Craniosynostosis are of several types; which include sagittal synostosis, frontal plagiocephaly and metopic synostosis. Signs and symptoms of craniosynostosis include the following:

• Shape of the head is unusual
• Increase in intracranial pressure
• Slow or no growth in the head size as the baby grows
• Affected sutures have a raised and hard ridge
• No fontanelle on the skull of newborn [8]

• Surgical Procedure

  A surgical procedure to advance the forehead is usually done before the baby is 6 months old. [craniofacialcenter.ucsf.edu]

  Your plastic surgeon will examine your child, determine if craniosynostosis is likely and discuss what types of surgical procedures are options for your child. [plasticsurgery.org]

  Blood transfusion was needed in 100% (n = 66) of the open surgical procedures but in only 21% (n = 26, 95% CI 15-30) of the endoscopic procedures. [ncbi.nlm.nih.gov]

  The prognosis for surgical correction of nonsyndromic cranisynostosis is good. Most cases of nonsyndromic craniosynostosis can be corrected with one surgical procedure. [clinicaladvisor.com]

• Short Stature

  Its phenotype includes uni- or bicoronal synostosis, short stature, facial dysmorphism and variable anomalies of the hands and feet. Due to its poor sonographic manifestation a prenatal diagnosis of SCS is challenging. [ncbi.nlm.nih.gov]

  stature, obesity, variable syndactyly, heart and limb defects Autosomal recessive Cloverleaf skull Can be associated with above syndromes, thanatophoric dysplasia Autosomal dominant Baller-Gerold syndrome Radial, carpal, and digital aplasia Autosomal [medlink.com]

• Weakness

  There also is a risk of injury to the underlying brain that can cause significant neurological abnormalities, including weakness and seizures. Outcome Nearly all children operated on for premature closure come out with an improved appearance. [uclahealth.org]

  Frequently also there is a weakness or loss of convergence. Nystagmus which may be present is of the ocular type. Headache is an almost constant complaint. It tends to disappear about the age of 8 years and comes back with severity in adult life. [ijo.in]

  Further, after surgery, many children can be extremely weak and be in need of services to help with recovery. The proper amount of therapy can help children return to their preoperative state and shorten postoperative recovery periods. [dovepress.com]

  In addition, infants have limited ability to move their head in the first few months of life because of their weak neck muscles. The presence of torticollis may further pre-dispose to positional plagiocephaly [Figure 3]. [ijps.org]

• Multiple Congenital Anomalies

  Noonan syndrome (NS) is a multiple congenital anomaly syndrome caused by germline mutations in genes coding for components of the Ras-mitogen-activated protein kinase (RAS-MAPK) pathway. [ncbi.nlm.nih.gov]

• Wound Infection

  Risks encompass those related to general anesthesia, wound infection, loss of blood and transfusion, which is often required. These risks are small. Most severe complications and deaths from surgery for craniosynostosis are related to blood loss. [uclahealth.org]

• Hypertension

  CONCLUSIONS: Headaches are common after cranial vault remodeling but are not very predictive of who will need revision surgery for intracranial hypertension. [ncbi.nlm.nih.gov]

  The frequency of increased intracranial hypertension and the risk of mental impairment depend on the age of the child and the type of craniosynostosis. [doi.org]

• Heart Disease

  Children with congenital heart disease undergoing noncardiac surgery have an increased risk of perioperative morbidity and cardiac arrest. [ncbi.nlm.nih.gov]

  disease Saethre–Chotzen TWIST/FGFR-2 AD Coronal, lamboidal±metopic (mild) Towering forehead, low set hairline, facial asymmetry with septal deviation Ptosis upper eyelid Cutaneous syndactyly Normal intelligence Syndrome Gene mutation and inheritance [academic.oup.com]

  Zschiesche, Sagittal craniostenosis, congenital heart disease, mental deficiency and various dysmorphies in two sibs ? a ?new? syndrome?, European Journal of Pediatrics, 10.1007/BF00647293, 146, 1, (75-78), (1987). Michael J. Noetzel, Jeffrey L. [doi.org]

