Cranial Configuration Research Articles

Orofacial findings and orthodontic treatment conditions in patients with down syndrome – a retrospective investigation

IntroductionThe most common chromosomal anomaly is Down syndrome/Trisomy 21, which can be associated with varying degrees of intellectual disability and physical malformation. Specific orofacial characteristics regarding orthodontic treatment options and features are described on the basis of a patient collective from the Witten/Herdecke University, Germany.MethodsData of 20 patients (14 boys and 6 girls, mean age: 11.69 ± 3.94 years) who underwent orthodontic treatment between July 2011 and May 2022 were analyzed. Baseline skeletal and dental conditions were assessed, as well as the presence of hypodontia, displacements, and treatment-related root resorptions. The treatment need was evaluated based on the main findings according to the German KIG classification. In addition, treatment success was determined in relation to patient compliance.ResultsThe patient group was characterized predominantly by a class III relationship (ΔANB: −2.07 ± 3.90°; ΔWITS: −3.91 ± 4.33 mm) and a brachyfacial cranial configuration (ΔML-NL: −4.38 ± 7.05°, ΔArGoMe: − 8.45 ± 10.06°). The transversal discrepancy of the dental arch width from maxilla to mandible was −0.91 ± 3.44 mm anteriorly and −4.4 ± 4.12 mm posteriorly. Considering the orthodontic indication groups, the most frequent initial finding and treatment indication represented hypodontia (85%), followed by frontal (75%) and unilateral lateral (35%) crossbite. In 55% of the cases, the teeth had a regular shape, but in 35% a generalized and in 15% an isolated hypoplasia. Only 25% of the patients could be treated with a fixed multiband appliance due to sufficient cooperation. In each of these patients, varying degrees of root resorptions were detected during treatment, and 45% of all treatments had to be terminated prematurely due to a lack of cooperation by patients or parents.ConclusionThe extent of dental and skeletal malformations and the high rate of findings requiring treatment in patients with Down syndrome represent a significant indication for orthodontic therapy, which can be well illustrated by the KIG classification. However, this is in contrast to the eventually increased risk of root resorption, with significantly reduced patient cooperation. A compromised treatment outcome and process must be expected. Consequently, the orthodontic treatment must be simple and realistic to achieve fast and therapeutically satisfactory treatment result.

Anatomic variations in the frontal and occipital bones: a comparative study of neurocranial morphology

Developmental variations in the cranial vault arise during early ontogenesis due to premature or delayed suture closure, non-union between the normal ossification centers, and emergence of additional ossification centers. These variations cause abnormal calvarial partition and are often associated with modifications of the overall cranial configuration. This study aims to evaluate the impact of metopic suture persistence and the development of supernumerary bones in the occipital region on neurocranial morphology. A series of 245 crania of Bulgarian males was investigated. According to the presence or absence of developmental variations in the frontal and occipital bone they were divided into four series: control (n = 115); crania with a retained metopic suture (n = 32); crania with supernumerary bones in the occipital region (n = 67); and metopic crania with supernumerary bones in the occipital region (n = 31). Polygonal 3D models were generated, on which the 3D coordinates of 35 landmarks describing the neurocranium were collected. Geometric morphometric analyses were applied to compare the size and shape of the landmark configurations. Significant size differences were observed only in the frontal and occipital bone configurations between the metopic crania with occipital supernumerary bones and the non-metopic crania. Significant shape differences were found in all comparisons between the metopic and non-metopic groups for all configurations, except the occipital one. Metopism has a significant impact on overall neurocranial morphology, which is intensified by the presence of supernumerary bones in the occipital region.

A detailed redescription of the mesoderm/neural crest cell boundary in the murine orbitotemporal region integrates the mammalian cranium into a pan-amniote cranial configuration.

