Assessment of patients with craniosynostosis

2021 ◽  
pp. 697-704
Author(s):  
Nicholas White
Keyword(s):  
Physiological State ◽  
Cranial Sutures ◽  
Skull Growth ◽  
Metopic Suture

The term craniosynostosis denotes the pathological partial or complete absence of one or more cranial sutures which manifests as abnormal skull growth. Although it is often referred to as premature fusion this is misleading as only the metopic suture fuses in the normal physiological state. The remaining sutures change and mature in macroscopic and microscopic form during growth but remain present. This chapter discusses the assessment of patients with craniosynostosis.

Study on Morphometric Features of Coronal Suture Along with it Absence and Craniosynostosis

2021 ◽  
Vol 9 (4) ◽  
pp. 8151-8155
Author(s):  
Khaleel N ◽  
◽  
Angadi A V ◽  
Muralidhar P S ◽  
Shabiya M ◽  
...  
Keyword(s):  
Birth Defect ◽  
Cranial Sutures ◽  
Coronal Suture ◽  
Cranial Suture ◽  
Medical Sciences ◽  
Hot Climate ◽  
Morphometric Features ◽  
Metopic Suture ◽  
Parietal Bones ◽  
The Brain

Background: Cranial sutures are syndesmosis between the cranial bones. The coronal suture is oblique in direction and extends between the frontal and the parietal bones. Craniosynostosis is a rare birth defect that occurs when the coronal suture in the skull fuses prematurely, but the brain continues to grow and develop. This leads to a misshapen head. There are a number of forms of this defect, such as coronal, sagittal, lambdoid, and metopic. Materials and Methods: Total 500 skulls were used for study, coronal suture length measured by thread method, distance between Nasion to bregma and midsupraorbital rim to coronal suture were measured. For finding skull with absence of coronal, sagittal, lambdoid, and metopic suture, we examined many skulls during routine osteology classes of Medical, Dental and other medical sciences students. Around 500 skull observed and we find only one skull with absence of left coronal suture completely. Results: The length of coronal suture was 24.8+1.4cm length, the distance between nasion to bregma was 126.7 +10.25 mm and Midsupraorbital rim to cranial suture was 102.76+8.64mm We have found only one skull with absence of coronal suture. Some of the skulls shows partly fusion of sagittal, coronal sutures. The skull with complete absence of coronal suture showing the features of other sutures clearly and right side of coronal suture is showing the complete suture. The skull was not damaged and it is in perfect condition which was using by students for their osteology study. Conclusion: We found the skull with absence of left coronal suture, which may resulted due to craniosynostosis. It may be due to hot climate in India also might be resulted for absence of suture. KEY WORDS: Birth defect, Skull, Coronal suture, Craniosynostosis.

Compensatory growth following premature closure of the coronal suture in rabbits

1982 ◽  
Vol 57 (4) ◽  
pp. 535-542 ◽  
Author(s):  
William J. Babler ◽  
John A. Persing ◽  
H. Richard Winn ◽  
John A. Jane ◽  
George T. Rodeheaver
Keyword(s):  
Compensatory Growth ◽  
Cranial Sutures ◽  
Coronal Suture ◽  
Skull Morphology ◽  
Content Type ◽  
Premature Closure ◽  
New Zealand White Rabbits ◽  
Skull Growth ◽  
Skull Deformation ◽  
Surgical Release

✓ Premature closure of a cranial suture results in skull deformation characterized by inhibited skull growth in a direction perpendicular to the course of the affected suture. Early surgical release of the closed suture results in a “normal” skull morphology. The present experimental study measured alterations in growth at the transverse cranial sutures that occurred following induced premature closure of the coronal suture and its subsequent release in New Zealand White rabbits. Using roentgenocephalometric methods, growth and morphometric changes were monitored at the frontonasal, coronal, and anterior lambdoid sutures following premature closure of the coronal suture at 9 days of age. The results indicated that premature closure of the coronal suture did not result in compensatory (increased) growth at the other transverse sutures of the vault. In fact, growth at these sutures was significantly reduced. This reduced growth at adjacent transverse sutures was not ameliorated by early surgical release of the fused suture.

