Vinh International Hospital: Successful surgery for dangerous strange disease in children

(Baognhean) - Vinh International Hospital has just successfully performed surgery on a congenital constriction ring case for a 4-month-old patient.

Baby Nguyen T. A, 4 months old, was brought to Vinh International Hospital by his family for examination, with 2 forearm constrictions, 1 lower leg, 1 finger, and clubfoot deformities, extra toes on the right foot. After examination, the patient was diagnosed with congenital constriction syndrome.

Surgery for congenital constriction disease for children at Vinh International Hospital.

Although it is a rare disease, the risk of leaving deformities in children is very high. If not operated on early, children may have atrophied limbs due to lack of blood supply; even necrosis of the hands and feet. However, at present, many parents still do not fully understand this disease to have timely treatment for their children. Some parents mistakenly think that their children have many deep folds on their hands and feet because they are chubby.

According to Dr. Tran Van Thuyen, Vinh International Hospital, congenital constriction is a rare syndrome with an incidence rate of 1/1,200 - 1/1,500 children. The disease occurs during fetal development, when the amniotic bands (Amniotic Band Syndrome - ABS) wrap around parts of the fetus, causing blood to not circulate in that part of the body. Even if the amniotic bands are completely tightened, it can lead to amputation, limb deformities such as syndactyly, clubfoot. If the amniotic bands attach to the head, face or neck, it can lead to deformities such as cleft lip, cleft palate, etc. More dangerously, if this band attaches to the umbilical cord or body, it can cause stillbirth.

In the case of TA, doctors will perform surgery early to release the constriction rings, avoiding limb necrosis due to lack of blood supply. This is a difficult surgery because the baby is too small, the rings tighten important structures such as bones, tendons, blood vessels and require the doctor to be extremely careful. With just one inaccurate operation, the doctor can cut into the baby's surrounding structures, causing damage later.

Children with congenital constriction ring syndrome.

The patient after surgery for congenital constriction of the hand.

The doctors have performed the first steps of surgically removing the constriction ring, followed by releasing the thick fibrous tissue surrounding and tightening the blood vessel structure, nerves, tendons and muscles at the constriction ring position. The constriction ring in Nguyen TA's arms and legs has been operated on and returned to normal. The clubfoot deformity and extra toes on the right foot due to the constriction ring will be operated on later by the doctors. After the surgery, the child's arms are free from the constriction ring, the blood vessels circulate well, and the surgical wound is dry. And this treatment process is divided into 2 stages, 3-6 months apart, to be completed.

Currently, medicine has not found the exact cause of constriction ring syndrome in children. Scientists have proposed a number of hypotheses: First, there is an abnormality in the embryo, creating adhesions during development that cause contractions of the fetus's organs. Second, related to amniotic rupture. When spontaneous rupture of the amniotic membrane occurs, it will separate tissues from the inner amniotic membrane, creating many small, thin fibers. These fibers can get caught in the arms and legs and cause constriction. The third hypothesis refers to trauma in the uterus during amniocentesis or fetal surgery. An injury in the uterus can lead to bleeding, creating adhesions that wrap around the limbs of the fetus, causing constriction.

Dr. Tran Van Thuyen noted that parents need to clearly distinguish whether their child's limbs have folds due to being chubby or due to a constriction ring in order to treat them early; avoiding bad risks to the child's health. Chubby limbs have very shallow skin folds that are not contiguous. Meanwhile, the constriction ring is very deep and tightens the entire circumference of the leg or arm. This syndrome is not hereditary and there is no way to prevent it. However, congenital constriction ring syndrome can be detected prenatally through 3D and 4D ultrasound. Pregnant women who fall into this case need to have regular prenatal check-ups, and the family also needs to have early treatment plans for treating the child's malformations later.

Dinh Nguyet