The morphology of the mammalian chondrocranium appears to differ significantly from those of other amniotes, since the former possesses uniquely developed brain and cranial sensory organs. In particular, a question has long remained unanswered as to the developmental and evolutionary origins of a cartilaginous nodule called the ala hypochiasmatica. In this study, we investigated the embryonic origin of skeletal elements in the murine orbitotemporal region by combining genetic cell lineage analysis with detailed morphological observation. Our results showed that the mesodermal embryonic environment including the ala hypochiasmatica, which appeared as an isolated mesodermal distribution in the neural crest-derived prechordal region, is formed as a part of the mesoderm that continued from the chordal region during early chondrocranial development. The mesoderm/neural crest cell boundary in the head mesenchyme is modified through development, resulting in the secondary mesodermal expansion to invade into the prechordal region. We thus revealed that the ala hypochiasmatica develops as the frontier of the mesodermal sheet stretched along the cephalic flexure. These results suggest that the mammalian ala hypochiasmatica has evolved from a part of the mesodermal primary cranial wall in ancestral amniotes. In addition, the endoskeletal elements in the orbitotemporal region, such as the orbital cartilage, suprapterygoid articulation of the palatoquadrate, and trabecula, some of which were once believed to represent primitive traits of amniotes and to be lost in the mammalian lineage, have been confirmed to exist in the mammalian cranium. Consequently, the mammalian chondrocranium can now be explained in relation to the pan-amniote cranial configuration.

A novel Fontan Y-graft for interrupted inferior vena cava and azygos continuation.

OBJECTIVESTo evaluate the hemodynamicdynamic advantage of a new Fontan surgical template that is intended for complex single-ventricle patients with interrupted inferior vena cava-azygos and hemi-azygos continuation. The new technique has emerged from a comprehensive pre-surgical simulation campaign conducted to facilitate a balanced hepatic flow and somatic Fontan pathway growth after Kawashima procedure.METHODSFor 9 patients, aged 2 to18 years, majority having poor preoperative oxygen saturation, a pre-surgical computational fluid dynamics customization is conducted. Both the traditional Fontan pathways and the proposed novel Y-graft templates are considered. Numerical model was validated against in vivo phase-contrast magnetic resonance imaging data and in vitro experiments.RESULTSThe proposed template is selected and executed for 6 out of the 9 patients based on its predicted superior hemodynamic performance. Pre-surgical simulations performed for this cohort indicated that flow from the hepatic veins (HEP) do not reach to the desired lung. The novel Y-graft template, customized via a right- or left-sided displacement of the total cavopulmonary connection anastomosis location resulted a drastic increase in HEP flow to the desired lung. Orientation of HEP to azygos direct shunt is found to be important as it can alter the flow pattern from 38% in the caudally located direct shunt to 3% in the cranial configuration with significantly reversed flow. The postoperative measurements prove that oxygen saturation increased significantly (P-value = 0.00009) to normal levels in 1 year follow-up.CONCLUSIONSThe new Y-graft template, if customized for the individual patient, is a viable alternative to the traditional surgical pathways. This template addresses the competing hemodynamic design factors of low physiological venous pressure, high postoperative oxygen saturation, low energy loss and balanced hepatic growth factor distribution possibly assuring adequate lung development.Date and number of IRB approval25 October 2019, 280011928-604.01.01.

RETRACTED ARTICLE: A new azhdarchoid pterosaur from the Late Cretaceous Javelina Formation of Texas

A historic specimen described for the first time revealed important autapomorphic characters, permitting the definition of a new species, Javelinadactylus sagebieli gen. n. et sp. n., which represents the second toothless species from the Javelina Formation, Big Bend National Park of West Texas (United States of America). The remains of J. sagebieli (Azhdarchoidea: Tapejaridae) were found in 1986, but were never properly studied, and its taxonomic affinity remains undefined. The description is based on a partially articulated skull and mandible, which offer information on the anatomy of a single azhdarchoid pterosaur. J. sagebieli exhibit a large nasoantorbital fenestra, a rostral index of medium value and is assigned to the clade Thalassodrominae, a group of tapejarid pterosaurs that were reported exclusively from the Romualdo Formation of Brazil, with only two genera known. Thalassodromines are characterized by a typical cranial configuration with toothless jaws and a high and wide premaxilla bar, formed by sub-parallel or parallel borders. The new specimen described here represents the first record of the Tapejaridae group in the Maastrichtian of North America, and the cranial morphology of the new taxon increase the richest of the diversity of the azhdarchoid pterosaurs during the end of the Late Cretaceous, suggesting that the tapejarids were still diversifying in the Maastrichtian.

