Craniofacial surgery
Encyclopedia
Craniofacial surgery is a surgical subspecialty
of maxillofacial surgery
, plastic surgery
, and ENT
that deals with congenital
and acquired deformities
of the skull
, face, and jaw
s. Although craniofacial treatment often involves manipulation of bone, craniofacial surgery is not tissue-specific, i.e., craniofacial surgeons
deal with bone, skin, muscle, teeth, etc. Craniofacial surgery does not, however, include surgery of the brain or eye.
Defects typically treated by craniofacial surgeons include craniosynostosis
(isolated and syndromic), rare craniofacial clefts
, acute and chronic sequellae of facial fractures, cleft lip and palate, micrognathia, Treacher Collins Syndrome
, Apert's Syndrome, Crouzon's Syndrome, hemifacial microsomia and many others.
Training in craniofacial surgery usually consists of a 1-year surgical fellowship completed after a residency in either plastic surgery
, oral and maxillofacial surgery
, or otolaryngology
.
results in increased intracranial pressure.
Craniosynostosis is called simple when one suture is involved, and complex when two or more sutres are involved. It can occur as part of a syndrome or as an isolated defect (nonsyndromic).
There are several classifications of deformities of the human skull, we will discuss them in order of prevalence.
Scaphocephaly
In scaphocephaly the saggital suture is prematurely fused. The saggital suture runs from the front to the back of the head. The shape of this deformity is a long narrow head, formed like a boat (greek skaphe, “light boat or skiff”). The incidence of scaphocephaly is 2.8 per 10 000 births in the Netherlands and is therefore the most common form of craniosynostosis.
Trigonocephaly
In trigonocephaly the metopic suture is prematurely fused. The metopic suture is situated in the medial line of the forehead. Premature fusion of this suture caused the forehead to become pointed, giving the head a triangular shape when viewd from above (greek trigono, “triangle”). The incidence of trigonocephaly is 1 - 1.9 per 10 000 births in the Netherlands.
Plagiocephaly
In plagiocephaly one of the coronal sutures is prematurely fused. The coronal sutures run over the top of the head, just in front of the ears. The shape of this deformity is an asymmetrical distortion (flattening of one side of the head) as you can see in figure 2. The incidence is 1 in 10 000 births.
Brachycephaly
In brachycephaly both of the coronal sutures are prematurely fused. The shape of this deformity is a wide and high head. The incidence at birth is 1/20 000.
In scaphocephaly the saggital suture is prematurely fused, preventing the skull from growing perpendicular to the suture. Thus the head becomes very narrow and long. If a scaphocephaly is diagnosed within 4 to 5 months after birth, it can be corrected with a relatively simple procedure whereby the saggital suture is surgically reopened. Once the suture has been opened the bone segments will be able to grow again and the head can regain its normal shape. This operation is only performed on patients younger than five months old with a scaphocephaly. This is due to the fact that the bone segments only have the ability to adapt so severely when the operation is performed at this young age. A scaphocephaly that is diagnosed and treated later in life requires a more extensive secondary operation than one which is treated before five months.
.
Subspecialty
A subspecialty is narrow field within a specialty such as forensic pathology, which is a subspecialty of anatomical pathology. A subspecialist is a specialist of a subspecialty....
of maxillofacial surgery
Oral and maxillofacial surgery
Oral and maxillofacial surgery is surgery to correct a wide spectrum of diseases, injuries and defects in the head, neck, face, jaws and the hard and soft tissues of the oral and maxillofacial region. It is an internationally recognized surgical specialty...
, plastic surgery
Plastic surgery
Plastic surgery is a medical specialty concerned with the correction or restoration of form and function. Though cosmetic or aesthetic surgery is the best-known kind of plastic surgery, most plastic surgery is not cosmetic: plastic surgery includes many types of reconstructive surgery, hand...
, and ENT
Otolaryngology
Otolaryngology or ENT is the branch of medicine and surgery that specializes in the diagnosis and treatment of ear, nose, throat, and head and neck disorders....
that deals with congenital
Congenital disorder
A congenital disorder, or congenital disease, is a condition existing at birth and often before birth, or that develops during the first month of life , regardless of causation...
and acquired deformities
Deformity
A deformity, dysmorphism, or dysmorphic feature is a major difference in the shape of body part or organ compared to the average shape of that part.Deformity may arise from numerous causes:*A Genetic mutation*Damage to the fetus or uterus...
of the skull
Human skull
The human skull is a bony structure, skeleton, that is in the human head and which supports the structures of the face and forms a cavity for the brain.In humans, the adult skull is normally made up of 22 bones...
