Craniosynostosis
Craniosynostosis is a craniofacial condition characterized by the premature closure of the sutures in an infant's skull, which can lead to abnormal head and facial shapes. The skull of a newborn contains open areas called fontanelles that allow for growth; when these areas close too early, it disrupts normal development. This condition can occur in isolation or alongside other congenital defects. Symptoms primarily manifest as irregularities in head and face shape.
Treatment typically involves surgical intervention, with fronto-orbital advancement being a common procedure. This surgery repositions the frontal bone and supraorbital rim to improve the skull's appearance and functionality. While surgery is necessary for correction, the recovery process involves monitoring for swelling and keeping the surgical site clean. Alternative, less invasive techniques are also available for younger infants, allowing for a shorter recovery time with the use of protective headgear. Overall, craniosynostosis is manageable and correctable, often leading to positive outcomes for affected children.
Craniosynostosis
ANATOMY OR SYSTEM AFFECTED: Bones, head
DEFINITION: The premature closing of the open areas between the bones in an infant’s skull
CAUSES: Birth defect
SYMPTOMS: Abnormal head and face shape
DURATION: Typically correctable, with side effects of surgery lasting several months
TREATMENTS: Fronto-orbital advancement, other surgeries
Causes and Symptoms
Craniosynostosis is a craniofacial abnormality that occurs in a variety of forms. The skull of a newborn infant contains several open areas between the bones that make up the skull. These areas, called fontanelles, allow the skull to expand as the child’s grows. Craniosynostosis is the closure of one or more of these open areas, resulting in the abnormal shaping of the head and face. Craniosynostosis may occur alone or in association with other defects.
Treatment and Therapy
During the 1960s the French surgeon Paul Tessier developed improved techniques for treating craniosynostosis. Treatment of craniosynostosis is often done with a surgical procedure known as fronto-orbital advancement. This technique involves cutting the skull in such a way that the frontal bone (the portion of the skull behind the forehead) and the supraorbital rim (the portion of the skull above and to the sides of the eyes) can be moved forward. These portions of the skull are then attached to the rest of the skull in their new positions with surgical wire. For some types of craniosynostosis, it may also be necessary to cut the frontal bone and the supraorbital rim down the middle to allow them to be reshaped. Fronto-orbital advancement usually takes place after the patient is three months old.
Correction of craniosynostosis is a complicated procedure, requiring the patient to be monitored in a special hospital bed for at least four or five days after surgery. After the surgery, the patient will experience severe swelling of the eyelids and scalp. Most patients will be unable to open their eyes until several days after surgery, and the swelling may not completely disappear for a few months. Care must be taken to keep the incision clean.
In some cases, craniosynostosis can be treated through a less invasive surgery in which a surgeon uses very small tools to remove bone from the skull through small incisions in the child's scalp. This surgery is usually performed on patients younger than six months. The recovery period is typically shorter than that of the more invasive surgery, although the patient must usually wear protective headgear for a time.
Bibliography
"Craniosynostosis." Mayo Clinic, 15 Sept. 2022, www.mayoclinic.org/diseases-conditions/craniosynostosis/symptoms-causes/syc-20354513. Accessed 1 Apr. 2024.
“Craniosynostosis.” Medline Plus, 9 Nov. 2021, medlineplus.gov/ency/article/001590.htm. Accessed 1 Apr. 2024.
“Craniosynostosis Repair.” Medline Plus, 11 Oct. 2022, medlineplus.gov/ency/article/007364.htm. Accessed 1 Apr. 2024.
"Facts about Craniosynostosis." Centers for Disease Control and Prevention, 28 June 2023, www.cdc.gov/ncbddd/birthdefects/craniosynostosis.html. Accessed 1 Apr. 2024.
Galli, Guido. Craniosynostosis. 1984. CRC Press, 2019.
Hayward, Richard, et al., eds. The Clinical Management of Craniosynostosis. New York: Cambridge University Press, 2004.
McCarthy, Joseph G., ed. Distraction of the Craniofacial Skeleton. New York: Springer, 1999.
Moore, Keith L., and T. V. N. Persaud. The Developing Human. 8th ed. Philadelphia: Saunders/Elsevier, 2008.
Sadler, T. W. Langman’s Medical Embryology. 11th ed. Philadelphia: Lippincott Williams & Wilkins, 2009.
Turvey, Timothy A., Raymond J. Fonseca, and Katherine W. Vig, eds. Facial Clefts and Craniosynostosis: Principles and Management. Philadelphia: W. B. Saunders, 1996.