A tale from the middle Paleocene of Denmark: A tube-dwelling predator documented by the ichnofossil Lepidenteron mortenseni n. isp. and its predominant prey, Bobbitichthys n. gen. rosenkrantzi (Macroridae, Teleostei)

The ichnofossil Lepidenteron provides a unique taphonomic window into the life habits of a tube-dwelling predator, probably an eunicid polychaete, and its fish prey. Here we describe a new tube-like ichnofossil Lepidenteron mortenseni n. isp. from the Kerteminde Marl (100–150 m palaeo-water depth) from the Gundstrup gravel pit near Odense, Fyn, Denmark. 110 individual tubes were examined which contain fish remains, including a variety of disarticulated bones and otoliths, by far dominated by a single gadiform taxon referred herein to as Bobbitichthys n. gen. The isolated otoliths here associated with disarticulated gadiform bones have previously been described, from the time equivalent Lellinge Greensand exposed in the Copen-hagen area, as Hymenocephalus rosenkrantzi, a grenadier fish (family Macrouridae). The abundance of associated bones and otoliths in the examined tubes allowed us to reconstruct part of the cranial configuration of Bobbitichthys rosenkrantzi and to tentatively interpret it as a stem macrourid. Bobbitichthys rosenkrantzi represents the earliest grenadier known in the fossil record. Additional, although considerably less abundant, skeletal remains and otoliths have been tentatively referred to a long-fin bonefish (family Pterothrissidae, Pterothrissus? conchaeformis), a viviparous brotula (family Bythitidae, Bidenichthys? lapierrei), a conger eel (family Congridae, possibly belonging to Rhynchoconger angulosus), and another unidentified gadiform.

Growth and cranial development in the Andean frogs of the genus Telmatobius (Anura: Telmatobiidae): Exploring the relation of heterochrony and skeletal diversity.

Andean frogs of the genus Telmatobius occur at high elevations, they have an aquatic mode of life and large tadpoles. There are more than 60 species that closely resemble one another and have low values of genetic divergence. However, the skeleton, particularly the cranium, is interspecifically variable with respect to the different levels of development of some elements. Heterochrony is considered to have played a prominent role in generating phenotypic variation, especially among closely related species. Herein, the developmental origins of the adult cranial configuration of two species of Telmatobius are explored. The interactions among larval and postmetamorphic growth, sexual maturation, and ossification sequence in T. oxycephalus and T. rubigo are studied. Although there are no substantial changes in the sequences of ossification of the cranium, it is likely that differential timing of larval periods is related to adult cranial characters. The prolonged larval development of T. rubigo may result in peramorphic configurations of bones that ossify during pre-metamorphosis. This long developmental time would also explain why the gonads of T. rubigo are highly differentiated by the end of metamorphosis. In this species, sexual maturation may be attained precociously in relation to metamorphosis, thereby reducing postmetamorphic developmental time of late-onset bones, which have paedomorphic configurations (e.g., vomer, neopalatine, and columella). An inverse pattern characterizes T. oxycephalus, suggesting that the duration of larval life is related to skeletal configuration in Telmatobius.

Three-dimensional cranial ontogeny in pantherines (Panthera leo,P. onca,P. pardus,P. tigris; Carnivora:, Felidae)

The Panthera lineage is a monophyletic clade of felids, supported by both morphological and molecular evidence. The lineage includes large species with cranial similarity such as Panthera leo and P. tigris, and other with very different cranium such as P. pardus. The aim of our work was to study the cranial ontogeny of Pantherines, elucidating whether their cranial shape is a product of size or phylogeny, and to compare these findings with available information about other carnivores. We studied 370 specimens using geometric morphometrics technique in three dimensions. Panthera leo and P. tigris show similar ontogenetic trajectories, sharing adult crania with wider rostrum, shorter basicranium and vertical occipital plate. The cranial configuration of P. leo is a scaling version of P. tigris. P. pardus shows the most different cranial pattern, with adults having a rounded braincase and zygomatic arches less expanded than the rest, whereas P. onca occupies an intermediate place between these patterns. P. pardus is the species with the smallest birth weight and the lowest growth rate, reaching a final size and shape later than the remaining species. Adult shape morphology reflects no relation to phylogenetic placement of the species and it is probably related to Pantherine body size.