, face, and jaw
Jaw
The jaw is any opposable articulated structure at the entrance of the mouth, typically used for grasping and manipulating food. The term jaws is also broadly applied to the whole of the structures constituting the vault of the mouth and serving to open and close it and is part of the body plan of...
s. Although craniofacial treatment often involves manipulation of bone, craniofacial surgery is not tissue-specific, i.e., craniofacial surgeons
Surgery
Surgery is an ancient medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...
deal with bone, skin, muscle, teeth, etc. Craniofacial surgery does not, however, include surgery of the brain or eye.
Defects typically treated by craniofacial surgeons include craniosynostosis
Craniosynostosis
Craniosynostosis is a condition in which one or more of the fibrous sutures in an infant skull prematurely fuses by ossification, thereby changing the growth pattern of the skull...
(isolated and syndromic), rare craniofacial clefts
Facial cleft
A facial cleft is an opening or gap in the face, or a malformation of a part of the face. Facial clefts is a collective term for all sorts of clefts. All structures like bone, soft tissue, skin etc. can be affected. Facial clefts are extremely rare congenital anomalies...
, acute and chronic sequellae of facial fractures, cleft lip and palate, micrognathia, Treacher Collins Syndrome
Treacher Collins syndrome
Treacher Collins syndrome , also known as Treacher Collins–Franceschetti syndrome, or mandibulofacial dysostosis is a rare autosomal dominant congenital disorder characterized by craniofacial deformities, such as absent cheekbones. Treacher Collins syndrome is found in about 1 in 10,000 births, ....
, Apert's Syndrome, Crouzon's Syndrome, hemifacial microsomia and many others.
Training in craniofacial surgery usually consists of a 1-year surgical fellowship completed after a residency in either plastic surgery
Plastic surgery
Plastic surgery is a medical specialty concerned with the correction or restoration of form and function. Though cosmetic or aesthetic surgery is the best-known kind of plastic surgery, most plastic surgery is not cosmetic: plastic surgery includes many types of reconstructive surgery, hand...
, oral and maxillofacial surgery
Oral and maxillofacial surgery
Oral and maxillofacial surgery is surgery to correct a wide spectrum of diseases, injuries and defects in the head, neck, face, jaws and the hard and soft tissues of the oral and maxillofacial region. It is an internationally recognized surgical specialty...
, or otolaryngology
Otolaryngology
Otolaryngology or ENT is the branch of medicine and surgery that specializes in the diagnosis and treatment of ear, nose, throat, and head and neck disorders....
.
Craniosynostosis
The bones of the human skull are joined together by cranial sutures (see figure 1). The anterior fontanelle is where the metopic, saggital and coronal sutures meet. Normally the sutures gradually fuse within the first few years after birth. In infants where one or more of the sutures fuses too early the growth of the skull is restricted, resulting in compensation mechanisms which cause irregular growth patterns. Growth in the skull is perpendicular to the sutures. When a suture fuses too early, the growth perpendicular to that suture will be restricted, and the bone growth near the other sutures will be stimulated, causing an abnormal head shape. The expanding brain is the main stimulus for the rapid growth of the skull in the first years of life. Inhibited growth potential of the skull can restrict the volume, needed by the brain. In cases in which the compensation does not effectively provide enough space for the growing brain, craniosynostosisCraniosynostosis
Craniosynostosis is a condition in which one or more of the fibrous sutures in an infant skull prematurely fuses by ossification, thereby changing the growth pattern of the skull...
results in increased intracranial pressure.
Craniosynostosis is called simple when one suture is involved, and complex when two or more sutres are involved. It can occur as part of a syndrome or as an isolated defect (nonsyndromic).
There are several classifications of deformities of the human skull, we will discuss them in order of prevalence.
ScaphocephalyScaphocephalyScaphocephaly , derived from the Greek skaphe , describes a specific variety of a long narrow head that resembles an inverted boat....
