A case of Keutel syndrome diagnosed in the neonatal period: associated with Binder phenotype

Keutel syndrome is a rare autosomal recessive disorder, characterized by brachytelephalangia (short, broad distal phalanges), midfacial hypoplasia, abnormal cartilage calcifications, peripheral pulmonary stenosis and hearing loss. Binder profile is a well known maxillonasal dysplasia composed of midfacial hypoplasia with absence of anterior nasal spine and facial dysmophism (short nose, flat nasal bridge, perialar flatness, convex upper lip). Here we report a Keutel syndrome presenting with Binder phenotype, abnormal calcifications, hearing loss and respiratory insufficiency in the newborn period. Keutel syndrome should be considered in the differential diagnosis of children with tracheobronchial calcifications, midfacial hypoplasia and stippled epiphysis.     

Surgical correction of the nose and midface in maxillonasal dysplasia (Binder's syndrome)

In 48 patients with maxillonasal dysplasia the retruded nasal base was corrected with onlay cancellous bone grafts after subperiosteal dissection using an oral vestibular approach. Support for the nasal dorsum was achieved in 39 patients with an L-shaped bone graft from the iliac crest introduced through the same approach. The advancement of the nose was found stable on lateral cephalograms; i.e., resorption did not occur. However, the grafts showed considerable remodeling. Half the patients found the stiffness of the nose to be disturbing. In nine patients, the cartilaginous septum was used instead as a support for the nasal dorsum and tip. At operation, the entire cartilaginous septum was mobilized after subperichondrial dissection and rotated forward either pedicled at the nasal dorsum or completely released. Cartilage regenerated in the periochondrial pocket left behind the advanced septum. The anterior transfer of the nose was 6 to 10 mm. The use of septal advancement is preferred over bone implants in the correction of maxillonasal dysplasia in patients in whom the bony nasal dorsum is of adequate height because it results in a soft and flexible nose and the risk of traumatic fracture and resorption is eliminated. The technique has been used in adolescents with promising results.     

Cephalometric and anthropomorphic observations of Binder's syndrome: a study of 19 patients

Binder's syndrome (maxillonasal dysplasia) is a disorder characterized by nasomaxillary hypoplasia. To ascertain the extent of underdevelopment of the midfacial skeleton and soft tissues, 19 of 29 patients with Binder's syndrome were retrospectively evaluated, both with cephalometry and anthropometry. Ten females and nine males were placed collectively into three age groups: 6 years, 10 years, and 16 to 17 years. Cephalometric measurements disclosed a short anterior cranial base (S-N), a normal length of the vertical maxilla (SE-PNS), a decreased horizontal maxilla (PNS-A, Co-A), a recessed orbitale (SNO), and a high-normal mandibular length (Co-Gn). Anthropometry revealed a large nasofrontal angle, acute nasal inclination and nasolabial angle, decreased nasal prominence (Sn-Prn), a decreased columellar length (C-Sn), and a normal vertical nose (N-Sn) and upper lip (Sn-Sto) length.     

The warfarin embryopathy: a rat model showing maxillonasal hypoplasia and other skeletal disturbances.

Sprague-Dawley rats were given daily subcutaneous doses of sodium warfarin (100 mg/kg) and vitamin K1 (10 mg/kg) for up to 12 weeks, starting on the day after birth. This dosing regimen creates an extrahepatic vitamin K deficiency while preserving the vitamin K-dependent processes of the liver. Control rats received either vitamin K1 only or were untreated. All rats survived without any signs of hemorrhage. The warfarin-treated rats developed a marked maxillonasal hypoplasia associated with a 11-13% reduction in the length of the nasal bones compared with controls. The length of the posterior part of the skull was not significantly different from controls. In the warfarin-treated rats, the septal cartilage of the nasal septum showed large areas of calcification, not present in controls, and abnormal calcium bridges in the epiphyseal cartilages of the vertebrae and long bones. The ectopic calcification in the septal cartilage may have been the cause of the reduced longitudinal growth of the nasal septum and the associated maxillonasal hypoplasia. It is proposed that (1) the facial features of the human warfarin embryopathy are caused by reduced growth of the embryonic nasal septum, and (2) the septal growth retardation occurs because the warfarin-induced extrahepatic vitamin K deficiency prevents the normal formation of the vitamin K-dependent matrix gla protein in the embryo.     

