[Secondary treatment of cleft lip and palate]. / Traitement secondaire des fentes labio-palatines.

For 18 years our protocol has corrected the cleft lip nose and achieved an intravelar veloplasty at the time of the first operation, leaving the least scaring as possible. No doubt that the best treatment of the sequellae is their prevention: - the oro-nasal fistulas have disappeared; the nostril is almost normal; the continuity of a wide maxillary arch is restored in primary dentition - all that favor a nasal ventilation. This context has changed the nature of the secondary treatment described here. When lip and nose are not good enough we must address the residual deformities with the primary surgery principles. The velopharyngeal insufficiency calls for a velar re-repair and the pharyngeal depth is to be reduced by lipofilling. The rare cases of failure are improved by an Orticochea sphincteroplasty.

[Primary treatment of cleft lip and palate. Its fundamental principles]. / Traitement primaire des fentes labio-palatines. Ses grands principes.

If the multiplicity of functional protocols of cleft lip and palate treatment has been bewildering, it is now a source of learning. The lessons we can draw from them assist us to choose the best age for the primary surgery and a chronology that prevents the palate from the worst scaring. Eventually, with 18 years of follow-up, the best functional achievement comes unexpectedly from an ambitious primary rhinoplasty that had till now been condemned. Not only do the patients have good appearance and social integration, but the nasal mode of breathing established at the time of the primary surgery favors a good facial growth without any compromise. Reciprocally, all the interacting functions benefit from a nasal ventilation.

Clinicopathological study of 13 cases of squamous cell carcinoma complicating hidradenitis suppurativa.

BACKGROUND: To the best of our knowledge, only 52 cases of squamous cell carcinoma (SCC) complicating hidradenitis suppurativa (HS) have been reported since 1958. We describe 13 new cases. METHODS: We propose a clinical and histological analysis of our cases. We include these results in a review of previously reported cases to analyze a total of 65 patients. In our series of 13 cases, we also investigate the presence of human papillomavirus (HPV) in tumor samples, by polymerase chain reaction (PCR) on paraffin-embedded material. RESULTS: Malignant transformation affects mainly men with a long-term history of genitoanal HS. Although our cases were 7 well-differentiated carcinomas and 6 verrucous carcinomas, lymphatic and visceral metastasis occurred in 2 and 3 cases, respectively. With PCR, we demonstrated presence of HPV in genitoanal tumoral lesions, principally HPV-16. CONCLUSION: SCC complicating HS evolves poorly, despite a good histological prognosis. Our results sustain the implication of HPV in the malignant transformation of HS.

[Reconstruction of the nasal alar defect with the superiorly based nasolabial flap described by Burget: report of seven cases]. / Reconstruction de l'aile narinaire par lambeau nasogénien à pédicule supérieur de Burget: à propos de sept cas.

BACKGROUND: The reconstitution of a nasal alar rim and lobule defect represent a difficult challenge in consideration of his situation, anatomy and function. This article describes the technique and the interest of the nasolabial flap when used to cover the entire alar subunit. METHODS: We present 7 cases of alar rim and lobule defect after skin cancer excision. In the series, there are two full-thickness with lining defect. The patients were reconstructed with a superiorly based nasolabial flap, according to the subunit principle as introduced by Burget. A free cartilage graft was used to restore structural support with marginal skin flaps were turned over for intranasal lining when necessary. RESULTS: The cosmetic and functional outcomes of each repair were judged from good to excellent by patients and surgeons. No case of flap infection or necrosis occurred. CONCLUSION: The superiorly based nasolabial flap, describe by Burget, provides an excellent choice for cosmetic and functional reconstruction of the nasal alar defect.

[Nasal sequels of unilateral clefts: analysis and management]. / Les séquelles nasales des fentes unilatérales: analyse et prise en charge.