• Visual Impairment

  That condition is called cranial stenosis and it often results in increased intracranial pressure leading possibly to visual impairment, sleeping impairment, eating difficulties, or an impairment of mental development combined with a significant reduction [chrichmond.org]

  There may be visual impairment, with an increased risk of developmental delay and mental retardation. [doi.org]

  impairment, deafness and cognitive deficits 4, 11, 12, 14, 18). [ncbi.nlm.nih.gov]

  When the child is more than three years old, more symptoms and signs can be observed: headache, vomiting, seizures, visual impairment. No neurological deficit and no papilledema or optic atrophy was observed. [biomedsearch.com]

  vomiting, visual disturbance, bulging of the anterior fontanel, altered mental status, papilledema and headache.[18] The main risks of prolonged elevated intracranial pressure may include cognitive impairment and impaired vision through prolonged papilledema [en.wikipedia.org]

• Blurred Vision

  He did not complain of headache, vomiting, or blurred vision. Three-dimensional computed tomography (CT) imaging was suggestive of craniosynostosis and lacunar skull (lückenschädel). [ncbi.nlm.nih.gov]

  The symptoms of ICP usually begin with: a persistent headache, which is usually worse in the morning and last thing at night vision problems, such as double vision, blurred vision or a "greying out" of vision an unexplained decline in the child's academic [hse.ie]

  The symptoms of ICP usually begin with: a persistent headache, usually worse in the morning and last thing at night vision problems, such as double vision, blurred vision or a 'greying out' of vision an unexplained decline in the child's academic abilities [your.md]

• Torticollis

  An assessment of neck movements also should be made to confirm or rule out the presence of torticollis. Infants with torticollis have some limitation of active rotation of their heads away from the flattened side of the occiput. [pediatrics.aappublications.org]

  Physical examination of a craniosynostotic baby involves careful palpation of the skull for suture ridges and soft spots (fontanelles), checking for abnormal neck position (torticollis) and for other deformities in the body. [skullbaseinstitute.com]

  Physical therapy may be needed to treat a tight neck muscle called torticollis. Some parents elect to use a molding helmet to improve the shape of their child’s head. Our specialists can offer more information about this. [hopkinsallchildrens.org]

  True plagiocephaly (ie, caused by craniosynostosis) often results in asymmetric orbits and is to be differentiated from positional plagiocephaly, which is due to torticollis or positioning the infant predominantly on one side and does not result in asymmetric [msdmanuals.com]

• Fracture

  Another possible explanation for the high occurrence of coxa vara is the loss of reduction after initial fracture reduction of implant failure in unstable fractures. [physio-pedia.com]

  The author demonstrates a case in which fracture of the femoral neck on one side healed in varus position. Varus osteotomy was subsequently done on the other side to equalize both hips. [ncbi.nlm.nih.gov]

  When there is atresia of the optic foramen as found out by radiological examination enlarging of the optic canal by means of an incision just above the brow and beneath the periosteum may be clone removing the fractured bits and incising the dural sheath [ijo.in]

  Sometimes bone grafts are placed to keep the out-fractured bones apart. What is multiple suture synostosis? When all the sutures are fused, the children are operated on as soon as possible. [childrens.memorialhermann.org]

  Common Problems With Hips Aseptic or Avascular necrosis Congenital Dislocation Perthes’ disease Aplasia of the acetabulum Coxa valga Coxa vara Osteoarthritis Dislocation (see image above of simple dislocation) Bursitis Legg-Perthes disease Bone tumor Fracture [healthpages.org]

• Acrocephaly

  Acrocephaly: Acrocephaly occurs with combined premature fusion of the lambdoid, coronal, and sagittal sutures and can be seen in Crouzon and Apert syndromes. The anterior skull overgrows, causing the cranium to slope from front to back. [clinicaladvisor.com]

  Series of neurosurgical interventions were carried out, principally for acrocephaly and posterior plagiocephaly. The most common achondroplasia mutation, a p.Gly380Arg in the fibroblast growth factor receptor 3 (FGFR3) gene, was detected. [ncbi.nlm.nih.gov]

