Temporal mapping of the closure of the anterior fontanelle and contiguous sutures using computed tomography,in silico models of modern infants

The aim of this study is to quantify and statistically model the age-related decline in the fibrous connective tissue interface of the anterior fontanelle in modern Australian infants, using three-dimensional, semi-automated computed-assisted design protocols. Non-linear regression with variance models, using power functions, combined with quantile regression of the 5th and 95th population percentiles, were utilised to assess absolute anterior fontanelle surface area (AFSA) as a function of age, using multi-slice cranial computed tomography scans obtained from 256 infants aged < 30 months (males: n = 126, females: n = 109) from Brisbane children’s hospitals. Normalised AFSA (NFSA), standardised for variation in cephalic size, followed a progressive decline from birth, the greatest velocity change occurring between the 3–6 and 6–9 month cohorts. Growth of the neurocranium is the most significant within the first 8 months postpartum, with a mean increase of 19.03 mm in maximum cranial length and 10.04 mm in breadth. Directionality of fontanelle closure, quantified using spline curves refutes fundamental assumptions that the anterior fontanelle is consistent with a quadrilateral, and contiguous sutures exhibit constant velocity of closure. The present study provides normative values for fontanelle size and diameters as well as new predictive non-linear models for age substantiation, screening of developmental abnormalities and indicators of suspected child maltreatment in modern infants aged birth to 30 months.     

Lethal persistent pulmonary hypertension of the newborn in Bohring–Opitz syndrome

Bohring–Opitz syndrome (BOS) is a rare disease with a number of characteristic features, including hypertelorism, prominent metopic suture, exophthalmos, cleft palate, abnormal posture, and developmental retardation. Here, we report a BOS patient presenting with lethal persistent pulmonary hypertension of the newborn (PPHN) and inspiratory respiratory failure. The female infant was treated with nitric oxide and vasodilator, which did not improve her condition. The inspiratory respiratory failure required management with deep sedation. She died on postnatal day 60 due to progressed heart failure. Whole exome sequencing revealed de novo mutation in the ASXL1 gene, c.1934dupG, p.Gly646TrpfsTer12.     

Restricted growth at the frontonasal suture: Alterations in craniofacial growth in rabbits

Apposition of bone at the sutural margin is generally thought to be a compensatory adjustment to growing soft-tissue organs such as the brain or eyes within the skull. The frontonasal suture which is located at the interface between the cranial and facial skeletons is a site of extremely active growth in the young rabbit. Recently, we showed that premature closure of a cranial suture, the coronal suture, can alter the growth not only at the adjacent frontonasal suture but also of the basicranium and midface. This study examines the effects of restricted growth at the frontonasal suture on both growth at adjacent cranial sutures and linear growth of the basicranium and midface. Thirty newborn New Zealand White rabbits were subdivided into experimental and sham-treated groups of equal size and distribution for sex and birth weight. At 9 days of age, the frontonasal suture of each experimental animal was immobilized by bilateral application of methyl-cyanoacrylate adhesive across the frontonasal suture. Growth and morphometric changes were monitored by radiocephalometric methods through 120 days of age by bilateral implantation of radiopague markers on each side of frontonasal, coronal, and anterior lambdoid sutures. Results indicate that restricted growth at the frontonasal suture results not only in a significant shortening of the midface but also in significant decreases in growth at the coronal and internasal sutures. Growth at the interfrontal and sagittal sutures is increased. Furthermore, growth at the anterior portion of the nasal bones is significantly increased, thereby offsetting a portion of the decreased nasal bone length resulting from frontonasal restriction.     