Usually, the nasal sequels of unilateral cleft patient are just considered as an esthetic problem to be addressed after the growth spurt of adolescence. This very narrow vision has led the cleft lip and palate treatment to a deadend. Actually, nasal sequels are the worst in terms of consequence on facial growth. 75% of complete unilateral cleft children are more oral than nasal breathers. Today, we know about the bad consequences of oral breathing on facial growth. It is not surprising to observe a high rate of small maxilla with cleft maxilla scars. In the fetus, the unilateral cleft nose deformities are well explained by the rupture of the facial envelope and the ventilatory dynamics of the amniotic fluid. Every step of the primary treatment threatens the nasal air way patency, whether when repairing lip and nose, suturing the hard palate that is the floor of the nose, or closing the alveolar cleft which controls the width of the piriform aperture. The functional and esthetic nasal sequels reflect the initial deformity, but are also the surgeon's skill and protocol choice. Before undertaking treatment, we must analyze the deformity at every level. Usually, the best option is to reopen the cleft completely to perform a combined revision of the lip, nose, and alveolar cleft after an adequate anterior maxillary expansion. If nasal breathing is necessary for an adequate facial growth, 25 years of experience showed us that it was very difficult to erase the cortical imprint of an early oral breathing pattern. So it is essential to establish a normal nasal breathing mode at the initial surgery. When the initial surgery is efficient and/or the secondary repair is successful, the final esthetic rhinoplasty, when indicated, is just performed for the sake of harmonization, with a classic internal approach and a few refinements.

[A functional approach in the primary treatment of labial-alveolar-velopalatine clefts for a minimum of sequels]. / Une approche fonctionnelle lors du traitement primaire des fentes labioalvéolopalatovélaires pour le minimum de séquelles.

Is the poor potential of growth an ineluctable consequence of mesodermal deficiency? Should we agree with the idea that all protocols are equivalent? Actually, these opinions reflect the empiricism of previous generations. We must now become rational and develop a project without compromise to achieve good functions at primary surgery. 'The normal structures are present on either side of the cleft, only modified by the fact of the cleft...' Victor Veau's hypothesis is the conclusion of rigorous anatomical and embryological research. Our current knowledge of the pathological anatomy allows for a better restoration of the normal anatomy. Anatomy is nothing if it is not functional. Every thing should be done to control the healing process to allow the best expression and interaction of the various functions, especially for those concerning nasal ventilation and masticatory efficiency. To correct the deformity, the cleft surgeon must perform a wide subperiosteal and subperichondrial elevation and must learn the skills of this accurate work to preserve the integrity of very fragile structures. The primary treatment must take into account a rational and uncompromising selection of the age of the first operation, of the successive procedures, and their chronology to benefit from the growth spurt of the maxilla, and to avoid the worse scars resulting from secondary epithelialization. Finally, if nasal breathing is the most important function concerning facial growth, it is essential to restore this normal function at the time of the first operation. The oral breathing pattern set at the time of the first operation leaves a cortical imprint that is very difficult to erase, even after clearing the nasal airways. The results of the functional approach we have used in the last decade are particularly consistent and very convincing. In this ambitious and demanding program, the patient comes first; we decrease the burden for him and his family, and give them the benefit of a good social life before school age.

[The role of the maxillary incisors in the development of the base of the nose. Applications in dento-facial orthopedics]. / Du rôle des incisives maxillaires dans le développement de la base du nez. Applications en orthopédie dento-faciale.

The neonatal respiratory distress observed in the event of a solitary median maxillary central incisor compels us to reconsider some of the traditional concepts relative to the transverse growth of the nasal level of the face. The "container-contents" connections associating maxillary incisor odontogenesis with the development of the premaxillary and facial envelopes draw the attention to the significant geometrical and mechanical expression of this morphogenesis. They require attributing to the maxillary incisors an important motor role in this development. They lead to granting the ontogenetic bonds, between malocclusions and disturbed nasal breathing, the place they deserve, taking into account the morphological integration combining them. They eventually open a new therapeutic prospect: the optimization of the development of the growing face, with regard to the various tissue mechanics and physiologies, becoming the best guarantor for the prevention of relapse after dentofacial orthopedics.

[Squamous cell carcinoma arising in Verneuil's disease: two cases and literature review]. / Dégénérescence de maladie de Verneuil en carcinome épidermoïde: à propos de 2 cas et revue de la littérature.

Verneuil's disease (hidradenitis suppurativa) is a chronic inflammatory, suppurating, fistulizing and scar-producing disease of apocrine gland-bearing skin. Its transformation into epidermoid carcimona has been reported 38 times in literature. We describe two new cases - two males aged 67 and 68-years-old. The first one is still alive with no recurrence after one year and the second patient died 2 months after surgery, showing generalised scattering. This rare complication is interesting for two reasons. It only concerns perianal location and it targets mainly men. Surgical treatment consists of wide excision. However, it is often too late. The outcomes are critical for it has been reported a 50% rate of deaths within 2 years after surgery. It is therefore imperative that both general practitioners and dermatologists follow patients with Verneuil's disease so that they can propose a preventive excision at the right time.