Nasal fossa malformations and paramedian facial cleft: new perspectives.

Choanal atresia may be associated with other cranio-facial malformations, including various degrees of nasal fossa malformation, and may be a part of paramedian facial clefts (as described by Tessier et al. [1977]). We identified five such cases with combined clinical elements corresponding to Tessier's paramedian facial cleft, including eyelid coloboma, mild to severe choanal and nasal fossa anomalies, ethmoidal hypoplasia and anterior skull base malformation, sometimes with proboscis lateralis and half-nose hypoplasia. These observations incited us, first, to elaborate a conception which accounts for the likely embryological mechanisms involved; second, to propose a new classification based on anatomical and pathogenic embryological considerations; and last, to propose the use of transpalatal approach to restore choanal permeability, since endonasal laser therapy is particularly dangerous in such cases.     

Binderoid complete cleft lip/palate

A small subset of infants with complete cleft lip/palate look different because they have nasolabiomaxillary hypoplasia and orbital hypotelorism. The authors' purpose was to define the clinical and radiographic features of these patients and to comment on operative management, classification, and terminology. The authors reviewed 695 patients with all forms of incomplete and complete cleft lip/palate and identified 15 patients with nasolabiomaxillary hypoplasia and orbital hypotelorism. All 15 patients had complete labial clefting (5 percent of 320 patients with complete cleft lip/palate), equally divided between bilateral and unilateral forms. The female-to-male ratio was 2:1. Of the seven infants with unilateral complete cleft lip/palate, one had an intact secondary palate and all had a hypoplastic septum, small alar cartilages, narrow basilar columella, underdeveloped contralateral philtral ridge, ill-defined Cupid's bow, thin vermilion-mucosa on both sides of the cleft, and a diminutive premaxilla. Of the eight infants with bilateral complete cleft lip, one had an intact secondary palate. The features were the same as in patients with unilateral cleft, but with a more severely hypoplastic nasal tip, conical columella, tiny prolabium, underdeveloped lateral labial elements, and small/mobile premaxilla. Central midfacial hypoplasia and hypotelorism did not change during childhood and adolescence. Intermedial canthal measurements remained 1.5 SD below normal age-matched controls. Skeletal analysis (mean age, 10 years; range, 4 months to 19 years) documented maxillary retrusion (mean sagittal maxillomandibular discrepancy, 13.7 mm; range, 3 to 17 mm), absent anterior nasal spine, and a class III relationship. The mean sella nasion A point (S-N-A) angle of 74 degrees (range, 65 to 79 degrees) and sella nasion B point (S-N-B) angle of 81 degrees (range, 71 to 90 degrees) were significantly different from age-matched norms ( = 0.0007 and = 0.004, respectively). The ipsilateral central and lateral incisors were absent in all children with unilateral cleft, whereas a single-toothed premaxilla was typically found in the bilateral patients. Several modifications were necessary during primary nasolabial repair because of the diminutive bony and soft-tissue elements. All adolescent patients had Le Fort I maxillary advancement and construction of an adult nasal framework with costochondral or cranial graft. Other often-used procedures were bony augmentation of the anterior maxilla; cartilage grafts to the nasal tip and columella; and dermal grafting to the median tubercle, philtral ridge, and basal columella. Infants with complete unilateral or bilateral cleft lip/palate in association with nasolabiomaxillary hypoplasia and orbital hypotelorism do not belong on the holoprosencephalic spectrum because they have normal head circumference, stature, and intelligence, nor should they be referred to as having Binder anomaly. The authors propose the term cleft lip/palate for these children. Early recognition of this entity is important for counseling parents and because alterations in standard operative methods and orthodontic protocols are necessary.     

Comparative macro- and microscopic anatomy of the nasal cavity of European insectivores]

