Seemingly surgery does not influence the high incidence of neurodevelopment problems in patients with metopic synostosis. Neurological disorders such as ADHD, ASD, ODD and CD are seen in patients with trigonocephaly. These disorders are usually also associated with decreased IQ. The presence of ADHD, ASD and ODD is higher in cases with an IQ below 85. This is not the case with CD which showed an insignificant increase at an IQ below 85. Epidemiology
The incidence of metopic synostosis is roughly between 1:700 and 1:15,000 newborns globally (differs per country). Trigonocephaly is seen more in males than females ranging from 2:1 to 6.5:1. Hereditary relations in metopic synostosis have been found of which 5.5% were well defined syndromic. Maternal age and a birth weight of less than 2500g may also play a role in trigonocephaly. These data are based on estimations and do not give factual information. Only one article gives valuable and reliable information regarding the incidence of metopic synostosis in the Netherlands. The incidence in the Netherlands showed an increase from 0.6 (1997) to 1.9 (2007) for every 10,000 live births. History
In former times people born with malformed skulls were rejected based upon their appearance. This still persists today in various parts of the world even though the intellectual development is often normal.
Volkmanns contracture is a permanent flexion contracture of the hand at the wrist, resulting in a claw-like deformity of the hand and fingers. Passive extension of fingers is restricted and painful. Causes
Any fracture in the elbow region or upper arm may lead to Volkmanns ischemic contracture, but it is especially associated with supracondylar fracture of the humerus. It is also caused by fractures of the forearm bones if they cause bleeding from the major blood vessels of the forearm.The condition may be caused by obstruction on the brachial artery near the elbow, possibly from improper use of a tourniquet, improper use of a plaster cast, or compartment syndrome. Pathophysiology
Volkmanns contracture results from acute ischaemia and necrosis of the muscle fibres of the flexor group of muscles of the forearm, especially the flexor digitorum profundus and flexor pollicis longus. The muscles become fibrotic and shortened. Prevention
Prevention of the condition requires restoration of blood flow after injury and reduction of compartmental pressure on the muscles. Any splints, bandages, or other devices that might be obstructing circulation must be removed.A fasciotomy may be required to reduce pressure in the muscle compartment. Treatment
If contracture occurs, surgery to release the fixed tissues may help with the deformity and function of the hand. History
It is named after Richard von Volkmann (1830–1889), the 19th century German doctor who first described it, in a paper on "non-Infective Ischemic conditions of various fascial compartments in the extremities".