[Therapeutic approach to cleft lip-maxilla-palate: for normal facial growth. A protocol and various technics to restore nasal respiration]. / Approches thérapeutiques des fentes labio-maxillo-palatines: pour une croissance faciale normale. Un protocole et des techniques au service de la ventilation nasale.

By contrast with the poor maxillary growth following primary surgery in infancy, unoperated adult cleft lip and palate subjects are known to have good facial growth. There is a strong consensus to consider that scarring from primary surgery is the main cause of this problem, particularly scarring from secondary epithelialization of denuded palatal bone, or of closure of the cleft in one layer. In an attempt to improve the outcome of facial growth, a lot of protocols have developed but, currently, none of them appears more valid and the differences between them are more in favor of the personal influence of each surgeon and his team. We are not in agreement with the widely spread opinion attributing the poor results to a severe hypoplasia which could explain the cleft itself. Actually, these patients have a normal potential of growth, but they need normal functions to show it. We think that oral breathing, so frequent among these patients, is enough to explain their poor growth. Over the past 22 years, we have tried to restore, with encouraging results, a nasal breathing mode, as early as 6 years of age, through precise secondary surgery of the nostril and the septum. But with experience, we have concluded that changing the first habit of oral breathing into a nasal one is particularly difficult in cleft patients, and that a nasal mode of breathing should be established once the primary surgery, in order to avoid compensation mechanisms and their consequences. For the last 6 years, our current protocol has allowed to achieve consistently this objective, with an evident influence on the outcome of the growth of the maxilla in complete unilateral and bilateral clefts. A longer follow-up is necessary to confirm it, but henceforth, all those who know the essential role of nasal breathing for a normal facial growth should endorse this process.

[Cleft lip, maxilla and palate treatment by Dr. Talmant's team in Nantes]. / Prise en charge des fentes labio-maxillo-palatines par l'équipe du docteur Talmant à Nantes.

Over the last 30 years, our private cleft lip and palate team has developed an increasing activity based on the Victor Veau's concept: "All the structures are present and only deformed". Our goal is to achieve an anatomical and fully functional repair in every fields with the first operation. A few recent refinements have improved our primary procedures: intravelar veloplasty; simultaneous lengthening of the columella and primary lip repair in bilateral clefts; nasal retainer for the 3 or 4 first postoperative months allowing the establishment of a nasal breathing mode at once. Our timing has been the same over the last 21 years if we except that we currently perform the gingivoperiosteoplasty between 4 and 5 years of age so that the width and the relationships of the maxillary arch are normal at the time of the mixed dentition. The timing is the same in uni and bilateral clefts. No preoperative orthopedics. At 6 months of age, nasolabial repair and closure of the soft palate with intravelar veloplasty. At 18 months of age, anatomical closure of the residual cleft of the bony palate in two planes without vomer flap or denuded bone. Between 4 and 5 years of age, after a short orthopedic treatment, closure of the alveolar cleft by a gingivoperiosteoplasty with iliac bone graft. From 6 years of age we start the orthodontic treatment. The current evolution allows to think that only few late corrections will be necessary.

[Nasal respiration and recurrence]. / Ventilation nasale et récidive.

Cause and effect relationships existing between "nasal breathing impairment" and "relapse" have inherited from controversies which have animated the debate between the different schools of orthodontics for more than a century. Those differences of opinion seem to have their origin in the lack of accuracy inherent with the general acceptance of the two concepts of "normal nasal ventilation" and "relapse". Rather than retracing their history, we would like to put forward a few proposals aimed at getting rid of the doubtful trouble the present difficulty confronts us with. We have therefore endeavored: 1. to define most exactly our concepts of "optimal nasal ventilation" and "relapse"; 2. to determine, afterwards, the mechanics responsible for facial shape alterations due to nasal obstruction; 3. hence, to deduce the therapeutic behavior enabling us to master those alterations more extensively. Bearing in mind that, in order to legitimate the risk of a treatment, acknowledge its benefit and justify its cost (which may become prohibitive because of relapse), the clinician has to base his options on a body of consistent arguments.

HOE 694 affords protection versus veratrine contractures in rat atria by Na+ channel blockade.