The topography of the nasal fossa and its epithelium were studied in 4 European Insectivores, Sorex araneus L., Crocidura russula (Hermann), Talpa europaea L. and Erinaceus europaeus L. The following results were obtained: 1. The length of the nasal capsules is in relation to the length of the head in all 4 species the same. 2. The noses of all forms studies, except E. europaeus are very similar because of the trunk-shaped pars anterior nasi. 3. The numbers of turbinals are constant. 4. Of all turbinals the atrio-turbinal is ventrally the rostral one, and in the shrews it is considerably longer than in E. europaeus and T. europaea. 5. There is a maxillo-turbinal caudal of the atrio-turbinal. Both of these turbinals can be found separated by an incisura atrio-maxillo-turbinalis in T. europaea and the shrews. In E. europaeus the skeleton of the 2 turbinals is bridged by a fold of mucous membrane. 6. The maxillo-turbinal is bilamellar in T. europaea and the shrews, with each of the laminae rolled up in opposite direction. The surface of the maxillo-turbinal of E. europaeus is increased enormously by means of secondary folds. 7. The naso-turbinal begins almost at the tip of the nose and approaches the lamina cribrosa, where it disappears. One can discern 3 differently shaped parts of the naso-turbinal: Crus orale, crus intermedium and crus aborale. The nasoturbinal originates out of 2 different structures (the crus orale of the naso-turbinal and the lamina semicircularis) which are ontogenetically different, however, they have become fused during ontogeny, thus forming a structure which seems to be homogenous. 8. There are 3 ethmo-turbinals. The 1st ethmo-turbinal is the largest one and its free anterior tip is found in the intermediate part of the nasal fossa. Its epi-turbinal is an accessory lamella found in the aboral part of the first ethmoturbinal. Except in S. araneus it always appears in paraseptal views between the ethmo-turbinal I and II. 9. There are 3 ecto-turbinals. 2 ecto-turbinals are situated between the naso-turbinal and the ethmo-turbinal I, whereas the 3rd ecto-turbinal appears between ethmo-turbinal I and II. 10. There are 3 recesses of the lateral parts of the nasal fossa: The recessus frontalis anterior is found rostral-dorsally of the pars intermediate of the nasal fossa. The recessus frontalis posterior communicates with the former, but is located caudal of it. The 3rd recessus sphenoidalis is actually a subcerebral niche of the nasal fossa in the os sphenoidale. 11. There is only one pneumatic cavity in Insectivores, the sinus maxillaris. 12. The nasal fossa can be divided into a regio vestibularis, regio respiratoria and regio olfactoria. The epithelium of the nasal fossa is similar in all forms studied. 13. The anterior part of the oral regio vestibularis is outlined by keratinized epithelium; posteriorly, by loosing its stratum corneum, it changes into unkeratinized pseudostratified epithelium. 14...     

Pure orbital blowout fracture: new concepts and importance of medial orbital blowout fracture.

Pure orbital blowout fracture first occurs at the weakest point of the orbital wall. Although the medial orbital wall theoretically should be involved more frequently than the orbital floor, the orbital floor has been reported as the most common site of pure orbital blowout fractures. A total of 82 orbits in 76 patients with pure orbital blowout fracture were evaluated with computed tomographic scans taken on all patients with any suspicious clinical evidence, including nasal fracture. Isolated medial wall fracture was most common (55 percent), followed by medial and inferior wall fracture (27 percent). The most common facial fracture associated with medial wall fracture was nasal fracture (51 percent), not inferior wall fracture (33 percent). This finding suggests that the force causing nasal fracture is an important causative factor of pure medial wall fracture as the buckling force from the medial orbital rim. Of patients with medial wall fractures, 25 percent had diplopia and 40 percent had enophthalmos. On plain radiographs, diagnostic signs were found in 79 percent of medial wall fractures and in 95 percent of inferior wall fractures. On computed tomographic scans, late enophthalmos was expected in 76 percent of medial wall fractures. Therefore, the medial orbital blowout fracture may be an important cause of late enophthalmos, because it has a high incidence of occurrence, a low diagnostic rate, and a high severity of defect. Among the causes of limitation of ocular motility, muscle traction of the connective septa and direct muscle injury were found frequently, but true incarceration of the muscle was extremely rare in all fractures. The medial and inferior orbital walls are clearly demarcated by the bony buttress, which is an important structure supporting these orbital walls. Its buttress was closely correlated with the fracture of these orbital walls. Most orbital blowout fractures without collapse of the bony buttress had a trapdoor fracture with or without small fragments of punched-out fracture.     