Forceps: Manejo en el Hospital Cayetano Heredia

En 3,767 partos atendidos en el Hospital cayetano Heredia se ha efectuado parto con fórceps en 520 pacientes. De éstos, 300 han correspondido a indicaciones maternas o fetales o materno-fetales y 220 a fórceps electivo. Se encontró las siguientes complicaciones en la madre: trauma de tejidos blandos o trastorno neurológico de vecindad; ambos han sido de limitada extensión y severidad y la evolución fue favorable. La evolución puerperal ha mostrado anemia (41.7%) e infección puerperal (11.2% de útero y 5% de episiotomía); sin embargo, es necesario aclarar que estas complicaciones han ocurrido casi paralelamente en las pacientes con parto espontáneo que fueron atendidas en el mismo lapso. Se ha revisado 445 historias clínicas pediátricas, observándose de un porcentaje elevado de complicaciones que podría atribuirse al uso del fórceps (20.4%); un porcentaje que llega al 5%, o sea 22 niños, presentaron hipoxia al nacer; los niños se recuperaron ulteriormente con tratamiento. Trauma en el recién nacido, tales como la lesión en piel o cuero cabelludo y la parálisis facial, señalan al fórceps como causa de la injuria; otras consecuencias probablemente atribuidos al fórceps serían los cambios en la configuración craneal y la hemorragia endocraneana. El fórceps ha sido un instrumento útil para solucionar complicaciones en el segundo período del parto; puede considerarse que su uso es insustituible; sin embargo es axiomático aceptar que es un instrumento de utilidad cuando los requisitos de uso no son cumplidos.

The Total Calvarial Remodeling with Transsutural Distraction Osteogenesis of 21 Cases of Craniosynostosis: New, Efficient, Safe and Natural Method in Craniosynostosis Surgery

Introduction: The majority of the present distraction osteogenesis techniques involve local site expansion that only produces localized decompression and affords limited decompression and cosmetic results. We designed a new surgical procedure, total calvarial transsutural distraction osteogenesis (TSuDO). Methods: We performed total calvarial TSuDO surgical procedures in 21 children. The total calvarial TSuDO method consisted of suturectomy and distraction for the sagittal, bicoronal and bilambdoid sutures. Results: The mean surgery duration was 110 ± 16 min, the mean transfusion volume was 38 ± 45 ml, an average 4.1 ± 0.4 distractors were applied with a mean latency period of 3.3 ± 0.9 days. The mean activation period was 45 ± 18 days with a consolidation period of 54 ± 23 days. There were 3 complication cases of early removal of the distractors: 1 boy with transient 6th cranial nerve palsy accompanied by fever and 2 children with mild pus discharge from the distractor sites. Preoperative lumbar puncture pressures decreased significantly after distractor removal secondary to surgery (p < 0.001). The preoperative cranium size increased significantly (p < 0.001). Conclusion: Total calvarial TSuDO is a simple and safe procedure that may produce wide generalized decompression and good cranial configurations that most closely resemble normal skulls.

