Causes, Symptoms, Treatment, and Diagnosis of Hemihypertrophy
What is Hemihypertrophy?
Hemihypertrophy is an uncommon medical condition defined by the greater-than-normal asymmetry of the body. The one side of the body in hemihypertrophy is growing at a faster rate than the other side of the body. The condition however can only affect one limb, finger, foot, face or the entire half of the body including the half of the brain and internal organs.
Hemihypertrophy is generally a harmless condition although this depends on the part of the body that is affected. Although harmless, hemihypertrophy is still a cause for concern as the condition is an indication of cancer. Normally, every individual has a cell in the body that triggers growth which generally stops once the certain size has been attained usually on a certain age. In hemihypertrophy, there is a presence of an abnormal cell that triggers the body to continually grow and increase in size even if the ideal size at a certain age has been reached.
Hemihypertrophy is a congenital disorder or that which is present at birth. It can equally affect both genders and without racial predilection. This congenital disorder may be harmless but the adverse effect may occur depending on which part of the body involved in the abnormal growth rate. Children with hemihypertrophy on the other hand are at increased risk for tumors particularly in the abdomen while the tumor growth may or may not be cancerous.
No individual is exactly symmetric but it is important to recognize the distinct characteristic of hemihypertrophy to recognize the potential health risks from having the condition.
The prominent symptom of hemihypertrophy is the asymmetry in one side of the body. The condition may be subtle affecting only a finger or it may be profound affecting the entire half of the body.
Hemihypertrophy may be simple and affecting only a toe or a finger or it can be a complex hemihypertrophy affecting the entire half of the body. Crossed hemihypertrophy involves both sides of the body while a hemifacial hyperplasia is characterized by an overgrowth on one side of the face.
Leg length discrepancy of hemihypertrophy is characterized by an overgrowth of one leg which cause for an orthopedic concern. Overtime, this leg length discrepancy can result to scoliosis or a curvature in the spine as a result of unequal leg length. Problem with walking or gait is common in children with leg length discrepancy of hemihypertrophy.
Facial distortion is also another concern for those with hemihypertrophy involving the face. The asymmetry in the facial bones may result to cleft lip or cleft palate or may have a tongue longer than the average size thereby affecting the functions of the tongue such as in speech.
In hemihypertrophy, an affected individual may have acne on only one side of the body while the other has none. The appearance of the hair may have thicker hair on one side of the head while the other is not. The skin may also have a difference between the left side and the right side of the body which may have the skin on the other side thicker than the other half. The arm or the leg on either side of the body may be seen with the apparent difference although hemihypertrophy rarely have one leg affected spontaneously involving the arm on the other half of the body.
Children with hemihypertrophy may grow with two sides of the body growing constantly. The difference however becomes apparent once the child has reached the stage of puberty or when the child reaches the age where growing is expected to cease.
The exact cause of hemihypertrophy remains unclear although the congenital disorder is believed to run in the family. Genetic mutation is also believed to play a role in hemihypertrophy particularly mutation in gene 11. Genetic involvement on the other hand is seen to differ from one person to another.
The onset of hemihypertrophy in some children is seen to be associated with other genetic syndrome such as:
Beckwith-Weidemann syndrome is an overgrowth syndrome that affects multiple parts of the body. It is a growth regulation disorder that may not always be present at birth although children born with this syndrome are often seen with omphalocele or an opening in the abdominal wall which results to the protrusion of the abdominal organs towards the navel. The syndrome is marked by macrosomia or the overgrowth of an infant relative to height and weight compared to average growth of infants. Beckwith-Weidemann syndrome is also characterized by an uneven symmetry in specific parts of the body.
Klippel-Trenaunay-Weber syndrome is an uncommon disorder of the vascular that involves the limb that is marked with port-wine stains or capillary malformations in the skin. The syndrome is also characterized by an overgrowth of the soft tissue of the bones although it commonly affects a single limb. Vascular anomalies are also seen in individuals with Klippel-Trenaunay-Weber syndrome such as the occurrence of varicose veins. The profound characteristic of the syndrome is the asymmetric limb hypertrophy where the leg or arm on one side of the body is larger than the other half of the body.
Neurofibromatosis is a genetic disorder characterized by a disruption in the cell growth in the nervous system. The disruption causes growth of tumor on the nerve tissue and the tumor may be benign or malignant. The tumor may develop in any part of the nervous system such as in the brain, the spinal cord or in the nerves.
Diagnosing hemihypertrophy is rather difficult due to its uncommon occurrence and similarity with other genetic syndrome. A child born with Hemihypertrophy is often attended to by a pediatrician who then refers the patient to a geneticist for further evaluation and possible associations of the syndrome.
The physical exam is the initial step in assessing the condition of hemihypertrophy. A dramatic difference of 5% in size and length in relation to both the left and right of the body is a guideline in identifying hemihypertrophy.
The discrepancy between the two sides of the body generally increases as the child continues to grow and where the discrepancy in some children doubles in size and length between the ages of 1 year and 5 years of life. Hemihypertrophy is further isolated from other genetic conditions with similar characteristics.
Hemihypertrophy is generally not a life-threatening condition except in cases when it is associated with other forms of cancer. Children born with hemihypertrophy are usually at increased risk for cancer particularly in the abdomen.
No treatment has been identified to directly cure Hemihypertrophy. The management of the condition is directed towards the concerns of other medical conditions that may occur with the condition. The immediate concern is focused towards the development of cancer that a child with Hemihypertrophy is regularly monitored and screened until complete child growth has been attained.
Orthopedic problem is the next concern of management. The discrepancy in the length and size of the limbs may result to abnormal curvature of the spine or scoliosis. The orthopedic problem can be addressed by equalizing the length of the legs through lifts or special shoes or may be equalized through surgery.
Facial discrepancies may warrant plastic surgery to correct the discrepancies and repair cleft lip and cleft palate. This form of surgery is generally attended by craniofacial team or by those who are licensed and expert in repairing both the cleft lip and cleft palate.
Hemihypertrophy is a condition that does not disrupt the normal life span of an affected individual. It is however a condition that can result to complications brought by the associated abnormalities which can therefore affect the quality of life. This can cause a child to have a low self-esteem and may cause a social stigma when the child grew up or has reached the stage of puberty when during this stage a child is expected to mingle with peers and others within the age group. Family support is necessary to help the child understand the present condition. Involvement of parents in the process of treatment proved to be effective that parents are encouraged to take their part in the treatment of their child with Hemihypertrophy.