Common Terminology

Most likely every parent that hears the news their child has Jeune's, is likely to find out every single thing they possibly can about the condition. This can be a feat within itself, as many of the terms related to Jeune's Syndrome will be words you will likely not be familiar with. We've compiled a list of the most common words having to do with the conditions.


Improper development of cartilage at the ends of the long bones, resulting in a small skeleton.

Alveolar hypoventilation

Deficient ventilation of tiny, thin-walled, capillary-rich sacs in the lungs where the exchange of oxygen and carbon dioxide takes place that results in reduction in the oxygen content or increase in the carbon dioxide content of the blood or both.

Anterior Mediastinum

The area in the front of the chest behind the breastbone.


Implies the front of an area (as opposed to the back).

Anterolateral areas of the rib cage

The area of the rib cage that is behind the arm as it hangs down at rest.


Temporary absence or cessation of breathing


The colored ring around the nipple

Arterial vessel wall thickening

Thickening of the sides of the blood vessels that carry oxygenated blood away from the lungs and heart


Inability to breath


Smothering or suffocating so as to cause inability to breath

Asphyxiating Thoracic Dystrophy

Another term for Jeunes Syndrome which describes the syndrome: Asphyxiation (inability to breath) because of dystrophy (malformation or deformity) of the thorax (chest).


The taking of foreign matter into the lungs with the respiratory current

Autosomal recessive disease

Describes an inherited disorder that can be passed on to an offspring from a parent who doesn't have that same disorder

Barnes Syndrome

A very similar syndrome to Jeunes Syndrome but also associated with a very small voice box and some other findings. The chest wall problem is the same as with Jeunes.

Bilateral expansion

To surgically enlarge both sides of the chest


Both sides


Disease of the small airtubes that makes them too soft so that they collapse when air tries to leave the lung.


The deposit of bone like material containing calcium- particularly in the bed of ribs when the rib has been removed from the bed.


A usually translucent somewhat elastic tissue that composes most of the skeleton of vertebrate embryos and except for a small number of structures (as some joints, respiratory passages, and the external ear) is replaced by bone during ossification in the higher vertebrates

Chest roentegenographs

An X-ray of the chest

Chest Tube

Surgical insertion of a hollow, flexible drainage tube into the chest. Chest tubes are inserted to drain blood, fluid, or air and allow full expansion of the lungs. The tube is placed between the ribs and into the space between the inner lining and the outer lining of the lung (pleural space).


A hereditary skeletal disorder characterized by improper growth of the cartilage portion of the ribs


Relating to the joint between the bony portion of the rib and the cartilage portion

CT Scan / Imaging

A special kind of x-ray study which, with the use of computer analysis, can create 3 dimensional pictures of the chest cage and its contents.

Diffuse rhonchi

A kind of sound in the chest you can hear with a stethescope that comes from partially blocked small airways


Any type of body situation that results in a very small person. Jeunes syndrome is one form of dwarfism.

Ellis-van Creveld syndrome

Ellis-Van Creveld Syndrome is a rare genetic disorder characterized by short limb dwarfism, additional fingers and/or toes (polydactyly), abnormal development of fingernails and, in over half of the cases, congenital heart defects. This disorder is inherited through an autosomal recessive trait

Endochondral bone formation in utero

A situation where, before birth, bone is formed where there should be cartilage instead. 

Fractional lung volumes

One of several measurements that can be made from a breathing test that helps to understand the mechanics of breathing in an individual patient.

Gentamicin aerosols for tracheitis prophylaxis

An antibiotic spray used to help prevent infection in the windpipe

Hilar vessels

Large blood vessels going to and from the lung at the root of each lung where it meets the heart


Water on the brain that makes for a very large head


Small or underdeveloped

Intercostal muscles

Any of the short muscles that extend between the ribs filling in the space between them and serving to move the ribs in respiration

Jeunes Syndrome

A potentially deadly form of congenital dwarfism characterized by a very small chest wall which can restrict the lungs and make breathing difficult or impossible. It may be severe requiring breathing machines in infancy or mild allowing survival into adulthood. In some cases there are associated problems with the kidneys.

Lateral thoracic expansion

An operation on the chest wall which spreads the ribs apart and creates a bigger cavity inside

Lung alveolar growth

Growth of the air cells of the lungs

Lung tissue disease

Disease that effects the tissues of the lung as opposed to the airtubes of the lung or the space surrounding the lung

Malacic airway

Any disease involving the airtubes which takes away the normal stiffness which is required to easily move air, and renders them too soft

Mediastinal structures

Structures in the middle of the chest cavity


The region the center of the chest between the lungs which includes the heart, windpipe, swallowing tube and certain glands



Parietal pleura

The inside lining of the chest wall that lies beside the outside of the lung

Pectus Carinatum

A deformity of the chest wall characterized my a major protrusion of the breastbone forward; also called “pigeon breast”

Pectus Excavatum

A deformity of the chest wall characterized by a major indentation of the breastbone; also called “funnel chest”


The collection of bones at the base of the belly that connects to the legs

Periosteal bridges

A situation where two adjacent ribs heal together to form a bridge of bone between them

Periosteal elevator

A surgical instrument which is used to scrape off the rib lining from the rib itself


The bed in which the ribs lie. If the ribs are separated from the rib bed, the rib bed can form more rib.


Fingers or toes

Plastic surgery

The branch of surgery concerned with restoration, reconstruction, or improvement of defective, damaged, or missing structures.


Lining of the lung cavity: "visceral pleura" lines the outside of the lung; "parietal pleura" lines the inside of the chest wall


An infection of lung tissue


A situation where cartilage is formed improperly in many different parts of the body


Situated behind

Prosthetic material

Any artificial material used for the replacement of missing body parts

Pulmonary hypertension

High blood pressure in the lungs (as opposed to high blood pressure in the body)

Pulmonary hypoplasia

Under-development of lung

Pulmonary parenchymal

Referring to the tissues of the lung as opposed to the airtubes of the lung or the space surrounding the lung


A subspecialty of internal medicine concerned with the study of the respiratory system. It is especially concerned with diagnosis and treatment of diseases and defects of the lungs and airtubes.


A specialty concerned with the use of x-ray and other forms of images in the diagnosis and treatment of disease

Renal dysfunction

Malfunctioning of the kidneys

Single lumen endotracheal anesthesia

Anesthesia administered by one single breathing tube, as opposed to a divided tube which can treat the two lungs differently

Sternal split

A technique to enlarge the chest by dividing the breastbone in the front and spreading it apart




Under the skin

Subperiosteal rib osteotomies

Dividing the rib separately from its underlying rib bed


Anything to do with the chest or its contents (heart, lung, etc)

Thoracic cage

Anything to do with the chest cage

Thoracic surgery

Surgery involving the area of the chest: chest wall, heart, lungs and esophagus


Surgical incision into the chest wall


The chest

Titanium struts

Small metal bars made of Titanium that can accommodate screws that go into the rib to hold two ends of rib together while they heal


 A highly technical x-ray system with a computer program that puts together many different xrays taken from many different angles and reconstructs a single two-or-three dimensional picture of an area of the body


Disease of the main windpipe that makes it too soft so that it collapses when air tries to leave the lung


Surgical construction of an opening in the trachea for the insertion of a catheter or tube to facilitate breathing

Transverse incision

A side to side incision (as opposed to an up and down incision


Breathing machine that moves air into and out of the lungs

Ventriculoperitoneal shunt

Surgical placement of a plastic tube to drain excess fluid from the brain and divert it into the abdomen, therefore preventing hydrocephalus or "water on the brain"