Paleopathological findings in radiographs of ancient and modern Greek skulls

The skull, when portrayed radiologically, can be a useful tool in detecting signs of systemic diseases and results of pathological growth mechanisms. The aim of this study was therefore to examine, compare, and classify findings in cranial configuration of pathological origin, in modern and ancient skulls. The material consists of 240 modern and 141 ancient dry skulls. Three radiographs for each skull (lateral, anteroposterior, basilar) provide enough evidence for differential diagnoses. Cases of osteoporosis are among the interesting pathological findings. A prevalence of female modern skulls in those determined as osteoporotic skulls is noted. Special interest is placed on the area of the sella turcica and many variations, regarding the shape and texture, are recognized both in ancient and modern skulls. Malignancies and important causes of cranial destruction are identified in both skull collections. Diploid thickening and osteolytic areas appear commonly among ancient remains. Moreover, from the ancient skull collection, one case possibly recognizable as fibrous dysplasia is noted while another case with an unusual exostosis gives rise to many questions. Interpreted with caution, the results of the present study, which can serve as an approach of paleopathology and paleoradiology, indicate similarity trends in cranial configuration of pathologic origin in modern and ancient people. Radiography and cephalometry were the main diagnostic tools used to gather evidence and are evaluated as a quite appropriate method to examine anthropological material and assess the internal structure of skeletal remains since they are non-destructive techniques.

Treatment of nonsyndromic bilateral coronal synostosis using a multiple bone flap rotation-reposition technique.

Brachycephaly is the result of premature fusion of the bilateral coronal suture. Various surgical procedures have been devised to manage brachycephaly, but there is no standard surgical method in brachycephalic treatment. Suboptimal results may be related to the tendency of misdirected bone growth of the remaining cranium in brachycephaly. Therefore, the abnormal growth vectors should be corrected toward a more normal configuration. Between 1997 and 2001, three nonsyndromic brachycephalic patients were treated using the described procedure. The cranial remodeling procedure used consists of supraorbital bar advancement and the rotation-reposition of multiple frontoparietal bone flaps. The frontoparietal bone was cut into seven segments consisting of two upper frontal, two lower frontal, one anterior parietal (T-shaped), and two posterior parietal segments. Each lower frontal segment was rotated 180 degrees and transposed with the corresponding contralateral upper frontal segment. Each upper frontal segment was then transposed to the upper margin of the supraorbital bar. The posterior margin of the frontal segment was not fixed (floating forehead). Anterior parietal and two posterior parietal segments were trimmed and repositioned to the original position. The postoperative follow-up period ranged from 17 to 52 months (mean = 30 months). The cranial configuration obtained after the operation was considerably improved, approaching a normal shape in all cases. The mean length of frontal advancement was 11 mm (range: 7-15 mm). The preoperative mean value of the cephalic index was markedly raised to 104.7 (range: 100-111). The postoperative (at last follow-up) mean value of the cephalic index was 89.2 (range: 86.3-94.3). A better skull contour could be obtained easily by converting abnormally directed long upward and transverse dimensions to short anteroposteriorly directed dimensions. Cranial remodeling using repositioned multiple bone flaps can produce excellent results functionally and esthetically in brachycephaly.

Cranial remodeling to treat craniosynostosis by gradual distraction with a new device

The authors describe their experience in remodeling the shape of the cranium in children with craniosynostosis by using gradual distraction. In half of the cases, a new distraction device developed by the authors was used. Twenty children with craniosynostosis underwent frontoorbital advancement and cranial remodeling assisted by gradual distraction. There were five children with brachycephaly, two with oxycephaly, five with scaphocephaly, two with plagiocephaly, one with combined scaphocephaly and plagiocephaly, and five with trigonocephaly. Four cases were syndromic and 16 were nonsyndromic. The patients ranged in age from 3 to 50 months (mean 14.5 months) at the first surgery. Simulated surgery was first performed on a three-dimensional solid model made of polyurethane, which accurately represented cranial flexibility, to determine the most favorable osteotomy line. Distraction was initiated 1 week postoperatively. The speed and extent of advancement (maximum extension 45 mm) were predetermined on the basis of previously reported criteria and the results of simulated surgery. Postoperatively, the cranial configuration was favorable in all cases. Spontaneous remodeling of irregularities and/or gaps apparent after distraction was found to occur 2 to 5 months after removal of the distraction devices, especially in patients with trigonocephaly or scaphocephaly. No major perioperative complication was observed in any patient. There were minor complications in six of the first 10 cases, including exposure of the device, shaft slippage, and fluid discharge. A new device was developed and used on the last 10 patients treated; it successfully eliminated device exposure and shaft slippage. A 3-year follow-up review confirmed that there was no relapse of advanced bones. Highly satisfactory results were achieved in cases of both syndromic and nonsyndromic craniosynostosis when gradual distraction was performed.

