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Encephalopathies – any of the various diseases of the brain.
Endoscopic surgery – surgery involving the placement of a thin-walled tube (endoscope) into an organ through a small incision. Endoscopic surgery is usually referred to as “minimally invasive”. Surgery is performed with the assistance of a fiber optic camera and small instruments which can be passed through the thin tube. In the brain, the camera and instruments can be moved around in the ventricles or the spaces around the brain. Endoscopic surgery usually carries the same risks as open surgery, which is the more traditional form of surgery. Endoscopic craniofacial and reconstructive surgery is done through smaller, more cosmetically acceptable incisions.
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Goldenhar Syndrome – Initially described in 1952, Goldenhar syndrome (also known as Oculo-Auriculo-Vertebral syndrome) is a very rare congenital, craniofacial syndrome. Goldenhar Syndrome presents as an incomplete development of the ear, nose, soft palate, lip, and mandible. Children may have dermoid cysts, pre-auricular skin tags (tags of skin in front of the ear), and strabismus (one eye turning in or out). The term is sometimes used interchangeably with hemifacial microsomia, although this definition is usually reserved for cases without internal organ/vertebrae disruption. Goldenhar syndrome affects between 1/3500 to 1/26000 live births. Hearing loss may occur and Goldenhar syndrome may be associated with scoliosis.
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Haberman nipple/ feeder – a special nipple for babies with cleft lip and/ or palate. May be used in Pierre Robin syndrome.
Hemifacial Macro – and Hemifacial micro-somia – These are the terms used to describe a condition where half or one side of the face is larger or smaller than the “normal” side.
Hydrocephalus – a condition in which there is an abnormal accumulation of cerebrospinal fluid (CSF) in the ventricles of the brain. This accumulation may cause enlargement of the skull and/ or compression of the brain. May be treated with medication in some conditions, but is usually treated surgically by shunts or endoscopic third ventriculostomies. There are many different kinds of hydrocephalus, and almost all forms can be successfully treated. If you have questions about hydrocephalus, please see the following links, or call our office. The Hydrocephalus Association provides support and assistance to patients and their families.
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Macrocephaly – a term used to describe a head that is larger than most. There are many possible causes.
Magnetic Resonance Imaging (MRI) – an imaging study which does not involve x-rays. There is NO radiation exposure. A magnetic resonance spectrometer is used to produce electronic images of specific atoms and molecular structures within the body. Commonly used to obtain fine-detailed pictures of the brain, white matter in the brain, spinal cord, and posterior fossa. MRI studies do take more time to perform compared to CT scans. This is why, for children, sedation or anesthesia, is often required. MRI provides better detail of the anatomy, but does not show bone lesions well. Functional MRI can show neurosurgeons and neurologists where functional areas of the brain lie. However, a child usually has to be older than 6 or 7 years old to be able to cooperate with functional imaging studies.
Metopic suture – the metopic suture is the space or gap between the right and the left frontal bones of the skull. A suture is the fibrous space between the bones of the skull. Where two bones meet, there is a suture or soft space. Occasionally, these soft spaces become abnormally “calcified” or stuck together. This is called craniosynostosis.
Metopic synostosis – Craniosynostosis is the abnormal fusion, calcification or “sticking together” of the sutures. Metopic synostosis is the abnormal fusion of the metopic suture of the skull. The metopic suture starts closing at about 4 months of age; if the suture closes early, this is called premature metopic synostosis. Sometimes children will develop a triangular shaped forehead called trigonocephaly, if the metopic suture fuses and closes too soon. Metopic synostosis can be treated with conventional open surgery or with new endoscopic surgical techniques and its associated helmet remodeling.
Microcephaly – a term used for a smaller than normal head size.
Micrognathia – a smaller than normal mandible, or a mandible or jaw bone, that appears small. Can be seen in Pierre Robin sequence/ syndrome and other craniofacial disorders.
Microsomia – an unusual “smallness” or small size of a body part.
Neuronavigation – the identification of neuro-anatomy based on computer guidance and pre-operative MRI or CT images. Neurosurgeons will request a MRI or CT scan to use for mapping out a brain or spinal tumor during surgery. The ability to localize a tumor in the brain assists a neurosurgeon to avoid important areas of the brain used for motor and speech. Sometimes, neuronavigation is used to guide a spine surgery, when screws or other devices need to be accurately placed in the bones of the spine.
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Orthognathic surgery – This procedure is needed when jaws don’t meet correctly and/or teeth don’t seem to fit with jaws. Any of these symptoms can exist at birth, be acquired after birth as a result of hereditary, congenital birth defects, environmental influences, or as a result of trauma to the face. Teeth are straightened with orthodontics and corrective jaw surgery repositions a misaligned jaw. This not only improves facial appearance, but also ensures that teeth meet correctly and function properly. Children who can benefit from orthognathic surgery include those with an improper bite or jaws that are positioned incorrectly. Jaw growth is a gradual process and in some instances, the upper and lower jaws may grow at different rates. The result can be a host of problems that can affect chewing function, speech, long-term oral health and appearance. Orthodontics alone can correct bite problems when only the teeth are involved. Orthognathic surgery may be required for the jaws when repositioning is necessary and is usually performed after a child’s facial bones have completely stopped growing, usually between the ages of 16-18. Many children with other craniofacial syndromes and or cleft lip and palate will require corrective jaw surgery at some point in their course of treatment.