At Birth,Tarsiers Lack a Postorbital Bar or Septum

Among primates, partial or complete posterior closure of the orbit has been widely accepted as a shared derived characteristic justifying an exclusive tarsier‐anthropoid clade, while some regard the tarsier lateral orbit as an elaborated postorbital bar (POB). To test these competing hypotheses while minimizing the confounding effect of tarsier orbital hypertrophy, we compared tarsiers and other primates at early (fetal and newborn) ages using dissection, micro‐CT scans and soft tissue histology. Our findings demonstrate unanticipated variation in the anatomy and development of the zygomaticofrontal (ZFA) articulation, which forms the orbit's lateral framework. Tarsiers uniquely exhibit a combination of two features: absence of a pre‐ and peri‐natal frontal spur to join with the zygomatic to form the ZFA; and, the spur's substitution by an elaborate ligament, which envelops the eye laterally as an expansive postorbital membrane (POM) that merges with the anterolateral fontanelle of the lateral cranial vault. In lacking a frontal spur, tarsiers are distinct from strepsirhines, while the ligamentous structure of the POM distinguishes its ZFA from that of anthropoids, which is a typical facial suture at and prior to birth. The POM of tarsiers may be thought of as an accessory fontanelle, a structural compromise that provides flexible stability and spatial separation of bones while anticipating rapid postnatal growth of an enormously enlarged eye. We regard the tarsier POM as part of a neomorphic eyeball hypertrophy complex, and reject the hypothesis of derived homology of the postorbital septa of adult tarsiers and anthropoids on histological, developmental and functional grounds. © 2013 Wiley Periodicals, Inc.     

后纵裂入路切除侧脑室三角区肿瘤的解剖学

目的 通过解剖学研究,探索顶枕部注入上矢状窦桥静脉的分布规律,比较各种至侧脑室三角区的经纵裂入路在外侧方向暴露范围,为临床设计、选择和改良手术入路提供解剖学依据。 方法 取4%甲醛固定、乳胶灌注动静脉血管的国人头颅标本5具,开颅并保留人字缝作为测量标志,观察后囟点前后桥静脉的分布情况,总结顶枕部桥静脉在后囟点前、后的分布规律。取4%甲醛固定、乳胶灌注的国人头颅标本5具,导航辅助下模拟同侧和对侧的经胼胝体、经扣带回、经楔前叶入路共6种手术入路的手术路径,以导航棒模拟手术视线,以导航棒与正中矢状面所成的角度代表手术入路在外侧方向的暴露范围,记录、计算各入路在外侧方向的暴露范围,并相互比较。 结果 没有桥静脉于后囟点和窦汇之间注入上矢状窦。在后囟点前方距离为s（s代表后囟点到窦汇的距离）的范围内注入上矢状窦的桥静脉,平均每个标本左侧有（1.4±0.5）条,右侧（1.6±0.5）条,双侧共（3.0±0.6）条。在同侧入路中,外侧方向的暴露范围经胼胝体入路＜经扣带回入路＜经楔前叶入路;对侧入路与同侧入路相同,暴露范围经胼胝体入路＜经扣带回入路＜经楔前叶入路。在切开脑组织位置相同的同侧和对侧入路中,外侧方向暴露范围同侧入路＜对侧入路。所有比较结果差异均具有统计学意义。 结论 顶枕部桥静脉集中分布在后囟点之前,后纵裂入路采用人字缝后方开颅较其前方更为安全。在经纵裂入路中,向外侧方向的暴露,经楔前叶入路优于经扣带回入路、优于经胼胝体入路,对侧入路优于同侧入路;对侧入路有利于保证导航准确性。对侧后纵裂经大脑镰经楔前叶（CITT）入路在功能保护、增加外侧方向暴露、保证导航准确性等方面有一定优势。     

Autosomal dominant craniosynostosis of the sutura metopica

Trigonocephaly due to craniosynostosis of the sutura metopica was found in two sibs with normal intelligence. Both were microcephalic. The father had a sloping forehead and possibly partial metopic craniosynostosis. The paternal grandfather had a bony ridge at the upper half of the metopic suture without significant head deformity. A paternal sister was possibly also affected. None of the affected persons showed significant other anomalies. Craniosynostosis of the metopic suture may be an autosomal dominantly inherited disorder, not associated with functional brain or other abnormalities.     