  Applicable To Acrocephaly Imperfect fusion of skull Oxycephaly Trigonocephaly The following code(s) above Q75.0 contain annotation back-references Annotation Back-References In this context, annotation back-references refer to codes that contain: Applicable [icd10data.com]

  We shall consider of these Oxycephaly which is otherwise called Acrocephaly, Turricephaly or tower skull. [ijo.in]

  exophthalmos, hypertelorism, strabismus, parrot-beaked nose, and hypoplastic maxilla with relative mandibular prognathism Premature closure of one or more sutures of the skull 756.0 Excludes Applies To Absence of skull bones Acrocephaly Congenital deformity [icd9data.com]

• Cranial Asymmetry

  The adverse effects associated with surgical correction of craniosynostosis include cerebrospinal fluid leak, injury to the brain, wound healing problems, cranial asymmetry, and the need for further surgery. [clinicaladvisor.com]

  Since the early 1990s, US pediatricians have seen an increase in the number of children with cranial asymmetry, particularly unilateral flattening of the occiput, likely attributable to parents following the American Academy of Pediatrics “Back to Sleep [pediatrics.aappublications.org]

• Headache

  Surgery is not required to prevent mental retardation, blindness, seizures or headaches in the vast majority of cases. No amount of surgical expertise can produce a perfect result. [uclahealth.org]

  RESULTS: The cohort included 383 patients, of whom 127 (33 percent) complained of headaches. [ncbi.nlm.nih.gov]

  This can cause headaches, developmental delay, seizures, and vision loss. This is much more common in patients with premature fusion of multiple sutures. [hopkinsallchildrens.org]

  If untreated, increased intracranial pressure can cause: Developmental delays Cognitive impairment Blindness Seizures Headaches Sept. 15, 2022 [mayoclinic.org]

• Seizure

  Surgery is not required to prevent mental retardation, blindness, seizures or headaches in the vast majority of cases. No amount of surgical expertise can produce a perfect result. [uclahealth.org]

  This can cause headaches, developmental delay, seizures, and vision loss. This is much more common in patients with premature fusion of multiple sutures. [hopkinsallchildrens.org]

  Abnormal head shape was noted in all 13 patients; 11 of these also had either chronic headaches, papilledema, seizures, or behavioral changes in the setting of functional shunt. Mean follow-up after the initial CVR was 3.3 years. [ncbi.nlm.nih.gov]

  If untreated, increased intracranial pressure can cause: Developmental delays Cognitive impairment Blindness Seizures Headaches Sept. 15, 2022 [mayoclinic.org]

• Stroke

  The Neuro-ICU cares for patients with all types of neurosurgical and neurological injuries, including stroke, brain hemorrhage, trauma and tumors. [uclahealth.org]

  The mission of the National Institute of Neurological Disorders and Stroke (NINDS) is to seek fundamental knowledge about the brain and nervous system, and to use that knowledge to reduce the burden of neurological disease. [my.clevelandclinic.org]

  Email: [email protected] and Duk‐Kyung Kim, Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. [doi.org]

• Abnormal Gait

  It tends to go unnoticed until walking age is reached, when the deformity results in a leg length difference or abnormal gait pattern. [physio-pedia.com]

The following tests are employed for diagnosing craniosynostosis:

• Physical examination: A preliminary physical examination would be carried out to check for facial deformities and abnormalities in suture ridges.
• Imaging studies: Imaging studies such as CT scan of the head will be carried out to check whether any sutures will be fused or not. In addition, x-rays would also be done to measure the dimension of the baby’s skull.
• Genetic tests: This is done to identify the causative factor behind development of craniosynostosis. Such type of tests is done through blood tests to identify the type of genetic syndrome behind the condition. In many cases, hair, skin or other tissue of the baby may also be tested for any abnormality [9].

