Development of an osteosarcoma following dental extraction after allogeneic stem cell transplantation.

OBJECTIVES: Radio-induced sarcoma is known to occur several years following bone irradiation especially when this treatment is combined to high dose chemotherapy regimens prior to allogeneic haematopoietic stem cell transplantation (HSCT) in very young children. However, little is known about the stimulus of aggressive bony surgery in the development of these tumours. MATERIAL AND METHODS: We report the case of a young girl in whom dental extraction was rapidly followed by the occurrence of a localized tumour 11 years after allogeneic haematopoietic stem cell transplantation using total body irradiation (TBI) for a haemophagocytic lymphophistiocytosis (HLH). RESULTS: This tumour involved tooth socket and all the right side of the mandible and was diagnosed as an osteogenic osteosarcoma of the zygomatic bone. CONCLUSION: This tumour had the characteristics of a radio-induced sarcoma. Thanks to the very short time between the dental extraction and the occurrence of the osteosarcoma at the same location, we discuss the role of the dental extraction as a trigger of osteosarcoma development.

Unusual presentation of a first Branchial cleft cyst associated with an abnormal bony canal -a case report.

BACKGROUND: First branchial cleft anomalies are rare, accounting for only 10% of all branchial cleft anomalies. We report an even more rare and unique case of a branchial cleft cyst with features of both first and second arch derivatives. CASE PRESENTATION: A 6-year-old boy presented to us with a left conductive hearing loss associated with pre-tympanic keratin debris and an ipsilateral painful cervical mass. He had a past medical history of left ear surgery for presumed cholesteatoma 2 years prior and left neck abscess drainage 6 months prior. CT and MRI revealed a lesion originating in the external auditory canal and extending cervically through a bony canal located medial to the facial nerve and terminating as a parapharyngeal cyst. The complete removal was accomplished in one surgical stage consisting of three distinct steps: robotic assisted transoral resection of the pharyngeal cyst, an endaural approach and a parotidectomy approach. CONCLUSION: We believe that our detailed description of this rare first branchial cleft cyst with pharyngeal extension, possibly a hybrid case between a first and second branchial cyst, can serve as a valuable tool to Otolaryngologists - Head and Neck Surgeons who come across a similar unusual presentations.

Mid-term rhinosinusal consequences of bony orbital decompression in Graves' disease: a retrospective study.

OBJECTIVES: The aim of this work was to detect and study mid-term rhinosinusal complications arising after bony orbital decompression via the inferior palpebral approach in patients with Graves' ophthalmopathy. MATERIALS AND METHODS: This retrospective study considered 48 patients treated for proptosis from 1999 to 2001 who underwent bony orbital decompression via the inferior palpebral approach. Nineteen (19) of these patients agreed to participate further by consenting to follow-up examinations performed specifically for the purpose of this study, which consisted of ophthalmologic (visual acuity, proptosis) and ENT (nasal dysfunction, rhinoscopy) examinations. RESULTS: Mean mid-term postoperative follow-up of the 19 patients was 43.5 months (+/-12 months). Predominant rhinosinusal signs (rhinorrhea, altered sense of smell, nasal obstruction) were observed in 20% of this group (4 patients) without any unfavorable effect on daily life. Fiber-optic rhinoscopy disclosed mucosal secretions and edema in 5% (one patient). CONCLUSION: Mid-term complications of bony orbital decompression via an inferomedial approach are relatively rare. Late postoperative sinusitis has not been reported. Signs of minor nasal dysfunction may occur but would have little impact on quality of life.

Leukocyte compartments in the nasal secretion medium.

Nasal secretions are known to play a role in respiratory tract and host defense. Besides the mucociliary transport and biochemical properties of the mucus, we hypothesize the role of a secretory leukocyte compartment. We designed a time-series study to count leukocytes in baseline, physically- and pharmacologically-induced secretions. Twenty-three healthy volunteers and 29 patients participated in the study. In healthy subjects, secretion weights significantly increased from 24 +/- 5 mg (mean +/- SEM) at baseline to 35 +/- 6 mg and 115 +/- 12 mg, respectively, in physically and methacholine-induced secretions (p < 0.05). The leukocyte count did not change between baseline (14,445 +/- 5,010) and physically induced secretions (13,396 +/- 6,8401), but significantly increased after methacholine (28,140 +/- 11,411; p = 0.02). Leukocyte differential counts showed, moreover, an increase of lymphocytes in the methacholine-induced compartment. In patients, secretion weights significantly increased from 70 +/- 12 mg at baseline to 117 +/- 22 mg and 223 +/- 28 mg, respectively. The leukocyte count significantly increased between baseline (172,109 +/- 95,890) and physically induced secretions (410,503 +/- 318,224; p = 0.02), but decreased after methacholine (112,774 +/- 54,860). These data argue for the existence of a secretory leukocyte compartment, with a subcompartment of surface and an intraglandular subcompartment of reserve, the kinetics of which are different in patients and control subjects.

