Life rarely gives us a second chance, however in the case of a newborn baby boy recently born at Ajman’s GMC Hospital, a dedicated team worked diligently to correct his extremely rare condition and put him on the road to recovery. HEALTH met with the medical team who made it happen…

The Condition

A synergistic pediatric team comprising of Consultant Pediatric Surgeon Dr. Lalit Parida, Consultant in Neonatology Prof. Dr. Zareen Fasih, and Sanju Daniel, NICU Incharge worked hard to correct an extremely rare condition known as congenital right-sided diaphragmatic hernia (CDH) in a baby boy born on March 5, 2015 at GMC Hospital Ajman. This is a disorder in which the baby’s diaphragm has a hole in it because it hasn’t formed properly in the womb. What this means is that the organs in the baby’s tummy can push through the muscle, squishing his lungs and preventing them from developing properly. The risk of right sided CDH is 1 in 20,000 babies.

The Symptoms
Dr. Zareen explains, “We received a baby from the labor room and as the baby had respiratory distress, we treated this baby boy as a case of congenital pneumonia. After the apparent chest infection was not settling down, we then needed to see other problems associated with this baby. We repeated a chest x-ray on day three which did not look good. It indicated the intestines and part of the liver were in the right side of the chest. This is not common at all. Usually we see this pathology on the left side. More importantly, we have ante-natal diagnosis in which we do ultra sound on mothers during pregnancy, but in this case the scans did not show any evidence of right sided CDH. More so, the first chest x-ray and ultrasound done on day one did not show diaphragmatic hernia.”

The Diagnosis
After that, the baby underwent a CT scan of the chest and abdomen which confirmed the findings. Dr. Zareen then consulted with Dr. Lalit Parida and the decision was taken to operate and was in fact the first neo-natal surgery case ever done at GMC Hospital. According to Dr. Zareen, typically when pneumonia is expected, the baby is placed on antibiotics and normally the baby shows a response. That’s clinical recovery. She adds, “However, in this case we weren’t seeing clinical recovery at all from antibiotics. Otherwise the baby was a good weight and healthy otherwise.”

The Surgery
On March 14, Dr. Lalit met with Dr. Zareen to examine the baby’s CT scan of the chest which showed presence of the liver on the right side.
Dr. Lalit continues, “In this case, 75 to 80 percent of the liver was in the baby’s chest cavity along with the small intestine and the large intestinal loops. It was mechanically pressing the right lung and heart all squishing up to the left side. The heart is supposed to be more in the center but in this case was completely shifted to the left side. In this case, the baby was breathing using a single lung.” It was for this reason Dr. Zareen had put the baby on a mechanical breathing machine to ease the baby. Secondly in this case, the team had to examine if there were any defects in the heart using heart ultrasound or echocardiogram. It was done and revealed as normal. After this, Dr. Lalit counselled the parents who were surprised by the diagnosis. He explained the diaphragm is a thick muscle which separates the chest cavity from the abdominal cavity that is important in terms of breathing. In this particular case, it was a huge defect as almost half of the diaphragm had split apart. From this right defect, Dr. Lalit explains that the liver had gone into the chest as well as the large and small intestinal. “In terms of surgery, I took very high risk consent because whenever the liver goes up certain veins of the liver (hepatic veins) are twisted at its connection to the main vein of the body,” he says, so when pushing the liver back these could potentially separate and cause bleeding. This can cause a baby to lose blood very quickly during surgery and can potentially result in death during surgery.

So as far as the surgery preparations were concerned, Dr. Lalit points out that sometimes these defects are so huge that they cannot be joined by simple stitches. “Sometimes it may require a plastic surgical mesh and that was procured on an urgent basis by the purchasing department in addition to other specialized surgical tools required,” he says. However, a mesh was not required during this surgery.

Anesthesia
The surgery was done under general anesthesia which Dr. Lalit tells is a first for a baby of just 12 days of age at our hospital. He adds, “Every aspect of this surgery had its own set of risks we could have lost the baby at any point but there were concerted efforts of the nursing department, anesthesia department, the pediatric department, as well as the obvious inputs from the surgery department.”

The Surgery Details
The liver was brought back surgically and the hole in the diaphragm was closed which is called Primary repair of the diaphragm just using sutures. Beyond that, Dr. Lalit explains the lung was also expanding well which is good. “Once the liver is brought back, we put in a plastic tube into the chest called a chest tube in case the baby develops fluid or air and all intestines were brought back into the abdomen and the abdomen was closed with sutures,” he says. The baby did quite well and was on a breathing machine initially and well taken care of by the neo-natal unit with excellent care. Prof.Dr. Zareen adds after the high risk surgery was the phase of critical postoperative period with the baby on mechanical ventilator support, fully sedated and paralyzed so that the wounds would heal nicely and for the lungs to expand properly. Also not forgetting, the slow and gradual feeding and fluids and electrolyte balance to be taken care of.

Great Teamwork
In particular, Dr. Zareen commends the marvelous job the nursing department did, both pre-operatively and post operatively, as well as the very experienced pediatric surgeon. She tells, “Whatever we can do, if the nursing team is not up to mark, there can be potential problems.
Certain special things were required from our nurses; one was minimal handling of the baby, proper ventilator care, and then of course special feeding instructions as this baby was not fed orally for 13 days. The technique of feeding was very gradual and slow and minute by minute. Some of the babies with this kind of diagnosis have other associated problems but fortunately this baby did not.”

Today 
Dr. Zareen and Dr. Lalit have seen him twice post discharge and points out he has gained 500 grams since discharge. Both doctors feel the baby has done exceptionally well and is well on his way to recovery.