In scaphocephaly the saggital suture is prematurely fused. The saggital suture runs from the front to the back of the head. The shape of this deformity is a long narrow head, formed like a boat (greek skaphe, “light boat or skiff”). The incidence of scaphocephaly is 2.8 per 10 000 births in the Netherlands and is therefore the most common form of craniosynostosis.TrigonocephalyTrigonocephalyTrigonocephaly is a congenital condition of premature fusion of the metopic suture leading to a triangular shaped forehead. The merging of the two frontal bones leads to transverse growth restriction and parallel growth expansion...
In trigonocephaly the metopic suture is prematurely fused. The metopic suture is situated in the medial line of the forehead. Premature fusion of this suture caused the forehead to become pointed, giving the head a triangular shape when viewd from above (greek trigono, “triangle”). The incidence of trigonocephaly is 1 - 1.9 per 10 000 births in the Netherlands.PlagiocephalyPlagiocephalyPlagiocephaly is a condition characterized by an asymmetrical distortion of the skull.-Causes:It is a common finding at birth and may be the result of a restrictive intrauterine environment. If there is premature union of skull bones, this is more properly called craniosynostosis...
In plagiocephaly one of the coronal sutures is prematurely fused. The coronal sutures run over the top of the head, just in front of the ears. The shape of this deformity is an asymmetrical distortion (flattening of one side of the head) as you can see in figure 2. The incidence is 1 in 10 000 births. BrachycephalyBrachycephalyBrachycephaly, also known as flat head syndrome, is a type of cephalic disorder. This can result from premature fusion of the coronal sutures or from external deformation . The coronal suture is the fibrous joint that unites the frontal bone with the two parietal bones of the skull. The parietal...
In brachycephaly both of the coronal sutures are prematurely fused. The shape of this deformity is a wide and high head. The incidence at birth is 1/20 000.Surgical procedures
In cases where the forehead is involved (trigonocephaly and plagiocephaly), a technique called fronto-supraorbital advancement is used to correct the shape of the head. The procedure is performed at a young age in order to provide the brain with enough space to grow and prevent further abnormal growth of the skull. Fronto-orbital advancement literally means moving the front of the skull including the eye sockets forward. A section of the skull, ranging from the coronal sutures to the eye sockets is cut loose in order to correct the shape of the skull. The incision is cut in a zigzag shape from ear to ear so that the hair will cover the scar and make it less visible. The incision is made to the bone only, leaving the underlying meninges intact. The top half of the eye sockets is cut loose. Once the eye socket section has been cut loose, a vertical incision is made in the midline, and the whole section of the eye socket is bent outwards in order to correct the pointed shape of the forehead. Because the section is now too wide, a wedge needs to be cut on either side to allow the section to fit into the skull. Figure 4 shows the sections that are loosened and adjusted, and figure 3 shows the location of the vertical incision (arrow A) and the two wedges (arrow B).In scaphocephaly the saggital suture is prematurely fused, preventing the skull from growing perpendicular to the suture. Thus the head becomes very narrow and long. If a scaphocephaly is diagnosed within 4 to 5 months after birth, it can be corrected with a relatively simple procedure whereby the saggital suture is surgically reopened. Once the suture has been opened the bone segments will be able to grow again and the head can regain its normal shape. This operation is only performed on patients younger than five months old with a scaphocephaly. This is due to the fact that the bone segments only have the ability to adapt so severely when the operation is performed at this young age. A scaphocephaly that is diagnosed and treated later in life requires a more extensive secondary operation than one which is treated before five months.
Ethical considerations
The Hastings Center, a prominent bioethics research institute, conducted a project called "Surgically Shaping Children". The project produced an edited volume (Parens, 2006) which considers reconstructive surgery on children with craniofacial anomalies, ambiguous genitalia, and achondroplasiaAchondroplasia
Achondroplasia dwarfism occurs as a sporadic mutation in approximately 85% of cases or may be inherited in an autosomal dominant genetic disorder that is a common cause of dwarfism...
.
External links
- Information about Craniofacial Surgery for Children and Adults
- BAOMS - What is craniofacial surgery?
- http://www.acgme.org/acWebsite/downloads/RRC_progReq/361pr997_u1004.pdf
- Journal of Craniofacial Surgery
- American Society of Craniofacial Surgery
- European Association of Cranio-Maxillofacial Surgery
- International Society of Craniofacial Surgery
- Cleft and Craniofacial Center at Hasbro Children's Hospital