, 1991, Genomics 18:7-13, 1993] and provide further evidence of genetic homogeneity in this syndrome.

Chromosomes, Human, Pair 5↗
PubMed1991

[Pierre Robin syndrome].

During the last few years, our knowledge of Pierre Robin syndrome has benefited from advances in embryonic neurobiology. It has been shown that the syndrome is related to an anomalous development of the foetal brainstem. A multidisciplinary study makes it possible to value prospectively the various anomalies with which it may be associated and to decide on a coherent treatment. This type of management should reduce the mortality and prevent the sequelae of Pierre Robin syndrome.

Humans↗
PubMed1991

Lingual localization of an inclusion body fibromatosis (Reye's tumor).

This report illustrates a lingual localization of an inclusion body fibromatosis, the so-called Reye tumor or infantile digital fibromatosis (IDF). The light microscopic features were identical to those found in IDF, showing eosinophilic perinuclear inclusions located in spindle-shaped cells arranged in interlacing fascicles. The immunocytochemical and ultrastructural findings suggested a fibroblastic and/or myofibroblastic nature of the proliferative cells. However, the inclusions in our case were strongly stained with vimentin and their ultrastructural appearance was in keeping with intermediate filaments. These findings have never been described in other reports of fibromatosis. Whereas most reviews state that IDF occurs exclusively on the digits, this unique case describes its possible occurrence in the tongue.

Fibroma↗
PubMed1991

[Bone anchored hearing aid. Results of bilateral applications].

In our experience B.A.H.A. (Bone Anchored Hearing Aid) with its direct bone conduction brings a better hearing rehabilitation for patients with draining ear and further more with major aplasia. 23 patients were tested, 12 have got a bilateral hearing aid, 5 unilateral. They reported a better comfort, a more natural sound and a better discrimination in noise. A bilateral application brought them a stereoacousy with a special directivity, we also note improvement of hearing by different hearing tests.

Audiometry↗