Duplication 7p in a family with t(7;11): association with anomalies of the anterior cranial base

Duplication 7p has been observed in association with several balanced translocations. Of eight previously recorded cases, only one exhibited duplication of the entire short arm of chromosome 7. We report on a newborn infant with multiple congenital anomalies and an abnormal chromosome constitution: 46,XX,-11,+der(11),t(7;11) (p11.1;p15.5)mat. The proposita appeared to possess an isolated duplication of the entire short arm of 7. The patient died at age 4 days because of respiratory complications of meconium aspiration. Clinical and postmortem findings included craniocerebral asymmetry, craniosynostosis of a lambdoid suture, arhinencephaly, hypertelorism, anomalies of the ethmoidal portion of the cranial base, large anterior fontanelle, low set ears, ventricular septal defect (VSD), dysplastic tricuspid and pulmonic valves, hypoplastic genitalia, bilateral dislocated hips, and other minor limb malformations.     

Trigonocephaly in a boy with paternally inherited deletion 22q11.2 syndrome

Deletion 22q11.2 syndrome is a well-known contiguous gene syndrome, for which the list of findings is extensive and varies from patient to patient. We encountered a unique patient who had a familial 3-Mb deletion 22q11.2 associated with trigonocephaly derived from craniosynostosis of the metopic suture. Almost all the symptoms of the patient, including polymicrogyria, microcephaly, facial abnormalities, internal anomalies, seizures, and mental retardation, were compatible with deletion 22q11.2 syndrome, except for synostosis of the metopic suture. This is the first report of a relationship between deletion 22q11.2 syndrome and trigonocephaly. Craniosynostosis of the metopic suture might be a minor complication of deletion 22q11.2, although coincidental occurrence cannot be ruled out.     

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 de fect, 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. © 1993 Wiley-Liss, Inc.     

Fetal valproate syndrome: is there a recognisable phenotype?

Four infants who were exposed to sodium valproate or valproic acid during pregnancy are described. Common facial features in the three surviving infants include epicanthic folds, a flat nasal bridge, a broad nasal base, anteverted nostrils, a shallow philtrum, and a thin upper lip with a thick lower lip. Ridging of the metopic suture, congenital heart defect, postaxial polydactyly, and hypospadias were additional features in individual cases. In agreement with previous authors, we feel that there is a distinctive 'fetal valproate' phenotype.     

Suture formation,premature sutural fusion,and suture default zones in Apert syndrome

On the basis of our studies, we postulate that suture formation in Apert syndrome is related to the relative maturity of abutting calvarial bones. The fused coronal suture, a consistent manifestation at birth, develops first because the ossification centers of the frontal and parietal bones are in intimate contact early during intrauterine life. Calvarial immaturity and the megalencephalic brain characteristic of the Apert syndrome appear to work in concert to produce a widely patent midline calvarial defect extending from the glabella to the posterior fontanelle. Because sagittal growth in the coronal sutures cannot take place, the megalencephalic brain grows upward and laterally, and bulges forward through the midline defect. The defect fills in by coalescence of bony islands without proper suture formation because the gap to be bridged is so great that the time window for developing sutural interdigitations may have closed. Other sutures, such as the lambdoid, squamosal, and sphenotemporal, develop with normal interdigitations because abutting bone margins are in close enough proximity to permit suture formation. © 1996 Wiley-Liss, Inc.     

The internal structure of bony tissue of a human metopic suture by Soft X-ray

A complete metopic suture was found in the frontal bone of a 79 years old male cadaver. Compact bone matrices were found in the border region of the metopic suture using a Soft X-ray apparatus. The coronal and sagittal sutures were also complete. The appearance of these complete cranial sutures may be due to continuous bone restructuring and resorption in the border region of sutures during brain development of brachy crany.     

An anatomical study of wormian bones from the eastern part of India: is genetic influence a primary determinant of their morphogenesis?