scholarly journals Clinical and Genetic Characterization of Craniosynostosis in Saudi Arabia

2021 ◽  
Vol 9 ◽  
Author(s):  
Malak Alghamdi ◽  
Taghreed R. Alhumsi ◽  
Ikhlass Altweijri ◽  
Waleed H. Alkhamis ◽  
Omar Barasain ◽  
...  
Keyword(s):  
Cranial Sutures ◽  
Saudi Population ◽  
Variants Of Unknown Significance ◽  
Patient Demographics ◽  
Genetic Patterns ◽  
Unknown Significance ◽  
Electronic Chart ◽  
Metopic Suture ◽  
Craniofacial Features

Background: Craniosynostosis (CS) is defined as pre-mature fusion of one or more of the cranial sutures. CS is classified surgically as either simple or complex based on the number of cranial sutures involved. CS can also be classified genetically as isolated CS or syndromic CS if the patient has extracranial deformities. Currently, the link between clinical and genetic patterns of CS in the Saudi population is poorly understood.Methodology: We conducted a retrospective cohort study among 28 CS patients, of which 24 were operated and four were not. Clinical and genetic data were collected between February 2015 and February 2019, from consenting patient's families. The electronic chart data were collected and analyzed including patient demographics, craniofacial features, other anomalies and dysmorphic features, operative data, intra cranial pressure (ICP), parent consanguinity and genetic testing results.Results: The most common deformity in our population was trigonocephaly. The most performed procedure was cranial vault reconstruction with fronto-orbital advancement, followed by posterior vault distraction osteogenesis and suturectomy with barrel staving. Genetics analysis revealed pathogenic mutations in FGFR2 (6 cases), TWIST1 (3 cases), ALPL (2 cases), and TCF12 (2 cases), and FREM1 (2 case).Conclusion: Compared to Western countries, our Saudi cohort displays significant differences in the prevalence of CS features, such as the types of sutures and prevalence of inherited CS. The genomic background allows our phenotype-genotype study to reclassify variants of unknown significance. Worldwide, the sagittal suture is the most commonly affected suture in simple CS, but in the Saudi population, the metopic suture fusion was most commonly seen in our clinic. Further studies are needed to investigate the characteristics of CS in our population in a multicenter setting.

Sex- and age-related variations in cranial measurements and suture closure in the Australian sea lion, Neophoca cinerea (Peron, 1816)

2003 ◽  
Vol 51 (5) ◽  
pp. 463 ◽  
Author(s):  
R. Tedman
Keyword(s):  
Sexual Dimorphism ◽  
Age Determination ◽  
Cranial Sutures ◽  
Neophoca Cinerea ◽  
Age Related ◽  
Cranial Measurements ◽  
Condylobasal Length ◽  
Skull Growth ◽  
Early Closure ◽  
Suture Fusion

A total of 65 skulls of the Australian sealion, Neophoca cinerea, was examined to investigate the extent to which sexual dimorphism is reflected in cranial dimensions (n = 32) and skull growth, and to determine whether cranial sutures (n = 18) can be useful in age determination. All adult skull dimensions studied display significant sexual dimorphism. Skull growth ceases close to 4–7 years of age for females (Suture Fusion Rating, SFR 25–34) but skull growth in males continues until at least 16 years of age. In animals with a SFR ≥� 25, male skulls have a minimum condylobasal length of 272 mm, whereas female skulls have a maximum condylobasal length of 259 mm. The relatively early closure of the cranial vault sutures (cessation of brain growth) is balanced by the continued growth of the bony projections that provide muscle attachment (e.g. mastoid width). The later fusion of the snout and palate sutures corresponds with the continued growth of the snout and palate to match the prolonged growth of the mandibles. The upper sixth postcanine tooth was present in 43% of the adult female skulls, but only 15% of the adult male skulls. The data suggest that it may be possible to determine age(s) from examination of the sequence of fusion of cranial sutures as well as by calculation of an overall suture fusion rating for the skull.

