Email: info@pierrerobin.org.za  |  Mobile: +27 21 827 0240  |  Address: 4B St James Street, Somerset West, Cape Town, South Africa 

Glossary

Glossary A – Z

Hearing so many different terms can be overwhelming and confusing to parents and caregivers.

We have listed several on two different pages for your reference.  You may never come into contact with some of these terms, some of them relate to issues that may not apply to every child, but we felt it necessary to list as many as possible.  If you would like us to add more terms that might be beneficial, please let me know.


Alveolar ridge – the bony ridge of the maxilla and mandible containing the teeth.


Anesthesiologist – physician who specializes in anesthesiology, administering medication to patients undergoing surgery.


Apnea monitor – a machine that will hook to the baby via either a belt or sticky type leads.  This machine will monitor your child’s breathing and heart rate.  It can be set to alarm if levels go below or above a certain predetermined level.


Artificial Nose – a filter device that fits onto the hub of a tracheostomy tube and is used to trap moisture and minimize exposure to germs or dust in the air as the patient breathes (AKA: HME, Thermo vent T, Swedish Nose)


Articulation – the process of forming speech sounds.


Aspiration – The inhaling of foreign matter in the lungs.


Aspiration pneumonia – refers to the inappropriate passage of food, water, stomach acid, vomit or another foreign material into the lungs. Aspiration, particularly involving gastric acid, will often result in a serious pneumonia.


Asthma – Chronic respiratory disorder characterized by labored breathing and wheezing resulting from obstructed and constricted air passages. Long-term control includes inhaled or oral bronchodilators (albuterol, theophylline), breathing exercises, and, if possible, the identification and avoidance of allergens. Short-term cortisone and prednisone can bring immediate relief in acute attacks.


Audiologist – professional who studies and tests for hearing disorders through identification and evaluation of hearing loss and the rehabilitation of hearing loss.


Barium Swallow – radiographic exam of the esophagus during and after introduction of barium sulfate.  Structural abnormalities of the esophagus may be diagnosed by the use of this technique.


Bolus – feeding given over a short period of time ranging from a few minutes to half an hour


Bronchoscope – endoscope that passes through the trachea to allow visual inspection of the tracheal area.


Bronchoscopy – examination of the bronchi through a scope.


CPAP – continuous positive air pressure.  A piece of equipment used to aid patients with breathing.


Cleft or Craniofacial Team – health care team.  The principle role of the team is to provide integrated case management for your child, to assure quality and continuity of care and to provide long term follow up.  The team usually consists of:  audiologist, geneticist, nurse, oral surgeon, orthodontist, otolaryngologist, pediatrician, pedodontist, plastic surgeon, prosthodontist, psychologist, social worker and speech-language pathologist.


Cleft Palate – opening in the roof of the mouth, generally horseshoe shaped in children with PRS.  Involves the soft palate (back part) and sometimes the hard palate.  A cleft lip is a separate condition and each can occur independent of each other.  A cleft lip is very rare in a child with PRS.


Conductive hearing loss – Conductive Hearing Loss-associated with the formation of fluid in the middle ear. Sound is heard through this fluid making the ears feel “blocked”. This  type of hearing loss is USUALLY not permanent and is reversed when fluid is drained/tubes are put into place.


Decannulation – removal of the trach.


Desaturation – A drop in oxygen levels due to apnea, obstruction or other medical conditions.


Developmental Pediatrician – physician who addresses special needs or special care issues for children with special needs.


Discharge Planning Nurse – person from the hospital that is usually responsible to make sure all arrangements have been made to take care of the baby at home.  This person will coordinate any DME or nursing care and usually communicates with the insurance company.


Durable medical equipment (DME) – items such as feeding pumps, apnea monitors, pulse ox meters.


Dysphagia – inability to swallow or difficulty in swallowing.


ENT (Otolaryngologist) – Ear, nose, throat specialist


Ear tubes – Ear tubes-also known as PE (pressure equalizing), Tympanostomy, or Ventilation tubes.  Inserted during surgery called MYRINGOTOMY which creates a small incision in the eardrum. This allows fluid to be drained and the tubes to be inserted into the eardrum. The tubes allow the eardrum to remain open, letting air flow into the ear and maintains drainage of fluid. The tubes do not stick out of the ear and are not usually visible without an exam of the ear.