The effects of fronto-parieto-squamosal suture fusion on cranial growth: an experimental study.

Craniosynostoses have been known for at least 20 centuries, but their etiopathogeneses are still unclear. There are three main theories to explain the etiology of craniosynostosis, Moss's theory being the most popular. According to Moss, the development of the neurocranial form is viewed as an integration of the growth of the skull base, the calvarial bones, the meninges, and the enclosed brain. Experimental studies, however, are generally focused on cranial vault suture synostosis. This study aims to demonstrate the effects of cranial base synostosis by performing fronto-parieto-squamosal suture fusion. This fusion affected the whole cranial configuration. These effects were more prominent closer to the cranial vault, increasing at the anterior facial height and the lower facial length, reflected by ventral dislocation of the total face, and increasing of the kyphosis at the cranial base. It has also been demonstrated that synostosis of a junction point near the cranial base affects the vault and the base simultaneously. Any approach that tries to treat the cause, and not the symptom, of synostosis must, then, be based on an understanding of how cranial growth occurs and of how sutural growth processes are related to the totality of cranial growth.

Geographic Variation in Myosorex longicaudatus (Soricidae) in the Southern Cape Province, South Africa

Myosorex longicaudatus is endemic to the southern Cape Province, South Africa, where it occurs in the Langeberg Mountains and widely in the Knysna Forest. Its long tail and set of paired or triple median palatal fissures are the most conspicuous features distinguishing it from both Myosorex varias and Myosorex cafer , but it differs from these species also in cranial configuration. A geographically isolated population in the Boosmansbos Forest (Langeberg Mountains) differs in size, external proportions, and cranial configuration from populations in the Knysna Forest and is described as a new subspecies whose IUCN conservation status should be considered “vulnerable.”.

Growth pattern in the Apert syndrome.

In this paper, we demonstrate that a discernible and unique growth pattern characterizes the Apert syndrome. The keys to understanding Apert newborn measurement values are brain size and cranial configuration. Both true megalencephaly and coronal synostosis are present at birth. Thus, the head is unusually heavy and the cranium is disproportionately high. Mean newborn length and weight are above the normal 50th centile. Of our newborn patients, 16% exceeded 4,000 g in weight. Preterm infants were appropriate or slightly large for gestational age. A biphasic linear growth pattern was found. In childhood, deceleration of linear growth occurs so that most values fall between the 5th and 50th centiles. From adolescence to adulthood, deceleration becomes more pronounced. This 2-step linear growth deceleration results in large measure from rhizomelic shortness of the lower limbs. Puberty takes place within the normal time frame. Although a disproportionate amount of the megalencephaly accounts for the dramatic increase in head height, the widely patent midline calvarial defect, allowing the brain to expand anteriorly into the metopic area, and some increase in the head breadth permit the mean head circumference at birth to normalize slightly above the 50th centile. During the growth period, the head circumference was studied in surgically unoperated Apert patients from the 1960s and earlier. The natural history of the growing cranium consists of gradual deceleration in head circumference from slightly above the 50th centile at birth to within or at -2 SD later on.

Trigonocephaly (metopic synostosis). Clinical, surgical and anatomical concepts.

The clinical features, surgical and anatomical concepts based on an evaluation of the development of the anterior cranial fossa in metopic synostosis are discussed. Thirteen cases of trigonocephaly of infancy during last 4 years are described. Five children were mentally retarded. A more marked triangular cranial configuration with acute nasopterional angle was associated with more severe mental retardation and other unfavorable conditions, including cardiopulmonary disorders and chromosome anomalies. From a quantitative analysis of the anterior cranial fossa development obtained from CT measurement in comparison with values in normal infants, the following procedures appeared to be important: (1) correction of acute nasopterional angle, achieving a flat forehead; (2) forward advancement of both pterions and anterolateral parts of the frontal bones, rebuilding the orbital roof and frontal eminences; (3) shortening of the overgrown midline structure by removal of the bony ridge of the metopic suture. Our procedure on the basis of these measurements for determination of surgical intervention is described.