scholarly journals Craniosynostosis: Clinical Presentation and Outcome of Surgical Treatment

2020 ◽  
Vol 24 (3) ◽  
Author(s):  
AURANGZEB KALHORO ◽  
A. SATTAR M. HASHIM
Keyword(s):  
Intracranial Pressure ◽  
Perioperative Management ◽  
Major Complication ◽  
Cranial Sutures ◽  
Raised Intracranial Pressure ◽  
Surgical Expertise ◽  
Metopic Suture ◽  
Age Range ◽  
Strip Craniectomy

Objective:  Craniosynostosis presents sclerosis cranial sutures, ossification and fusion involving the vault of the base, sutures involved can be one or more sutures. This study aimed to determine the surgical outcome of craniosynostosis. Material and Method:  The prospective study was conducted at the neuro spinal and cancer care institute, Karachi. Patients presented with sagittal, metopic, unicoronal, lambdoid, bicoronal craniosynostosis were included in the study, while patients with coagulopathy, previously operated cases were excluded from the study. Results:  We had 26 children in our study, age range about 1–3 years. Patients were cleaved into groups depending on their age. Most of the children 15 (57.6%) were in 1–2 years age group and 11 (42.3%) were in 2-3 years of age. Boys were 18 (69.2%) and girls were 8(30.7%). Coronal 11 (42.3%) was the most common suture involved, followed by sagittal 9 (34.6%). Lambdoid suture 3 (11.5%), metopic 2 (7.6%) and 3 (11.5%) case presented with raised intracranial pressure with multiple sutures closed involved. Strip craniectomy was done in all cases. We did bicoronal flap and Scalp flap turned into a supraorbital region while in metopic suture Fronto-orbital advancement and remodeling approach were used. No major complication was observed. Conclusion:  Cases which are managed early age have given good acceptable results in follow up, proper surgical expertise, perioperative management of temperature, blood loss, relieving the restriction of sutures and normalizing raised intracranial pressure can decrease the morbidity and mortality.

scholarly journals Trigonocephaly associated with myelomeningocele in infant: Case report

2021 ◽  
Author(s):  
Julia Gabriela Oliveira Marchiori ◽  
Jennyfer Paulla Galdino Chaves ◽  
Maria Cecilia Closs Ono ◽  
Adriana Keijiro Maeda
Keyword(s):  
Case Report ◽  
Three Dimensional ◽  
Cranial Sutures ◽  
Reconstruction Surgery ◽  
Recommended Treatment ◽  
Fetal Movements ◽  
Dimensional Reconstruction ◽  
Early Closure ◽  
Metopic Suture ◽  
Infant Case

Context: Trigonocephaly is a type of craniostenosis due to the early closure of the metopic suture. His diagnosis is eminently clinical, but imaging tests are essential. The recommended treatment is surgical and should preferably be performed at the age of 3 to 9 months. Association between craniosynostosis and neural tube defect (NTDs) is rare and was sometimes considered as mere coincidence. However, Martinez-Lage et al. hypothesize that there is a causal relationship between these malformations since myelomeningocele reduces intracranial pulse pressure, which stimulates the early closure of cranial sutures. For Graham et al. the restriction of fetal movements in NTD carriers predisposes to craniosynostosis, as they are likely to keep the skull fixed against the mother’s pelvic bones. Case report: Male patient, 11 months, with West Syndrome. He congenitally presented myelomeningocele, and this deformity was corrected on his first day of life. He subsequently developed a keel-shaped forehead, protrusion of metopic suture, and hypertelorism. The diagnosis of trigonocephaly was ratified by computed tomography of the skull with three-dimensional reconstruction. Surgery was performed at 11 months. The access chosen was coronal and there was a wide exposure of the calvarium. Then all the metopic suture was removed through a bifrontal craniotomy, which allowed the remodeling of the frontal bone. Conclusions: It is necessary to make a diagnosis and early treatment of both comorbidities since delays in the conduct can result in disastrous consequences. In addition, there is a need to conduct more research to elucidate the interrelationship between craniosynostosis and NTD.