Clinical factors influencing the eosinophil infiltration of nasal polyps.

AIMS AND METHODS: Our study, based on a retrospective chart analysis, was aimed 1) to describe the varying degree of eosinophil infiltration in a series of 263 adult patients operated on diffuse and bilateral nasal polyposis (NPS) after failure of medical treatment, in 15 cystic fibrosis patients with bilateral nasal polyps, and in 31 patients with chronic sinusitis without polyps (18 bilateral, 13 unilateral) 2) to search for clinical factors that might influence the degree of eosinophil infiltration. Eosinophil infiltration was expressed semi-quantativity as a percentage of inflammatory cells. RESULTS: Our study confirms that eosinophil infiltration is a striking feature of nasal polyposis. All patients with chronic sinusitis showed less than 10% eosinophils (mean +/- SEM = 2 +/- 2%) whereas 88% of patients with NPS showed more than 10% eosinophils (50 +/- 2%). Cystic fibrosis lied in between with 40% of patients showing more than 10% eosinophils. In idiopathic bilateral NPS the number of eosinophils was increased in patients with asthma (58 +/- 3%) and even more in Widal's triad (75 +/- 4%). Atopic patients did not have more eosinophils (52 +/- 5%). Patients treated with systemic steroids within two months before surgery showed decreased eosinophil infiltration (22 +/- 3% vs 50 +/- 2 for treated versus untreated) whereas patients treated with topical steroids did not (47 +/- 2%). CONCLUSIONS: Thus, a link might exist between clinical presentation and eosinophil infiltration. Chronic sinusitis and nasal polyps are probably not the same disease. Eosinophils appear as a link between nasal polyps, asthma and aspirin intolerance. Atopic status does not modify eosinophil infiltration of nasal polyps. Systemic steroids appear significantly more effective to reduce the eosinophil infiltrate than topical steroids in our selected group of operated patients.

Eosinophil count in nasal secretions of subjects with and without nasal symptoms.

The aim of this paper, based on a cross-sectional study of 129 patients with nonallergic chronic nasal symptoms and 40 healthy controls, was to examine the leucocyte differential count in nasal secretions as a diagnostic test. Nasal secretions were collected using preweighed suction glass canulas under controlled conditions (-100Pa, 30 sec). Leucocyte and differential counts were performed using a Thoma hemocytometer and on cytospin slides after May-Grünwald-Giemsa staining. The percentage of eosinophils (Eo) was significantly higher in patients (mean +/- SEM: 15.1 +/- 2.3%) than in controls (5 +/- 2.6%) (p < 0.04). Comparison of the frequency distribution of the percentage of Eo in patients and controls clearly showed a subgroup of patients presenting with nasal secretion hypereosinophilia, and allowed us to set the positivity criterion at Eo = 20%. Diurnal variations in Eo count in 11 controls and 8 patients confirmed the value of the cutoff point. In 28 patients with nasal polyposis who underwent surgery, a correlation was found between secretion and tissue eosinophelia (r = 0.58, p = 0.001). Patients with nasal secretion hypereosinophilia had no more leucocytes in their secretions than healthy controls, the increase in eosinophils being balanced by a decrease in neutrophils. In patients without hypereosinophilia, the number of leucocytes per milligram of secretion was four times higher (8672 +/- 2521) than in the controls (2020 +/- 823) (p = 0.06) (cut-off point = 2500 leu/mg). These data show that the nasal cytogram can be modified either in qualitative or quantitative way, probably depending on the underlying inflammatory process.

[Lipoma of the internal auditory canal. Report of two cases simulating acoustic neuroma]. / Lipomes du conduit auditif interne. A propos de 2 observations simulant un neurinome de l'acoustique.

We report two cases of intra-auditory canal lipoma, presenting as acoustic neuroma, observed in respectively 45 and 53 year old patients. Cerebellopontine angle lipomas are unusual lesions, more exceptional than their intracranial counter-parts. The knowledge of these tumoral lesions and of their close relationships with cranial nerves incite to perform a frozen section when imagery is evocative, to limit functional postoperative sequelae.

