Congenital Limb Deformity: Replace, Repair or Remove
By Danielle Sendou Ringgit
Did you know that congenital limb deformities occur in approximately 6 per 10,000 live births, with upper limbs affected commonly compared to lower limbs?
Congenital deformities, also known as birth defects, is a condition that exists at or before birth and can be caused by a genetic or non genetic disorder.
According to orthopedic surgeon and Professor of Orthopaedics at Unimas’ Faculty of Medicine and Health Sciences Professor Dr Ahmad Hata Rasit, among the factors need to be considered when treating a child with congenital limb deformity are the severity of the deformity, parent’s and family belief, socioeconomic factor and the child’s overall wellbeing.
“We have to consider all the factors and before we decide on surgery, we must consult the parents and remember that every parent had their own privacy and different needs, so do not compare them to yourself,” he said, adding that each family may come from different financial background.
“Imagine having one child with congenital limb deformity, what if you had six? It is a distress to the family, so when we prescribe treatment, we have to be balanced; consider the family and their socioeconomic situation and also the child’s wellbeing,” he said.
According to Dr Ahmad who delivered his inaugural lecture on ‘Congenital Limb Deformity: Replace, Repair Or Remove’ at the Islamic Center, UNIMAS on March 2nd, there are three phases of the grieving process: denial followed by anger and later distress.
In the denial phase, parents tend to minimise the impact of the deficit. It is important to collect as much information and history to avoid making incorrect assumptions about the child’s condition.
In the anger phase however, parents might become angry at the surgeon’s failure to diagnose the disease prenatally.
In the distress phase, parents will normally experience guilt and anxiety about future pregnancies and may feel loss of control. Genetic counseling during this phase would be recommended.
Caused by a variety of factors such as infections, chemicals, genetics, environmental or combination, the deformity can be easily recognised and may need additional investigation such as radiography, ultrasound or genetic analysis for further investigations.
Explaining to the audience the many types of limb deformities during the talk, Dr Ahmad said that there are four major categories: malformations, deformities, distruptions and dysplasias.
“Congenital limb deformity is a rare disease and can happen due to multiple causes. It is easily recognised and may need additional investigation. The treatments include replacing with artificial limbs, reconstruction of limbs and surgical amputation or the combination of these,” said Dr Ahmad.
Artificial limb treatment is most useful in conditions such as complete or partial absence of limb known as amelia which is most valuable for lower limb deficiencies.
According to Dr Ahmad, the device used must be simple and durable and it is very important to assess the functioning capacity of the limb before recommending an artificial limb.
Surgical reconstruction is most beneficial for cases of syndactyly, where some or all of the fingers or toes are wholly or partly united, either naturally (as in web-footed animals) or as a malformation.
Noting that timing and planning is important for this treatment option, Dr Ahmad said that the timing of the surgical repair should be as early as possible if the neurovascular function distal is compromised.
The option to remove or surgically amputate is normally performed in cases such as duplication like polydactyly (extra fingers on the hands or toes on the feet) and duplicated foot or mirror foot (which needs both surgical amputation and reconstruction of the functional foot).
Graduating from the National University of Malaysia in 1994, Dr Ahmad furthered his master of orthopedic surgery in 2001 and is now serving as a senior consultant orthopedic surgeon in Sarawak General Hospital (SGH).
As an orthopaedic surgeon, he provides clinical service to patients with orthopaedic problems admitted to SGH.
In 2002, Dr Ahmad set up a pediatrics orthopedic service in SGH after the increasing number of pediatric orthopedics cases in Sarawak.
After serving at SGH over the last 15 years, Dr Ahmad said that he has seen about 200 cases of of congenital limb deformity where most of them were of congenital talipes equinovarus (CTEV), cases better known as ‘club foot’.
“Most of those who have club foot can be treated without the need for surgery, only about 10 per cent of the cases would need surgery,” said Dr Ahmad.
Explaining further, he said that most cases would only require to be treated with serial manipulation and weekly casting to correct the deformities.
“In Sarawak, we have trained every hospital in Sarawak how to carry out treatments and later would train the orthopedic specialists, medical officers and nurses so that they will be more well informed about it. We hope that the awareness will reach out to the community easily,” said Dr Ahmad.
“Those who have children with congenital limb deformities may refer them to any government hospital and later they would be referred to SGH, where we would proceed with further treatment,” he added, referring to the training which has already started this year in UNIMAS.
Dr Ahmad also stated that perhaps by the middle or end of the year, the training would also begin at Sibu, Miri and Bintulu.