scholarly journals Open suturectomy management in craniosynostosis of bilateral coronal and metopic suture

2021 ◽  
Vol 4 (1) ◽  
pp. 15-18
Author(s):  
Marthinson Andrew Tombeng ◽  
I Wayan Niryana
Keyword(s):  
Computed Tomography ◽  
Rare Case ◽  
Surgical Complication ◽  
Male Infant ◽  
Timing Of Surgery ◽  
Cranial Sutures ◽  
Head Shape ◽  
Operative Complications ◽  
Abnormal Head ◽  
Metopic Suture

Craniosynostosis is a cranial deformation that is characterized by the premature fusion of one or more of the cranial sutures. Synostosis of multiple suture is a rare case and can be treated with open suturectomy procedure which is one of the surgical management by removing the fused suture with the purpose to allow the constricted area to expand with the growing brain. We present a case of a 2-month-old male infant with abnormal head shape since birth with a non-contrast 3D computed tomography (CT) scan of the head confirmed closure of the bilateral coronal and metopic suture. Open suturectomy was performed with no post operative complications. Open suturectomy technique can be performed in the management of multisutural craniosynostosis as indicated. The diagnosis of which suture are affected, the timing of surgery, and the prevention of surgical complication such as excessive blood loss are the important factors need to be considered.

Advances in Microprobe Analysis Applied to Muscle, Nerve and Gland

1982 ◽  
Vol 40 ◽  
pp. 404-407
Author(s):  
T. E. Hutchinson ◽  
D. E. Johnson ◽  
A. C. Lee ◽  
E. Y. Wang
Keyword(s):  
Biological Tissue ◽  
Microprobe Analysis ◽  
Physiological State ◽  
External Environment ◽  
Physiological Relevance ◽  
Muscle Nerve ◽  
Technique Development

Microprobe analysis of biological tissue is now in the end phase of transition from instrumental and technique development to applications pertinent to questions of physiological relevance. The promise,implicit in early investigative efforts, is being fulfilled to an extent much greater than many had predicted. It would thus seem appropriate to briefly report studies exemplifying this, ∿. In general, the distributions of ions in tissue in a preselected physiological state produced by variations in the external environment is of importance in elucidating the mechanisms of exchange and regulation of these ions.

Conformational characterization of nucleosome structure by Electron Microscopy

1993 ◽  
Vol 51 ◽  
pp. 194-195
Author(s):  
Gregory J. Czarnota
Keyword(s):  
Electron Microscopy ◽  
Structural Changes ◽  
Physiological State ◽  
Gene Activation ◽  
Three Dimensional ◽  
Macromolecular Complex ◽  
Ultrastructural Studies ◽  
Ionic Environment ◽  
Nucleosome Structure ◽  
Dimensional Reconstruction

Chromatin structure at the fundamental level of the nucleosome is important in vital cellular processes. Recent biochemical and genetic analyses show that nucleosome structure and structural changes are very active participants in gene expression, facilitating or inhibiting transcription and reflecting the physiological state of the cell. Structural states and transitions for this macromolecular complex, composed of DNA wound about a heterotypic octamer of variously modified histone proteins, have been measured by physico-chemical techniques and by enzyme-accessibility and are recognized to occur with various post-translational modifications, gene activation, transformation and with ionic-environment. In spite of studies which indicate various forms of nucleosome structure, all current x-ray and neutron diffraction studies have consistently resulted in only one structure, suggestive of a static conformation. In contrast, two-dimensional electron microscopy studies and three-dimensional reconstruction techniques have yielded different structures. These fundamental differences between EM and other ultrastructural studies have created a long standing quandary, which I have addressed and resolved using spectroscopic electron microscopy and statistical analyses of nucleosome images in a study of nucleosome structure with ionic environment.

Stimulus + Physiological State = Behavior

1980 ◽  
Vol 25 (3) ◽  
pp. 265-266
Author(s):  
JACQUELINE LUDEL
Keyword(s):  
Physiological State ◽  
State Behavior
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