[Does idiopathic naso-sinusal polyposis exist in children?]. / La polypose naso-sinusienne idiopathique de l'enfant existe-t-elle?

UNLABELLED: Nasal polyposis, a rare disease in childhood, can present itself as an idiopathic disease. The aim of this study was to describe some of the clinical features of idiopathic nasal polyposis in children and to emphasize this condition as a specific clinical entity. POPULATION AND METHODS: The study was based on a retrospective analysis of 26 cases of idiopathic nasal polyposis that were studied to our department between 1979 and 1996. In addition, a questionnaire was sent to parents of which 20 were returned completed. RESULTS: The clinical characteristics of idiopathic nasal polyposis in children (11 males and nine females; median: 12 years; range: 8-15 years) were very similar to those observed in adults (median: 50 years), in particular the relation to asthma (ten of 20 cases) and aspirin intolerance (two Fernand-Widal syndromes of 20 cases). Recurrent ENT infections during the maturing period of the immunological system (0-7 years) did not seem to play a role. There appeared, however, to be a strong genetic component as half of our cases had a family history of nasal polyposis and/or asthma. DISCUSSION: Idiopathic nasal polyposis should be recognized as an entity among nasal polyps in childhood. Management of idiopathic and secondary nasal polyposis is however different. Although the pathogenesis of nasal polyposis is not well understood, the study of this disease in children leads one to suspect a genetic transmission.

[Minor functional disorders after surgery of acoustic nerve neuroma]. / Les troubles fonctionnels mineurs après chirurgie du neurinome du nerf acoustique.

UNLABELLED: Acoustic neuromas must be removed surgically, otherwise serious complications may occur. Removal of an acoustic neuroma most often leads to disorders of balance, hearing, and sometimes facial motor function. Many earlier studies have approached the hearing and facial motor function. However, the facial nerve also comprises sensory and secretory fibers which, when injured, may produce minor disorders affecting patient's quality of life. The purpose of this work was to evaluate the quality of life, secretory and taste disorders after acoustic neuroma surgery. PATIENTS AND METHOD: Among patients operated on between May 1985 and May 1996, we selected only those who presented a normal facial function 3 months after surgery. We thus selected 93 patients and sent them a questionnaire in December 1997. We received 74 answers. RESULTS: The period between the operation and the dispatch of the questionnaire was on an average 5.5 years (range 19 months-12 years 9 months). Impairment of quality of life was frequent (59% of cases), and changing way of life less so (30%). 74% of the patients described balance disorders. One-quarter of the patients indicated they experienced disorders of facial mobility. Only 3 patients had no disorder. CONCLUSION: Secretory and taste disorders are frequent after neuroma surgery. Despite their mildness, patients should be advised accordingly because of possible impact on quality of life. This study also points out the lack of precision in the estimation of facial mobility. A better evaluation of patient complaints would be useful.

[Anterior frontal laryngectomy without epiglottoplasty or modified Tucker's technique. Proposal for a surgical simplification]. / La laryngectomie frontale antérieure sans épiglottoplastie ou "Tucker modifié". Proposition pour une simplification chirurgicale.

By replacing epiglottic reconstruction with a muscular translation, we attempted to simplify the near total laryngectomy with epiglottic reconstruction and improve swallowing disorders. Ten operated patients were followed up for at least 9 to 22 months. We obtained 9 good results and 1 failure. The long terms results have to be evaluated to assess risk of laryngeal stenosis.

[Mechanical sutures in ORL cancers, value, indications, techniques and results apropos of 30 cases]. / Les sutures mécaniques en carcinologie O.R.L., intérêt, indications, techniques, résultats à propos de 30 cas.

The authors report their experience in closure of the pharynx following total laryngectomy or total pharyngolaryngectomy by mean of the TA 55 instrument. The technique is described stressing the particular point of good presentation of the mucosa and the importance of the crossing of the lines of staples when two clips are used. An analysis of 30 cases studies is presented with the results as of the date of removal of the nasogastric catheter and the percentage of fistula. This technique appears to be rapid, safer than conventional methods of closure and less complications have been encountered in this series.

[Nasal dermal sinus in children: a review based on a series of 6 cases]. / Fistules du dorsum nasal de l'enfant: mise au point à propos d'une série de 6 cas.