Early Intervention Services – programs or services designed to identify and treat a developmental problem as early as possible, before age 3.  Services for 3-5 year olds are referred to as pre-school services.


Eustachian tubes – the air duct which connects the nasopharynx (the back of the throat) with the middle ear; usually closed at one end, opens with yawning and swallowing; allows ventilation of the middle ear cavity and equalization of pressure on two sides of the eardrum.


Feeding therapy – therapy provided by a speech/language pathologist, occupational therapist and/or dietician to address concerns of eating and swallowing.


Fistula – abnormal opening in the palate.  They are sometimes closed surgically and many times close on their own.


Foley Catheter – generally this is the initial tube used for g-tubes.  It is also a flexible plastic tube inserted into the bladder to provide continuous urinary drainage.


Frenulum – material that attaches the lower side of the tongue to the floor of the mouth.  It is sometimes “tight” and is considered “tongue-tied.”


Frenulum puddle (excessive tongue tissue near frenulum): Excess tongue tissue under the base of the tongue. Gives the appearance of two tongues stacked on top of each other.  This is a common occurrence in babies with Pierre Robin and should become less noticeable, or even disappear completely, as the child grows.  This is probably not a true medical term, but our families refer to it as either frenulum or tongue puddle.


Fundoplication – surgically reducing the size of the opening into the stomach and sewing the esophagus to it,  it is done to prevent reflux and aspiration.


G-tube – (Gastrostomy) surgical creation of a fistula through the stomach so a tube may be inserted to aide in feeding.


Gastric Emptying Test – evaluates the emptying of food from the stomach.


Gastroenterologist (GI) – physician who specializes in the study of the physiology and pathology of the stomach, intestines, esophagus, liver, gallbladder and pancreas.  These specialist will help your child if they have reflux and would be involved in care of the g-tube.


Gavage – feeding with a stomach tube or with a tube passed through the nose, pharynx and esophagus into the stomach.


Geneticist – physician that will perform a physical and order any tests needed to get an accurate diagnosis for your child.


Glossoptsis – a dropping of the tongue downward out of normal position.


Granulization – mass of red, moist, granular tissue that develops on the surface of a wound. Often develops around the stomas of the tracheostomy tube and gastrostomy tube.


Haberman feeder – special bottle used for infants with cleft palates, they are manufactured by Medela.


Hard Palate – The front part of the roof of the mouth containing bone covered by mucosa (pink “skin”)


Home health care – Agency which will provide nursing care, respiratory therapists, physical therapists and any other specialized care your child needs in your home.


Horseshoe cleft – description of the typical shape of the cleft in a child with PRS,  may be similar in appearance to a mouth guard used for sports


Hypotonia – loss of tone in the muscles or low muscle tone.


Intubation – the insertion of a tube into the larynx for entrance of air.


J-tube – surgical creation of an opening in the jejunum (second portion of the small intestine).  A tube is inserted to aide in feeding.  NOTE:  The contents of the tube bypass the stomach in this procedure.


Jaw Distraction (Mandibular Distraction) –surgery performed on a child with PRS to extend the lower jaw.  Most of the procedures are done externally and the parents use a device to turn part of the hardware to extend the jaw.


Kangaroo Pump – a feeding pump that is attached to a gastrostomy button in order to provide a continuous “drip” feeding over a period of time (Eg. through the night).


Larynx – enlarged upper end of the trachea below the root of the tongue.


Mandible – the lower jaw.


Maxilla – the upper jaw.


Medicaid – in the U.S.A., it is a government program for providing medical care of those who are unable to afford it and meet certain criteria.


Mic-key Button – a type of gastrostomy button which is placed in the abdomen and creates an artificial opening into the stomach. Another type of gastrostomy button is the “Bard”.


Micrognathia – abnormal smallness of jaws, especially the lower jaw.


Mist Collar – mask that is attached to the patient and some sort of system that provides humidity to the tracheostomy tube (AKA tracheostomy mask).


Myringotomy-minor surgery which requires the use of anesthesia. Involve the creation of a small incision in the eardrum which allows for fluid drainage and insertion of ear tubes.


NICU – Neonatal Intensive Care Unit.  Intensive care unit that is designated to care for newborn babies.


NPO – Nothing by mouth.   This would be ordered by a physician to indicate the child is not to have any food or liquid by mouth.  They can get nourishment and medications through IVs, g-tubes or j-tubes.


