What is Ivemark Syndrome?
Ivemark Syndrome is an super uncommon pathologic condition sham multiple organ of the consistence . Ivemark Syndrome is qualify by underdevelopment or in some cases complete absence seizure of spleen , significant malformations of heart , and malformation in the manner the organ of the body like the pectus and abdomen are arranged within the soundbox .
The symptoms of Ivemark Syndrome are quite varying and depend on the organ organisation that is involved . Many children be given to have congenital nerve defects due to Ivemark Syndrome with them sustain bluish discolouration of the skin due to lack of atomic number 8 in the blood , warmness murmurs , and other sign of congestive heart failure such as shortness of breath .
Ivemark Syndrome tends to cause a number of complications some of which may be potentially life threatening . There is no real known cause of Ivemark Syndrome .
What are the Causes of Ivemark Syndrome?
As stated , the exact cause of Ivemark Syndrome is not known and most of the case have been stimulate for reasons not known . Researchers are of the belief that various genetic and environmental factors are at playing period in the development of Ivemark Syndrome .
In some case , Ivemark Syndrome has come in the phallus of the same family which may suggest that it may be an inherited precondition . Anatomically verbalize , during fetal development , the internal variety meat first develop and then they are placed either on the right or on the left side of the body .
In Ivemark Syndrome , this distribution does not take place resulting in malformed position of the chest and stomach . Researchers believe that sure gene mutant responsible for development of left - right asymmetry may be find fault for development of Ivemark Syndrome .
What are the Symptoms of Ivemark Syndrome?
The main symptoms of Ivemark Syndrome are due to misplaced and abnormal arrangement of some of the home organ of the organic structure , more specifically the pectus and the venter . The liver is misplaced near the meat of the body .
There is also abnormal locating of the bowel and hard underdevelopment and even in some grammatical case complete absence seizure of the irascibility . In Ivemark Syndrome , the unexpended and proper side of the fondness and lungs are not clearly or distinctly defined as is under normal case . This make babe with Ivemark Syndrome to have several cardiac flaw at birth .
The most common heart defect associated with Ivemark Syndrome is termed as doubled wall plug right ventricle . In this , the aorta and the pulmonary artery both rise from the correct ventricle or else of the left ventricle which should be the eccentric .
As a result of these center defect , there is blue discoloration of the pelt as a result of unequal O in the blood . There may also be presence of heart murmur vowel and other symptoms of congestive gist bankruptcy like curtness of breath and unyielding fatigue .
Some of the complicatedness stimulate by the heart defects as a result of Ivemark Syndrome can be potentially life threatening in infant . An developing or even over absence of short temper is yet another symptom of Ivemark Syndrome . A missing or underdeveloped spleen may predispose the small fry to frequent infection include sepsis which may again be life peril for the infant .
to boot , some infants with Ivemark Syndrome complain of sudden severe acute pain in the abdomen as a answer of malpositioned abdominal cavity or enteric malrotation . There may also be bilious atresia present in youngster with Ivemark Syndrome .
How is Ivemark Syndrome Diagnosed?
For a confirmed diagnosing of Ivemark Syndrome gross story of the patient will be taken and a thorough clinical evaluation will be done of the patient . The classical presenting feature of Ivemark Syndrome can be identified by require radiological studies of the abdominal cavity which will show malpositioning of the intimate organs of the abdomen .
Radiological studies may also let on an underdeveloped short temper which is a hallmark of Ivemark Syndrome . An echocardiogram may detect any mettle defect that may be present due to Ivemark Syndrome . A foetal echography before the birth of the child , may argue underdeveloped or absence of spleen and heart defects , which are the classic symptoms of this term , and will confirm the diagnosis of Ivemark Syndrome .
How is Ivemark Syndrome Treated?
The mainstay of discourse of Ivemark Syndrome is diagnostic . The treatment will require a multidisciplinary movement from a team of pediatrician , cardiologists , pediatric gastroenterologists who will evaluate and form a treatment design comfortably suit for the child suffering from Ivemark Syndrome .
touched children may require cardiac surgeries to decline some of the congenital heart fault . youngster with Ivemark Syndrome will also require preventive antibiotic therapy to forbid them from contagion as want of a fully developed lien will predispose them to frequent infections .
In case if a child has an contagion then it needs to be care for aggressively with antibiotic and the immunisation condition of the child should also be up to date in cases of children with Ivemark Syndrome .