Over the years a number of investigators have analysed the morphology of wormian bones in different population groups across the world. There have been significant variations between findings reported in these studies, and this has prompted researchers to focus on the influence of genetic factors on the morphology of these bones. In the light of the above observation, we considered it justified to conduct anatomical studies on wormian bones in different population groups; hence, we undertook the present study to look into the morphological details of these bones among a population in the eastern part of India. We observed a total of 120 adult dry human skulls of unknown age and sex, and noted the anatomical details of wormian bones when present. It was observed that wormian bones were present in 45 % of skulls, and that 30 % of skulls had more than one wormian bone. We also found that 2.5 % of the skulls had ten or more wormian bones, which is considered as pathognomonic. Maximum incidence (53.33 %) was observed at the lambdoid suture and minimum incidence at the bregma and metopic suture (0.61 % in each case). We noted a high incidence (21.21 %) of Inca bone/lambdoid ossicle, and bilaterally symmetrical wormian bones were present in 12.5 % study skulls. There were statistically significant (P < 0.05) variations between the findings of the present study and values reported in previous studies conducted in other regions of India and different parts of the world. Our observations favour the view that genetic influence primarily determines the morphology of wormian bones.     

Wormian bones: A review

Wormian bones are abnormal ossicles that develop from extra ossification centers within the cranium. They are most frequently located in the lambdoid suture or the coronal suture, and have been seen in the fontanelles, particularly the posterior fontanelle. It is unclear at this time exactly how or why they are formed, although genetic as well as environmental factors have been proposed. Their initial formation is thought to be caused by a degree of dural strain and increased sutural width. These conditions can result from mechanically induced stress due to intentional deformation like that practiced in ancient cultures, premature sutural closure, or from reduced skull ossification as seen in metabolic bone diseases. The cause of the malformation can have an influence on the number and location of Wormian bones. Clinically, Wormian bones are used as markers in the diagnoses of many autosomal dominant genetic disorders, namely, craniosynostosis and osteogenesis imperfecta. Clin. Anat. 26:922–927, 2013. © 2013 Wiley Periodicals, Inc.     

“Münster correlation” in temporal bone: surgical relevance of an anatomical study

Resection of the temporal bone to various degrees provides different levels of access to lesions of the posterior fossa. However, precise distances to the petrosal bone are still not clearly described. We evaluated the different distances of temporal bone landmarks in order to assess their variations and the possible correlations between them. We also evaluated the surgical relevance of the possible correlations. An anatomical study was performed on 60 temporal bones from 60 human cadavers. All bones contained an adequate portion of the petrous apex and attached fossa dura. We analyzed the variation in different distances between landmarks and also the correlations between different measured distances. There was a statistically significant correlation between the distance between the inferior axial plane of the posterior semicircular canal to the superior plane of jugular bulb and the thickness of the mastoid cortex at the M point (M point was defined by the authors). This correlation is important for estimating the height of the jugular bulb during a lateral suboccipital craniotomy in cerebellopontine angle surgery. The Münster correlation is an easy and safe method for estimating the height of the jugular bulb in cerebellopontine angle surgery.     

Trigonocephaly in rabbits with familial interfrontal suture synostosis: the multiple effects of premature single-suture fusion

Previous studies from our laboratory have characterized the craniofacial morphology and growth patterns of an inbred strain of rabbits with autosomal dominant coronal suture synostosis. A number of rabbit perinates from this colony have been collected sporadically over a 5-year period with premature interfrontal suture synostosis. The present study describes the very early onset of craniofacial dysmorphology of these rabbits and compares them to similar-aged normal control rabbits. A total of 40 perinatal New Zealand White rabbits were used in the present study. Twenty-one comprised the sample with interfrontal suture synostosis and ranged in age from 27 to 38 days postconception (term = 31 days) with a mean age of 33.53 days (+/-2.84 days). Nineteen rabbits served as age-matched, normal controls (mean age = 33.05 days +/-2.79 days). Lateral and dorsoventral radiographs were collected from each rabbit. The radiographs were traced, computer digitized, and 12 craniofacial measurements, angles, and indices were obtained. Mean measures were compared using an unpaired Student's t-test. All synostosed rabbits were stillborn or died shortly after birth. Grossly, these rabbits exhibited extreme frontal bossing, trigonocephaly with sagittal keeling, and midfacial shortening. No somatic anomalies were noted. Radiographically, rabbits with interfrontal suture synostosis had significantly (P < 0.05) narrower bifrontal widths, shorter cranial vault lengths, kyphotic cranial base angles, and different cranial vault indices (shapes) compared to controls. Results reveal severe and early pathological and compensatory cranial vault changes associated with premature interfrontal suture synostosis in this rabbit model. The 100% mortality rate noted in this condition may be related to the inheritance of a lethal genetic mutation or to neural compression from reduced intracranial volume. Results are discussed in light of current pathogenic hypotheses for human infants with premature metopic suture synostosis.     