A novel population of neuronal cells expressing the olfactory marker protein (OMP) in the anterior/dorsal region of the nasal cavity

The olfactory marker protein (OMP) is expressed in mature chemosensory neurons in the nasal neuroepithelium. Here, we report the identification of a novel population of OMP-expressing neurons located bilaterally in the anterior/dorsal region of each nasal cavity at the septum. These cells are clearly separated from the regio olfactoria, harboring the olfactory sensory neurons. During mouse development, the arrangement of the anterior OMP-cells undergoes considerable change. They appear at about stage E13 and are localized in the nasal epithelium during early stages; by epithelial budding, ganglion-shaped clusters are formed in the mesenchyme during the perinatal phase, and a filiform layer directly underneath the nasal epithelium is established in adults. The anterior OMP-cells extend long axonal processes which form bundles and project towards the brain. The data suggest that the newly discovered group of OMP-cells in the anterior region of the nasal cavity may serve a distinct sensory function.     

One-stage closure of the entire primary palate

Timing of the closure of the anterior palate and alveolus is a subject of debate. Late repair of this defect is complicated by high fistula formation and subjects the patient to the problems of palate fistula for extended periods of time. We have utilized a single procedure performed when the child is 3 months of age that completely closes the anterior hard palate and alveolus along with the cleft lip. Our series consisted of 61 consecutive patients with unilateral clefts of the primary and secondary palate. Mucosal turnover flaps from the vomer along with lateral nasal mucosal flaps provide the nasal lining. A buccal sulcus flap with a Veau flap completes the oral repair. Ninety-five percent (58 of 61) of the patients had complete and stable closure of their anterior palate and alveolus after 1 year. The incidence of fistula formation in our series (3 of 61) is much lower than that reported with the utilization of other protocols. Excellent exposure of the anterior palate and alveolar defect during lip repair, early restoration of anatomic relationships, establishment of a good nostril floor and sill, and very low fistula formation are among the benefits of this procedure. The increase in operative time is considered minimal in light of aforementioned advantages.     

An additional manifestation in acrocallosal syndrome: temporal lobe hypoplasia

Acrocallosal Syndrome is a rare genetic disorder which is characterized by moderate to severe mental retardation, agenesis or hypoplasia of the corpus callosum and polydactyly of fingers and toes. The spectrum of this syndrome is very variable. Prominent forehead, broad nasal bridge, short nose and mandible, hypertelorism, epicanthic folds, large anterior fontanelle and tapered fingers, omphalocele and inguinal hernia are some other common findings in this syndrome. Twenty percent of the patients have associated brain abnormalities such as cerebral atrophy, hypothalamic dysfunction, small cerebrum, micropolygyria, hypoplasia of pons, hypoplasia of cerebellar hemispheres, hypoplasia of medulla oblongata, agenesis or hypoplasia of cerebellar vermis and corpus callosum abnormalities. Here we present a 10-month-old female infant with clinical and radiological findings indicative of acrocallosal syndrome. She was noted to have craniofacial abnormalities suggestive of acrocallosal syndrome, optic atrophy and polydactyly. MRI revealed cerebral atrophy, corpus callosum agenesis, dilated lateral ventricules and unilateral right temporal lobe hypoplasia, the latter not previously reported in the spectrum of this syndrome. Based on this observation we conclude the importance of screening brain abnormalities and present temporal lobe hypoplasia as a new additional anomaly in this syndrome.     

A Quantitative Study of Olfactory, Non-Olfactory, and Vomeronasal Epithelia in the Nasal Fossa of the Bat Megaderma lyra

In complexity, the mammalian nasal fossa is unparalleled among vertebrates. Although total nasal epithelial surface areas (SA) have been reported for numerous mammals, detailed quantitative reports on individual structures exist for few mammals. Here, we examine mucosal distribution in the nasal fossa of the greater false vampire bat, Megaderma lyra (Megadermatidae, Chiropera). The SA of the left nasal fossa of one adult Megaderma was measured in serial histological sections; the development of the nasal fossa was assessed using three fetal specimens. The nasal fossa of Megaderma has seven ethmoturbinals and multiple smaller interturbinals between them, all bearing olfactory mucosa. Nearly half of the total olfactory SA of the nasal fossa lines these turbinals; the remainder lines recesses and parts of the medial (septal) and lateral walls of the nasal fossa. The maxilloturbinal is diminutive, and the nasoturbinal is absent. Volumetric measurements of the fetal and adult vomeronasal organ suggest age-related reduction. Thirty-five percent of the nasal fossa is lined with olfactory mucosa, within the range reported previously for chiropterans. In Megaderma the frontal recess contributes little to total nasal SA (2% of all olfactory SA). This represents a significant departure in morphology compared to other mammals, including some bats, in which the frontal recess is much larger. The significance of the emphasis in olfactory SA distribution to central or more peripheral (paranasal) spaces could be investigated using a large sample of phylogenetically diverse mammals, such as bats. This study emphasizes the need for more histological detail to further such studies.     