We examined the effects of the benzoylguanidine derivative HOE 694, an inhibitor of Na(+)-H+ exchange, against veratrine-induced diastolic contractures and action potentials recorded in rat isolated left atria. Concentration-dependent protective effects against veratrine-contractures, in the absence of negative inotropic responses, were observed with HOE 694 (IC50 = 20.1(7.6-27.0) microM, n = 24) and with the chemically related amiloride derivatives DMA, EIPA, HMA and MIA, but not with amiloride itself. Concomitant Na(+)-H+ exchange blockade by a high concentration of amiloride (100 microM) failed to significantly modify the protective effects of HOE 694. HOE 694 decreased Vmax significantly at 10 microM (166.7 +/- 21 vs 154.7 +/- 20 V/s, P < 0.05, n = 6) without any effect on resting potential or action potential duration. High concentrations (100 microM) of HOE 694 further decreased Vmax and increased action potential duration. The protective effects of HOE 694 were compared with three of the class 1 antiarrhythmic agents, quinidine, lidocaine and flecainide against veratrine contracture. These Na+ channel blockers exerted protective effects in the same range of concentrations as HOE 694. Our findings demonstrate that HOE 694 prevents veratrine contractures at concentrations which presumably affect Na(+)-H+ exchange. However, the mechanism by which HOE 694 affords protection is apparently mediated by class 1-type Na+ channel blockade.

[Reflections on the etiopathogenesis of cleft lip and palate and the development of their treatment]. / Réflexions sur l'étiopathogénie des fentes labio-maxillo-palatines et l'évolution de leurs traitements.

The management of cleft lip and palate patients should achieve good function, particularly concerning nasal breathing. For this purpose, we must have a sound knowledge of the pathological anatomy, to correct the deformity of the cartilaginous structures, and to restore a good muscular balance. However, surgery is the main cause of facial growth disturbances. Procedures which produce areas of denuded bone are the worst and the VY Veau-Wardill closure of the palate was abandoned for this reason. Since 1981, the new protocol consists of simultaneous repair of the lip, nostril and soft palate at 6 months of age, and a very simple repair of the hard palate at 18 months of age, without denuded bone or early orthopaedics. The procedure results in good facial growth at ten years of age, with less velopharyngeal insufficiency. The value of good management of the cleft alveolar arch with secondary periosteogingivoplasty saving the lateral incisor space, and the need for an early patent nasal air way are stressed.

Investigation of the mechanism by which ketanserin prolongs the duration of the cardiac action potential.

Action potential duration (APD) lengthening is believed to underlie the cardiac arrhythmogenicity of ketanserin, a serotonin (5-HT)2A/2C receptor antagonist. We wished to determine (a) whether this activity involves blockade of 5-HT2A/2C receptors and (b) the precise mechanism of ketanserin-induced APD prolongation. APs were recorded in guinea pig isolated papillary muscles by conventional "floating" microelectrodes, and potassium currents in guinea pig isolated myocytes were recorded in the whole-cell configuration. Ketanserin (1-10 microM) increased APD (EC50 value for enhancing APD at 90% repolarization (APD90) 3.1 +/- 2.7 microM, n = 24), without affecting resting potential, maximum upstroke velocity (Vmax) or AP amplitude (APA). Pirenperone (10 microM), a ketanserin congener, similarly increased APD90 from 204 +/- 3 to 241 +/- 7 ms (p < 0.001, n = 6). No increase in APD was observed, however, with ritanserin or ICI 170809, even at high concentrations (10 microM, n = 6, respectively), two 5-HT2A/2C receptor antagonists chemically distinct from ketanserin, thereby excluding the involvement of 5-HT2A/2C receptors in mediating APD lengthening. That APD prolongation was mediated specifically by the benzolyl-piperidine moiety of ketanserin and pirenperone was confirmed by 1-propyl-4(4-fluorobenzoyl)piperidine (PFBP), which evoked APD lengthening effects remarkably similar to those produced by ketanserin and pirenperone (EC50 3.73 +/- 2.6 microM, n = 12). In isolated cardiomyocytes, ketanserin (1-32 microM) selectively and concentration-dependently reduced the IKr component of the delayed outward current (IK) without affecting the inward rectifier current, IK1. Thus, ketanserin (32 microM) significantly reduced IK at a potential value of -20 mV from 813 +/- 65 to 569 +/- 55 pA (p < 0.001, n = 6), whereas at a potential value of -110 mV, IK1 was not significantly affected (730 +/- 103 vs. 603 +/- 143 pA, respectively; n=6). The results demonstrate that APD is prolonged by ketanserin and congeners but not be chemically different 5-HT2A/2C receptor antagonists. The benzoyl-piperidine moiety appears to mediate the APD-prolonging effects of ketanserin and pirenperone specifically. Furthermore, ketanserin-induced APD lengthening does not appear to involve 5-HT2A/2C receptors but is consecutive to direct blockade of myocardial potassium channels.