Orthotic (brace) design – an orthopedic appliance designed to straighten or support a body part. An orthotist is a trained specialist who constructs orthotics or braces. A cranial molding helmet is an orthotic custom molded to encourage the skull to become more symmetrical and/or proportional. They do not squeeze the baby’s head into shape, but rather redirects growth and provides the space into which the flattened portion of the skull can grow.
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Pierre Robin and Pierre Robin Sequence – characterized by a small jaw or mandible (micro-gnathia) retraction of the tongue (glossoptosis) and upper airway obstruction. U-shaped cleft palate may also occur. This syndrome may be associated with velo-cardial-facial syndrome, fetal alcohol syndrome, and/ or Treacher Collins syndrome. PRS is rare, affecting about 1/ 20,000 live born babies.
Plagiocephaly – an abnormal asymmetrical deformity of the skull due to premature closure of the sutures of the skull, or from positional molding. Plagiocephaly may be treated with physical therapy, molding helmets, and/ or surgery. Babies with plagiocephaly are sometimes referred by their pediatricians for a cranial molding helmet. It is usually advisable to see a pediatric neurosurgeon, or craniofacial plastic surgeon, to rule out craniosynostosis first. Craniosynostosis can occur in infants and does require surgery. Plagiocephaly is usually caused by positional molding or flattening of the thin, cartilaginous infant skull. Many babies are referred to our Craniofacial Team every month, to rule out craniosynostosis, and to be treated for plagiocephaly. Our Craniofacial Center provides the most comprehensive team approach in the state.
Positron emission tomography (PET) – tomography in which a computer-generated image of local metabolic and physiological functions in tissues is produced through the detection of gamma rays that are emitted when introduced radionuclides decay and release positrons. Also used for imaging the functional status of the brain.
Psychometrics – the branch of psychology that deals with the design, administration, and interpretation of quantitative tests for the measurement of psychological variables such as intelligence, aptitude, and personality traits.
Sagittal suture – the sagittal suture is the space or gap between the right and the left frontal bones of the skull. A suture is the fibrous space between the bones of the skull. Where two bones meet, there is a suture or soft space. Occasionally, these soft spaces become prematurely “calcified” or stuck together. This is called craniosynostosis.
Sagittal synostosis – Craniosynostosis is the abnormal fusion, calcification or “sticking together” of the sutures. Sagittal synostosis is the abnormal fusion of the sagittal suture of the skull. Sometimes children will develop a long and narrow shaped head called scaphocephaly, if the sagittal suture fuses and closes too soon. This condition requires surgical intervention and helmet therapy; new endoscopic surgical treatment may be used.
Scaphocephaly – an abnormally long and narrow head. May be caused by positional deformity or may be caused by sagittal synostosis. It may be treated using helmet therapy.
Shunts – a surgically implanted tube which diverts spinal fluid from the brain or spinal canal, to the peritoneum (abdomen), pleura (lungs) or gallbladder. Shunts were developed in the later part of the 20th century. With early shunts, there were high complication rates. Newer shunts have safer technology and better techniques so that the risks of shunt implantation are substantially lower. Even so, shunt infection, blockage or breakage may occur. It is important to follow up regularly with your neurosurgeon if you have a shunt.
Single photon emission computed tomography (SPECT) – an imaging study that uses tomography of metabolites to evaluate physiological functions in various organs, including the brain. The image is acquired by a computer synthesis of data that is transmitted by single gamma photons emitted by radionuclides administered to the patient.
Stereotactic guidance – intraoperative, computer-based localization of brain anatomy and function based on pre-operative neuro-imaging. Stereotactic guidance is also called neuronavigation. Neurosurgeons will often request a MRI or CT scan to use for mapping out a brain or spinal tumor during surgery. The ability to localize a tumor in the brain helps a neurosurgeon avoid important areas of the brain that are used for motor and speech. Neuronavigation is also used to guide a spine surgery, when screws or other devices need to be safely and accurately placed in the bones of the spine.
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Torticollis – Torticollis is a tilting or twisting of the head that may be congenital or acquired. Babies may have torticollis at birth from in utero positioning or from a bony malformation of the spine. Usually x-rays and other imaging studies are deferred. Babies with active torticollis, limited range of motion, or active head turning, should have imaging studies done. Babies, who tilt their heads at rest, without active twisting, are usually followed conservatively with physical therapy, stretching at home, respositioning and increased tummy time. Regardless of the cause of torticollis, babies with this disorder usually develop cranial or skull deformity. This skull asymmetry is called plagiocephaly. Plagiocephaly can be mild to severe and is treated in many different ways, depending on its’ severity. Many infants are referred to our Craniofacial Center for evaluation of torticollis and plagiocephaly. For more information, or to request our brochure or appointment, please call our office.
Trigonocephaly – a term used to describe a triangular head shape. May be caused by premature fusion of the metopic suture. Trigonocephaly can be treated surgically using endoscopic procedures and in conjunction with a cranial molding helmet once the suture is released.
Tummy Time – Tummy time with your infant is a great way to spend time with your infant. Placing your baby on their tummy on the floor, encourages him or her to develop strong neck muscles and a round head. Before long your baby will learn how to crawl as they build up their muscle tone. Carrying your baby facing forward also encourages him to develop great head control.
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Vascular malformations – Vascular malformations or arterio-venous malformations (AVMs) are congenital blood vessel abnormalities. Some children are born with an abnormal tangle of blood vessels. These vessels can be arteries or veins. Vascular malformations are classified by size, location within the brain, depth within the brain, and blood flow. The easiest AVM’s to remove are small, superficial, low flow, and located in non-eloquent cortex (a “quiet” area of the brain). Facial hemangiomas are one kind of vascular malformation.