Incidence of metopism in the Lebanese population

This study was carried out on 968 skull X-rays to determine the incidence of the metopic suture in the Lebanese population. Complete and incomplete metopism was present in 0.82% and 0.93% of cases, respectively, leading to an overall incidence of 1.75%. In both categories, the incidence was relatively higher in males (1.84%) than in females (1.62%). Persons living in rural areas had a higher incidence of complete and incomplete metopism compared to persons living in urban areas, with ratios of 4:1 and 4:2, respectively. Other findings included an absence of frontal sinuses in all but one skull with complete metopism, accessory ossicles in the majority of cases of complete metopism (7/8), and hydrocephalus in one case. Medical records did not reveal any other associated diseases or abnormalities. Despite the low incidence of metopic suture in the Lebanese population, in X-ray diagnosis of fractures of the frontal bone, metopic suture must not be ruled out.     

Three‐dimensional computed tomography reconstruction measurements of acetabulum in Chinese adults

The present study was designed to define the morphological dimensions of the acetabulum in normal Chinese adults and to statistically compare these data with the available data worldwide. This information is important for the diagnosis of dysplasia and treatment of total hip arthroplasty. In this study, the gender and bilateral differences were evaluated. One‐hundred CT scans of patients were retrospectively studied. These individuals showed no signs of developmental disturbances in either of the hip joints. Thirty‐five morphometric parameters of the acetabulum were measured. The size of acetabulum was evaluated by the acetabular perimeter, anteroposterior diameter, vertical diameter, the depth and width of fossa ovalis in both transaxial and coronal plane. The parameters of acetabular orientation were the acetabular angle, anterior center edge angle, neck shaft angle, acetabular anteversion, and abduction angle. The coverage of acetabulum was examined as the, acetabular head index, center edge angle, the distance between the femoral head, and acetabulum. Gender and bilateral differences were analyzed for each parameter, and compared with available worldwide data. The results showed statistically significant differences between the Chinese genders and also between the Chinese and other human races in some parameters. In conclusion, gender, bilateral and racial differences exist in the morphology of acetabulum. The data may be helpful for the design of total hip arthroplasty for the Chinese population. Anat Rec, 297:643–649, 2014. © 2014 Wiley Periodicals, Inc.     

Navigational area of the cranio-orbital foramen and its significance in orbital surgery

The cranio-orbital foramen (COF) is located on the lateral wall of the orbit. It is a potential source of hemorrhage during deep lateral orbital dissection, since it functions as an anastomosis between the lacrimal artery and the middle meningeal artery. The aim of this study was to guide and facilitate the surgical procedures in the orbit, so as to determine a navigational area and the precise location of the COF and to standardize certain anatomical marks. The navigational area of the COF and topographical features were studied in 75 craniums with presented COF. 33 bilateral main COFs, 41 (18 on the right, 23 on the left) unilateral main COFs at the main cranium and 19 accessory COFs were studied for their navigational features on the orbit. The distances between the COF and the fronto-zygomatic suture, supraorbital notch, lateral angle of the superior orbital fissure (SOF) and Whitnall’s tubercle were measured. The mean distance of the COF from the fronto-zygomatic suture, supraorbital notch, lateral angle of the SOF and Whitnall’s tubercle was 26.3, 37.3, 92 and 27.1 mm, respectively. For the navigational area signs of the COF, areas of the orbit that form the transversal and vertical lines are generated on the reference points. Whilst the upper outer area of the orbit contains a potential bleeding risk, the bottom section of the outer column is identified as safe for the surgical operations of the lateral orbital wall. The fronto-zygomatic suture and Whitnall’s tubercle are recommended as the most reliable navigational landmarks for identifying the COF. Hence, the transversal and vertical orientation of the COF should be mastered by the surgeons reconstructing the anterior base of the skull and the orbit.