INTRODUCTION: Nasal dermal sinus in children (NDSC) is a rare malformation (1/20,000 to 1/40,000). Apart from local infection, they present as median nasal lump or pit on the dorsum and their diagnosis and treatment are often delayed. Consequences of untreated NDSC are: local infection, meningitis, and empyema, due to their frequent intracranial extension. PATIENTS AND METHODS: Six cases of NDSC were retrospectively reviewed (5 boys and one girl), all treated between 2006 and 2012 in our institution (Nancy University Hospital). All patients underwent a brain and facial CT-scan and MR imaging to check any bony lesions, skull base extension by foramen cæcum, course of the sinus and the possible associated brain malformations. Evolution, treatment and follow-up (FU) were conducted by the same multidisciplinary team (neurosurgeon, ENT surgeon, and plastic surgeon). Children were operated on by a conjoined approach (cranial and facial) for removal of the sinus and its intracranial extension. RESULTS: Mean age at diagnosis was 12 months (birth-36 months). Initial presentation consisted of three local infections, one dorsum nasal lump, one CSF leakage, and one asymptomatic child. Five children presented with a skull base extension. There were no associated brain malformations. We observed only one surgical complication (bleeding from the anterior part of the superior sagittal sinus during dissection) leading to blood transfusion. Pathology results confirmed three dermoid cysts, one epidermoid cyst, one cyst with granulation tissue, and negative in one case. Average FU was 30.8 months (4-84 months). Two recurrences (same child) occurred, leading to two re-operations. There were no recurrences or complications at the end of FU. CONCLUSION: NDSC are rare malformations, mostly diagnosed before the age of three years, due to an infectious complication. The aim of the treatment is complete removal to avoid recurrence, and a multidisciplinary strategy is required.

[Avatars in face transplants]. / Les avatars de la transplantation à la face.

Avatar carries a pejorative connotation often related to an unfortunate hazard. In face transplants, incarnations were numerous. The analysis of their evolution through time and increasing sophistication of procedures turn out to be informative regarding the wide disrepairs in the craniofacial area. The authors report the principal constraints of face transplant and the evolution in minds to deal with it.

Fortuitous FISH diagnosis of an interstitial microdeletion (5)(q31.1q31.2) in a girl suspected to present a cri-du-chat syndrome.

Constitutional interstitial deletions of 5q are relatively rare and most are poorly characterized cytogenetically. Consequently a definite karyotype-phenotype correlation is difficult to establish. We report on a new case of a girl presenting with an abnormal cry, upslanting palpebral fissures, hypertelorism, anteverted nostrils, microretrognathia, growth retardation, and an adenoid cyst at the base of the tongue. The first suspected diagnosis was cri-du-chat syndrome because of the mewing cry. Standard cytogenetic analyses were interpreted as normal, but FISH studies using the probe of cri-du-chat syndrome with the control probe EGR1 (5q31.2)/D5S23 (Abbott) revealed a 5q31.2 microdeletion which was then confirmed by CGH-array (Abbott). FISH studies using PACs and BACs clones (Rocchi, Italia) enabled us to characterize the breakpoints of the deleted region. Cytogenetic analysis with FISH studies revealed a normal karyotype with normal 5q31 region in both parents. This case is compared with the other cases reported in the literature.

[Prosthesis fitting after maxillectomy: an indispensable factor in acceptance and rehabilitation]. / Appareillage après maxillectomie: indispensable facteur d'acceptation et de réinsertion.

Surgical resection is often required for maxillary cancer, producing a communication between the oral cavity and the nose or paranasal sinuses. After maxillectomy, patients experience major dysfunction in speech and swallowing which have a very negative psychological effect. These problems can be overcome with an immediate prosthesis but to be fully successful, coordinated work is required between the surgeon and the maxillofacial prosthodontist before, during and after surgery. Ten days after surgery, an interim obturator is made with soft lining materials. This prosthesis will evolve as scar tissue forms, preparing space for the definitive obturator which will restore good quality of life for maxillectomy patients.

[Osteosynthesis using a screwed plate in fractures of the mandibular condyle]. / L'ostéosynthèse par plaque vissée dans les fractures du condyle mandibulaire.

The surgical treatment of sub-condylar fractures allows rapid reestablishment of the bite, temporo-mandibular joint function and facial appearance. A pre-auricular approach is used involving dissection of the facial nerve and its upper branch. Surgical reduction is performed under direct vision. Fixation is via a plate with 4 screws. The condylar fragment needs to be large enough to accept at least two screws and hence this needs to be confirmed preoperatively by CT scan examination. In the adult we feel that fixation is indicated for low displaced sub-condylar fractures. We do not feel that surgery is indicated for recent trauma in children.