Alleviation of contractile dysfunction in ischemic hearts by slowly inactivating Na+ current blockers.

We hypothesized that the slowly inactivating component of Na+ current, which is an integral part of the Na+ window current, is a major pathway for Na+ loading during myocardial ischemia. The putative protective effects of tetrodotoxin (TTX) and R-56865, at concentrations that selectively blocked the Na+ window current, as assessed by action potential plateau shortening without affecting maximum upstroke velocity (Vmax), were examined in isolated Langendorff-perfused guinea pig hearts subjected to 50 min of normothermic global ischemia and 60 min of reperfusion. In papillary muscles, TTX reduced action potential duration at > or = 10 nM but reduced Vmax only at > or = 1 microM. R-56865 (10 nM-10 microM) failed to affect Vmax but concentration dependently reduced action potential duration. Ischemia-induced cardiac dysfunction, including increases in left ventricular end-diastolic pressure and lactate dehydrogenase and creatine phosphokinase release at reperfusion, was attenuated by TTX and R-56865 (0.1-320 nM). Ischemic contracture (increase in left ventricular end-diastolic pressure) was abolished by drug concentrations as low as 1 nM, whereas higher concentrations (> 10 nM) of TTX and R-56865 were required to restore systolic function at reperfusion. TTX and R-56865 had little or no effect on hemodynamic variables. Evidence is provided of pronounced and direct cardioprotective effects of low concentrations of R-56865 and TTX in cardiac muscle during ischemia.(ABSTRACT TRUNCATED AT 250 WORDS)

Nasal malformations associated with unilateral cleft lip. Accurate diagnosis and management.

The results of correction of nasal defects in patients with unilateral cleft lip are often disappointing because the malformation has not been clearly understood. Many illustrations of deformity of the lower lateral cartilage are anatomically incorrect. To understand the true nature of the deformity, the surgeon must have a fundamental knowledge of the musculature of the region, particularly the nasolabial portion of the orbicular muscle and the nasal muscle complex. Correction of the nose must establish a physiological nasal airway, which is necessary for good subsequent facial growth. This can be accomplished without either excessive scarring or a cartilage graft, by careful reconstruction of nasolabial muscular integrity with the anterior nasal spine and the septopremaxillary ligament, functional repair of the orbicular muscle, and finally by raising and rotating the displaced alar cartilage. These principles apply equally to both primary and secondary operations.

Veratrine-induced tetanic contracture of the rat isolated left atrium. Evidence for novel direct protective effects of prazosin and WB4101.

An investigation has been made of the putative direct myocardial protective effects of the alpha 1-adrenoceptor antagonists, prazosin and WB4101, against tetanic contractures of rat isolated left atria following modified Na+ channel function and consequent Ca2+ loading elicited by veratrine. Veratrine evoked concentration-dependent, reversible, tetanic contractures which were critically dependent upon the external Ca2+ concentration. Tetrodotoxin (TTX), prazosin, WB 4101 and R 56865 (0.1-10 microM) prevented tetanic contracture elicited by veratrine (100 micrograms/ml) at concentrations which were significantly lower than those which decreased active tension development. The apparent Hill coefficients (nH) obtained for TTX, prazosin, WB 4101 and R 56865 were comparable (range 0.79-0.93), and are consistent with a single site of action. In contrast, the class 1 antiarrhythmic agents, quinidine and lidocaine, elicited no significant inhibition of veratrine-induced contracture at 30 microM, but almost completely abolished the contractures at 100 microM. The nH values for quinidine and lidocaine were found to be significantly greater than unity (3.1 and 2.6, respectively). The L-type Ca2+ channel blockers, diltiazem, nicardipine, nifedipine and verapamil only weakly prevented tetanic contracture, whilst markedly, and concentration-dependently, decreasing active tension development. Neither atropine (10 microM) nor propranolol (1 microM) significantly modified either veratrine-induced contractures or active tension development. In conclusion, evidence is presented of novel, direct protective effects of prazosin and WB 4101 against tetanic contracture following modified Na+ channel function and Ca2+ loading provoked by veratrine. The precise mechanisms involved are unclear at present, but appear to be distinct from blockade of atrial alpha 1-adrenoceptors or L-type Ca2+ channels.(ABSTRACT TRUNCATED AT 250 WORDS)