Nasal Endoscopy – procedure in which the plastic surgeon, ENT or speech therapist can see if complete palatal closure is acquired during normal speech or if air is escaping.  They spray an anesthetic in one nostril or they swab it on with a Q-tip type swab.  They insert a very small flexible scope with a micro-camera on the end of it.  The entire session is video taped so they can review it to see if therapy needs to be continued or if surgically intervention is necessary.  It is relatively painless but can be a little uncomfortable for the child.


Nasal trumpet – small tube with a round “stop” on one end. Inserted into the nasal opening to allow airflow into the lungs. Helps to keep the airway open by bypassing the oral cavity. May be used to establish a temporary airway during an emergency or may be used on a more permanent basis.


Nasogastric tube (NG) – tube inserted through the child’s nose and down into the throat.  This is used for children who are having trouble taking a bottle.  Babies are fed via an NG tube while at the hospital and at home.  Parents and caregivers are trained by the hospital to change the tube.


Nasopharyngeal tube (NP) – a short rubber tube that is inserted through the nose. It goes as far as the back of the throat. It’s main job is to keep the airway open so that the child can breathe with a little less effort. Sometimes, it is inserted at night so that the baby could obtain deep sleep without desating.


Nebulizer – apparatus that produces a fine mist or spray.  Can be used to pass water droplets through a tube to provide moisture for a child with a trach or can be used to pass medication into a breathing apparatus for a child with asthma.


Neonatologist – physician whose specialty is studying and caring for newborn infants up to one month of age with special health needs.


Neurosensory hearing loss – is caused by nerve damage or dysfunction. Sounds reach the inner ear but are not transmitted to the brain by the nerves as they should be. Usually both ears are affected.


Nissen wrap (fundoplication) – A surgical procedure in which the lower esophagus and stomach are manipulated, and a zone of increased pressure is created in the lower esophagus. The increase in intraluminal pressure will discourage the reflux of stomach acid back into the esophagus.  The stomach is wrapped around the esophagus, thus lessening the chance for the back flow of stomach acid.


O2 – oxygen


Occupational therapist (OT) – one who evaluates the self-care, work, play and leisure time tasks performance skills of individuals.  This person evaluates and implements programs to accomplish basic daily tasks.


Ophthalmologist – a medical doctors that treats eye disorders and performs surgical procedures on the eye.


Oral defensiveness – this is what can happen when the baby is tube fed completely and there is no oral stimulation. They will not allow anything in or around their mouths. It is the first obstacle to beat in order to oral feed your child.


Oral surgeon – a doctor that performs surgery on the mouth.


Orthodontist – a type of dentist that generally does work with braces for children.


Otis media – inflammation of the middle ear.


PH Probe – a 24 hour pH monitoring, typically by inserting a small probe through the nose and into the esophagus. The probe remains in place for 24 hours and readings and events are recorded automatically and manually by the patient


PRS – Pierre Robin Sequence


Palate prosthesis – artificial plate often used to cover the opening in the palate to aid the child with eating issues related to PRS.


Passy Muir Valve – one way valve that attaches to the hub of a tracheostomy tube and allows air to be directed up through the larynx and pharynx enabling speech as air passes through the vocal cords and through the oral and nasal cavities.  Our parents have found this can help with swallowing in some cases.


Pediatrician – medical doctor that is responsible for the “normal” care of a child.


Pharyngeal Flap – surgery to reduce nasality in speech, a rectangle of tissue from the pharyngeal wall is used to build an artificial non-normative structure that bridges the difference the soft palate has to cross to accomplish closure during speech.


Pharyngoplasty – surgery to reduce nasality in speech, done by turning and reattaching mucosa in the pharyngeal area.   This creates a loose sphincter which acts like a drawstring to help close off the airway during speech.


Physical Therapist (PT) – individual responsible for planning and conducting a program to assist individuals with mobility issues.


Plastic surgeon (PS) – physician that generally performs the cleft palate repair and any surgeries to enhance speech.  This person is usually the director of the cleft palate or craniofacial team.


Polyhydraminos – an excess of amniotic fluid in the bag of waters in pregnancy.


Positioning – the placement of an infant with PRS.  Many babies with PRS need to be on their stomachs, so their tongue will fall forward.