Primary definitive nasal correction in patients presenting for late unilateral cleft lip repair

Almost 25 percent of unilateral cleft lip and palate patients present with their deformity in their teens or later years in the developing world. Because more than 80 percent of the world population lives in the developing world, the established protocol for repair of these deformities is not applicable to these patients. Despite the magnitude, there are no significant reports in the literature that deal with this problem. Several issues need to be addressed, but the author limits himself here to the correction of the nasal deformity. The patients at this age are very much concerned with the aesthetic outcome. Procedures described hitherto for primary nasal correction in infants are not successful in restoring nasal shape and symmetry at this late age of presentation. Our experience with radical correction of secondary nasal deformity in unilateral cleft lip patients presenting late prompted us to extend the concept by undertaking a definitive primary correction of the nasal deformity in cleft patients presenting late. Twenty-two patients with unilateral cleft lip deformity (nine male patients and 13 female patients) with ages ranging from 13 to 22 years, presenting between August of 1997 and December of 2000, are included in this study. Of these, 11 patients had a cleft of the lip alone, eight also had a cleft of the alveolus, and three had a cleft of the palate continuous with the cleft lip. All patients showed some maxillary hypoplasia. An external rhinoplasty with lip repair was carried out in all patients. The corrective procedures on the nose included columellar lengthening; augmentation along the pyriform margin, nasal floor, and alveolus using bone grafts; submucous resection of the nasal septum; repositioning of lower lateral cartilages; and augmentation of nasal dorsum by bone graft. Clinical follow-up ranged from 4 to 24 months, and the median follow-up period was 13 months. Results have been very good, and much better than results seen earlier with other primary rhinoplasty techniques. While repairing unilateral cleft lip in adolescents, the author thinks it would be most appropriate to address the entire gamut of the deformity in a single stage, provide complete vector reorientation, and augment the hypoplastic elements by autologous tissue. It is not just the fear of poor follow-up, but that merely correcting the lip deformity in these patients without attempting definitive rhinoplasty, in the author's opinion, would be insufficient surgical intervention.     

In vivo localization of Staphylococcus aureus in nasal tissues of healthy and influenza A virus-infected ferrets

An in vivo ferret model was used to study the association of Staphylococcus aureus with specific tissues of the nasal cavity in both control and influenza A virus-infected animals. Ferrets were inoculated intranasally with various doses of influenza A3/Hong Kong/1/68 virus. On Days 2, 5, 9 and 14, four or five virus-inoculated and two uninoculated controls were challenged intranasally with a 1-ml volume of radiolabeled S. aureus (3 mg dry wt), a clinical isolate of low passage history. Ferrets were allowed to clear the staphylococci in vivo for 60 to 90 min before sacrifice. The animals were anesthetized, exsanguinated, and decapitated, and the lower jaw was removed. The nasal fossae were exposed by dissection and turbinates from the left nasal fossa were used for virus isolation. The median septum and tissues from the right nasal fossa, which included vestibule and anterior and posterior turbinates, were harvested and processed for radioassay. The percentage of recoverable staphylococci from virus-infected ferrets (Days 2 and 5) was greater than or equal to 10-fold higher compared with controls and animals infected with suboptimal doses of virus; greater than or equal to 76% of the recoverable staphylococci, whether from controls or virus-infected animals, was associated with the anterior turbinates. Histologic examination of the anterior turbinates from virus-infected ferrets, particularly on Days 2 and 5 postexposure to virus, showed that the staphylococci were adhering to desquamating respiratory epithelial cells. In contrast, the anterior turbinates from control ferrets uninoculated with virus and posterior turbinates from both control and virus-infected animals showed no evidence of bacteria adhering to host cells; instead, the staphylococci were found in association with the mucus gel layer of respiratory mucosa. Examination of vestibular tissue showed staphylococci in association with cells of the stratum granulosum in both virus-infected and control animals. Results of this study suggest that the early events of S. aureus interaction with different sites of ferret nasal tissues are effected by different mechanisms, and that the interaction is significantly enhanced by virus-infection.     