• Mild cases of craniosynostosis, require no treatment and abnormalities in the shape of the head become less evident as the baby grows. Helmet therapy may be recommended in such cases that would help reshape the head and in turn provide space for development of baby’s brain [10]. In other cases, surgery is the only resort to correct the deformity. The various types of surgical procedures that are involved include the following:
• Traditional surgery: In this type, an incision is made in the skull, and the affected portion is then reshaped with the help of plates and screws. In many cases, a second surgery may also be required, because there are chances of recurrence of craniosynostosis.
• Endoscopic surgery: It is a less invasive form of surgery, but is not meant for all cases of craniosynostosis. An endoscope is inserted through small incisions made in the skull, and the sutures are opened to provide room for brain development [11].

Children with no other associated underlying conditions do well after surgical intervention. Prognosis of craniosynostosis depends on the number of sutures involved and overall health condition of the child [6].
In case, when other associated anomalies are involved then the head deformity can be permanent, even if treatment is not initiated on time. Failure to initiate treatment on time can cause development of seizures, intracranial pressure and developmental delay [7].

The exact cause of craniosynostosis is unknown. However, the involvement of genes in the premature fusion of cranial sutures of the skull has been found to play foul [2]. Craniosynostosis is classified under 2 categories: nonsyndromic and syndromic. The cause of the former condition is unknown. In the latter type, genes and heredity are known to play foul. Genetic syndromes such as Crouzon syndrome, Apert syndrome and Pfeiffer syndrome are associated with abnormal skull development of the baby. In the year 1912, Crouzon was the first to describe heredity as the factor for development of craniosynostosis [3].

Craniosynostosis is estimated to occur in 1 in every 2000 live births globally. Complex craniosynotosis, wherein more than 1 suture is affected, is prevalent in 5 – 15% of the population. Occurrence of Apert syndrome and Pfeiffer syndrome is rare; whereas incidence of Coruzon syndrome has been recorded to be 1 in every 2,500 live births. About 40 – 55% of cases of craniosynostosis, is of the form sagittal synostosis [4].

Children with craniosynostosis, have abnormal osteoblactic activity along with increase osteocalcin levels, decrease in production of alkaline phosphatase and a decreased growth rate. In addition, affected children also have increases epidermal growth factor and platelet – derived growth factor.
New bone formation has been observed when resected sutures were histophatlogically examined. Such a phenomenon causes development of ridges in the affected area, which is palpable during clinical examination and is also evident during surgical procedure [5].

So far, no guidelines have been given to prevent craniosynostosis. However, there are certain small pieces of information suggesting that women who took adequate quantities of riboflavin, vitamin E, C and B6 were at a low risk of developing such anomalies than those who did not. Studies have suggested that, maternal nutrition has significant association with development of structural abnormalities. In view of this fact, it can be safely concluded that, consuming a nutritionally rich diet during the first trimester of pregnancy, can prevent the fetus from craniosynostosis.

This leads to significant change in the growth pattern of the skull. Craniosynostosis, in many cases is associated with underlying anomalies which may cause improper development of the brain. One or more joints may be affected by this condition and surgery is the only option to correct such a condition. If there is no underlying disease condition, then after surgery, the brain can develop appropriately [1].

• Definition: Craniosynostosis, is a condition, characterized by premature closing of sutures, which leads to improper shape of head. It is a birth defect, wherein the sutures close much before the brain gets fully developed. In this condition, one or more joints of the skull are affected.
• Cause: In many cases, the exact cause of craniosynostosis is unknown. Presence of underlying disease conditions is thought to play foul in development of certain forms of craniosynostosis. Certain type of genetic syndromes such as Apert syndrome, Coruzon syndrome and Pfeiffer syndrome can significantly affect the development of baby’s skull.
• Symptoms: Symptoms of craniosynostosis include development of unusual shape of head, slowed growth of head as the baby grows, increase in intracranial pressure and development of hard ridges in the affected area.
• Diagnosis: A preliminary physical examination will be carried out to determine abnormalities such as suture ridges and facial deformities. In addition, imaging studies such as CT scan and x-rays will also be required to determine fused sutures. Genetic testing also forms an essential part of diagnosis and is done to analyze the presence of genetic syndromes if any.
• Treatment: Mild cases of craniosynostosis seldom require any treatment if only one suture is involved and there are no underlying genetic abnormalities. In severe cases, surgery is the only available option to treat the condition.

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