Prosthodontist – a dentist who specializes in the mechanics of making and fitting artificial appliances for the mouth.


Psychologist – a member of the team that is responsible for the mental well being of the child and the other family members.


Pulmonologist – medical doctor who is responsible for breathing issues, including the lungs.


Pulse/oximeter – a machine that will indicate your child’s pulse and oxygen saturation level.  It can be set to alarm if levels go below or above

a certain predetermined level.


Reflux – a return or backward flow (regurgitation) of stomach acid back through the esophagus.


Respiratory therapy – treatment to preserve or improve pulmonary function.


Respite care – providers that either come into your home or provides a place for you to take your child so you can have some time to yourself.  These providers are normally nurses, if your child has a trach.  Some state agencies pay for them and sometimes families get a reduced rate from other sources.


Retraction – drawing backward, usually referred to when a child is having trouble breathing.  The ribs are visually seen pulling back with the strain of breathing.


Retrognathia – recessed jaw


Sequence – the order of occurrence of a series of related events.


Silver Nitrate – A compound used to remove granulation tissue.


Sleep study – this is a study done overnight. The child is attached to a Pulse Oximeter with a print out. The doctors check to see the number

of times a child desats while in deep sleep.


Social Worker – person trained to assist parents with issues regarding their child.  This person would have a good knowledge of services and agencies available to help the family both while the child is in the hospital and after the child is home.


Soft Palate – Soft Palate – the back portion of the roof of the mouth containing muscles and mucosa (pink “skin”), also called velum.


Speech Language Pathologist – professional that assists your child with his or her speech.  This person can also help with the feeding and swallowing issues.


Stickler Syndrome (SS) – a connective tissue disorder that is found in about 25-30% of those with PRS.  It can affect hearing and vision and include some characteristic facial features.


Stoma – a surgically created opening like the opening in the neck where the tracheostomy tube is located, also the opening in the stomach where a g-tube is located.


Stridor – harsh noisy sound during respiration; high pitched, rattly and resembling the blowing of wind, because the air passages are obstructed.


Sub mucosal cleft – a type of cleft where there is no actual opening in the palate, there is skin present but a lack of muscles above the skin.  Often hard to diagnose.


Suction Catheter – long, narrow flexible tube used to remove tracheal secretions (mucous) from the tracheostomy tube.


Suction – the process of removing tracheal secretions (mucous) from the tracheostomy tube using a suction catheter attached to a suction machine.


Syndrome – a group of symptoms and signs of disordered function related to one another by means of some anatomical, physiological or biochemical peculiarity.


Tongue/lip adhesion – surgical procedure using anesthesia which involves pulling the tongue forward, over the lower lip and stitching it to the lower lip. An alternative to tracheostomy. Usually undone at time of palate repair.


Tongue tied – tightness of the frenulum (the material on the underside of the tongue).


Trach ties – twill tape that ties around the patient’s neck and secures a tracheostomy tube in place. Patients may use other types of trach ties such as Velcro straps, metal trach chains, colored cotton shoelaces or umbilical cord tape.


Tracheomalacia – a congenital weakness and floppiness of the walls of the trachea (main airway) caused by a defect in the rings made of cartilage that keep the trachea open.  Manifestations would include:  stridor breathing, hoarseness, and difficulty feeding.


Tracheostomy – an operation in which an opening is made into the trachea and a tube is inserted to maintain an effective airway.


Tracheostomy tube – the tube that is inserted into an opening in the trachea to assist with breathing. This tube is what is commonly referred to by people as a “trach”.


Tracheotomy – Cutting of an airway into the trachea


Uvula – small soft structure hanging from the free edge of soft palate in midline above the root of the tongue.   It is made of muscle, connective tissue and mucous membrane.


U-Shaped Cleft – cleft palate that is in the shape of the letter U.


V-shaped Cleft – cleft palate that is in the shape of the letter V.


Velum – the Latin name for the soft palate.


Videofluoroscopy – a tape recorded x-ray examination of the speech mechanism during function, focusing on the soft palate (velum) and walls of the throat (pharynx).  Useful in assessing velopharyngeal function.


Videonasalendoscopy (VNE) – a procedure used to check the ability of closure during speech.  The nose is anesthetized by squirting a substance in one nostril.  The doctor inserts a very small scope with a camera on the end and lowers it far enough to watch how the palate moves to close off the air escaping through the nose.