Is packing after septorhinoplasty necessary? A randomized study

This randomized study was conducted to evaluate the role of nasal packing following septorhinoplasty. Fifty septorhinoplasty patients were selected (on a random basis) to either receive or not receive nasal packing. Twenty-three of 25 patients with nasal packing and 22 of 25 patients without nasal packing were available for follow-up. This study suggests that patients with nasal packing are less likely to develop recurrent septal deviation and synechia and more likely to have improvement in the nasal airway. Only one of the patients with nasal packing found the removal of the packing the most uncomfortable part of the surgery. The most impressive and statistically significant finding, however, was the significant difference between the two groups in terms of airway improvement, which was 96 percent in the nasal packing group and 64 percent in the group without nasal packing. There was also a higher incidence of recurrent or residual septal deviation in the group without nasal packing (41 percent), while the group with nasal packing had only (13 percent) recurrent or residual deviation.     

Lip-nasal aesthetics following Le Fort I osteotomy

Forty-one patients undergoing Le Fort I osteotomy for superior and/or anterior repositioning of the maxilla were prospectively studied for changes in soft-tissue morphology of the nasomaxillary region. Nasal parameters studied were changes in interalar rim width and nasal tip projection. It was observed that alar rim width increases with anterior and/or superior repositioning of the maxilla, but increases in nasal tip projection occur only when there is an anterior vector of maxillary movement. These nasal changes could not be quantitatively correlated to magnitude of maxillary movement. Lip changes studied were the horizontal displacement at the vermilion border and subnasale versus that of the incisal edge and point A, respectively, when the maxilla is sagittally advanced and the vertical shortening of the lip versus that of the incisal edge when the maxilla is shortened. Using linear regression analysis, horizontal displacement of the upper lip at the vermilion border was 0.82 +/- 0.13 mm for every 1 mm of maxillary advancement at the incisal edge (p less than 0.001) and 0.51 +/- 0.13 at the subnasale for every 1 mm of maxillary advancement at point A (p less than 0.001). Eighty percent of patients undergoing maxillary intrusive procedures had lip shortening ranging from 20 to 50 percent of the vertical maxillary reduction. Surprisingly, no statistically significant correlation could be demonstrated for lip shortening versus extent of vertical maxillary reduction. Previous literature in disagreement with these findings is discussed. Guidelines for treatment planning utilizing these data are suggested.     

New ontogenetic evidence on the septomaxilla ofTamandua andCholoepus (Mammalia,Xenarthra), with a reevaluation of the homology of the mammalian septomaxilla

An intramembranous ossification at the anterior end of the cartilaginous nasal capsule is described for the first time in prenatal specimens of the anteaterTamandua and the slothCholoepus and redescribed in prenatal specimens of the armadillosDasypus andZaedyus. From comparisons of this bone with the septomaxilla of monotremes and various Mesozoic mammals, it is concluded that (1) the bone inTamandua andCholoepus is homologous with the central part (processus ascendens) of the bone inDasypus, Zaedyus, and other armadillos and (2) the xenarthran processus ascendens, in turn, is homologous with the central part of the septomaxilla of monotremes and various Mesozoic mammals. Therefore, the bone in question in xenarthrans is a true septomaxilla. It is further concluded that the armadillo septomaxilla has two neomorphic components: a lamina palatina beneath the cartilaginous nasal floor and a processus intrafenestralis extending rostrally into the nasal fossa.     

Internal nasal floor configuration in Homo with special reference to the evolution of Neandertal facial form

The presence of a steeply sloping or depressed nasal floor within the nasal cavity of Neandertals is frequently mentioned as a likely specialization or autapomorphy. The depressed nasal floor has also been seen as contributing to a relatively more capacious nasal cavity in Neandertals, which is tied to cold-climate respiratory adaptation and energetics. These observations have been limited largely to a relatively few intact crania, and the character states associated with this trait have not been as precisely codified or analyzed as those published for Plio-Pleistocene hominins (McCollum et al., 1993, J. Hum. Evol. 24, 87; McCollum, 2000, Am. J. Phys. Anthrop. 112, 275).This study examines the internal nasal floor topography in complete crania and isolated maxillae in European, west Asian, and African fossil Homo (n=158) including 25 Neandertals, and a wide range of recent humans from Europe, the Near East, and Africa (n=522). The configuration of the internal nasal floor relative to the nasal cavity entrance is codified as: 1) level, forming a smooth continuous plane; 2) sloped or mildly stepped; or 3) bilevel with a pronounced vertical depression. The frequency of these nasal floor configurations, and their relationship to both nasal margin cresting patterning and a comprehensive set of nasofacial metrics is examined.Neandertals show a high frequency of the bilevel (depressed) configuration in both adults and subadults (80%), but this configuration is also present in lower frequencies in Middle Pleistocene African, Late Pleistocene non-Neandertal (Skhul, Qafzeh), and European Later Upper Paleolithic samples (15%-50%). The bilevel configuration is also present in lower frequencies (ca. 10%) in all recent human samples, but attains nearly 20% in some sub-Saharan African samples. Across extinct and extant Homo (excluding Neandertals), internal nasal floor configuration is not associated with piriform aperture nasal margin patterning, but the two are strongly linked in Neandertals. Variation in internal nasal floor configuration in recent humans is primarily associated with internal nasal fossa breadth and nasal bridge elevation, whereas in fossil hominins, it is associated primarily with variation in facial height. Cold-climate and activity-related thermal adaptation as an explanation for the high frequency of pronounced nasal floor depression in Neandertals is inconsistent with all available data. Alternatively, variation in internal nasal floor configuration is more likely related to stochastically derived populational differences in fetal nasofacial growth patterns that do not sharply differentiate genus Homo taxa (i.e., cladistically), but do phenetically differentiate groups, in particular the Neandertals, especially when considered in combination with other nasofacial features.     

Primary correction of the unilateral cleft nasal deformity

An 18-year experience with the management of the unilateral cleft nasal deformity in 1200 patients is presented. A primary cleft nasal correction was performed at the time of lip repair in infancy; a secondary rhinoplasty was done in adolescence after nasal growth was complete. The technical details of the authors' primary cleft nasal correction are described. Exposure was obtained through the incisions of the rotation-advancement design. The cartilaginous framework was widely undermined from the skin envelope. The nasal lining was released from the piriform aperture, and a new maxillary platform was created on the cleft side by rotating a "muscular roll" underneath the cleft nasal ala. The alar web was then managed by using a mattress suture running from the web cartilage to the facial musculature. In 60 percent of cases, these maneuvers were sufficient to produce symmetrical dome projection and nostril symmetry. In the other 40 percent, characterized by more severe hypoplasia of the cleft lower lateral cartilage, an inverted U infracartilaginous incision and an alar dome supporting suture (Tajima) to the contralateral upper cartilage were used. Residual dorsal hooding of the lower lateral cartilage was most effectively managed with this suture. This primary approach to the cleft nasal deformity permits more balanced growth and development of the ala and domal complex. Some of the psychological trauma of the early school years may be avoided. Also, because of the early repositioning of the cleft nasal cartilages, the deformity addressed at the time of the adult rhinoplasty is less severe and more amenable to an optimal final result.     

Probable case of Binder syndrome in a skeleton from Quarai,New Mexico

Binder syndrome (maxillonasal dysplasia) is a not uncommon disorder reported in the clinical literature and is characterized by hypoplastic development of the midface. An extensive review of the paleopathology literature did not reveal any examples of Binder syndrome. In this paper, a probable case of Binder syndrome in a female skeleton, 16-17 years at age of death, from Quarai, New Mexico (ca. AD 1375-1450) is presented. This case was identified during standard documentation prior to repatriation at the National Museum of Natural History, Smithsonian Institution.The skull of this individual (381243) exhibits unusual facial features, including an underdeveloped midface, flattened glabella, absent nasal spine, and apparent alveolar prognathism, in addition to a vertebral anomaly. All of these characteristics are consistent with skeletal dysmorphologies associated with Binder syndrome. Measurements of the Quarai skull are compared with published data on Binder patients and normal control groups in order to quantify the nature of the observed morphology. Univariate analysis of craniometric/cephalometric data provides further support for a diagnosis of Binder syndrome, as critical measurements on the Quarai skull are consistent with those reported in Binder patients and significantly different from those reported for normal control groups. In addition to presenting a probable prehistoric case of Binder syndrome, this paper demonstrates the applicability of using direct comparisons of clinical data to help identify unusual conditions in skeletal remains.