I was again given the opportunity to deal with a very interesting, intriguing, puzzling case in my career as a neonatologist.
I was called for the delivery of a baby presenting with hydrops as seen during prenatal ultrasound. (Hydrops refer to the generalized swelling of the baby, and is defined as the presence of fluid accumulation in third spaces including pleural, peritoneal, pericardial spaces, edema of the skin, as well as swelling/thickening of the placenta). Accordingly, the ultrasound detected fluid in the abdominal cavity and testicular sacs, but none wheresoever. The consideration of hydrops is dubious but still it was highly considered. I called on the different personnel who will be involved in the baby’s care later on. Meanwhile, the obstetrician has already spoken to the mother (the husband is overseas) and explained the possible outcome of the baby’s birth. (Hydrops usually is dreadful, some expire within an hour). They were preparing that the baby might indeed expire immediately after birth.
Upon baby’s birth, we noted that the abdomen was distended, scrotum was bulging, as big as an apple. The skin was not edematous however. It seems that baby did not satisfy the criteria for hydrops. Baby had a weak breath, thus I had to intubate him. In less than an hour, I took an x-ray to confirm presence of fluid in other body cavities. Liver was palpable on examination (though it may be normally appreciated about 2 finger-breadth below the right subcostal margin, baby’s liver was about 4 finger-breadth palpable); there was also a soft mass I palpated at the right side, seemingly an enlarged spleen. Pleural space (where lungs are) is free of fluid. This at least ensures that baby’s breathing would not be difficult, unlike when there’s pleural effusion (fluid in the pleural space), the fluid will prevent full expansion of the lungs with every breath, letting to less oxgenation of the blood, which will make the heart work double time. There was a single bubble (signifying air entry into the stomach), but none for the rest. This is understandable as the x-ray was obtained just minutes after birth. It will takes hours before the air swallowed will pass into intestines, then about 24 hours until the air passes out of the anus.
I requested for ultrasound to see what in the abdomen is causing this disorder in him. But to my and the sonologist’s surprise, we saw nothing but a large liver. There was no tumor, the intestines were being pushed down though, ebbing and bobbing into the scrotal sac. The mass I palpated indeed was the spleen. But these don’t seem explain why there is fluid in the abdomen, and the scrotal sac enlarged, as seen in the prenatal ultrasound. With a liver enlarged, I tried to work up the baby along that line. I tested for blood samples reflecting liver functions, decided to repeat the x-ray of the chest and abdomen 6 hours after birth, this will give ample time for air to pass through to the rest of the intestines distal to the stomach.
Repeat chest and abdominal x-ray showed passage of gas into the distal intestine. This can eliminate pyloric stenosis (part of the stomach before duodenum) as a possible entity. Albumin was low, so I corrected.
But this wasn’t of a significant help. It won’t still explain why there was herniation of intestines into the scrotal sac, nor the large liver. It may explain the fluid accumulation, but that’s just it. I was at a loss. I was at a loss what next step to take. There were no other organ systems showing abnormalities, which might suggest a syndrome. It was a clear cut gastrointestinal system derangement. I didn’t shame to call on a gastroenterologist to help me solve this puzzle. I didn’t want to miss a thing for the sake of the baby, who I am being asked to do everything possible to make him survive. (A herculean request).
The gastroenterolist also seemed puzzled by the baby’s case. A toss between malignancy and… it was hard to come up with differential diagnoses… On baby’s second day of life, his abdominal girth dramatically increased, but remained soft. There was feces meconium coming out of the baby’s anus, though the color was like that of a caramel; there was no vomiting. This tells you that there seems no obstruction of the intestinal tract. But we wanted to sieve and scan through and don’t want to miss anything. Even if it was initially mentioned that CT scan might not be of help, we still did it. Surprisingly, the CT scan of the abdomen revealed only a large pneumoperitoneum (air inside abdominal cavity, but outside the intestines – which should never happen in a normal individual), fluid accumulation, but nothing more. This then puzzled me all the more; air can only escape into the abdominal cavity if there is a perforation along the intestinal tract. I then referred the baby to a pediatric surgeon, who scheduled baby for an emergency explore laparotomy.
At the operating room, the stomach, duodenum and jejunum were intact. At the middle of ileum however, there was a perforation, and an atresia (obstruction due to non-recanalization of the intestinal cavity during the development). The atretic portion, together with the perforated segment was then excised, washing and evacuation of the spilled fluid and meconium into the abdominal cavity and creation of ileostomy were done. We marveled at the actual operative findings in this baby because we never expected it to be so. Gladly, it wasn’t a cancer or a tumor. So, in retrospect, the perforation occured even before the baby was born. The reason and when it happened, we dont know. This lead to leakage of fluid and meconium into the abdominal cavity that incited reaction from nearby organs, including the liver which became enlarged. There were adhesions also of the intestines due to inflammatory reaction to the meconium that was spilled. The liver then produced low albumin as well that lead to the edema formation.
Baby did well during the operation. No untoward complications took place. We will have to play close attention to the function of the intestines as we have yet to commence feeding.
One week after the discharge, he came back for his return visit to the office. I was surprised upon seeing him. He was so malnourished, his skin was wrinkled and he looked severely dehydrated. In the medical parlance, that is a condition we refer to as “failure to thrive.” Painful as it may be, I had to admit baby again, to “rehabilitate” him from his dreadful status. It seemed that while on ileostomy, most of the milk he has been taking was not absorbed. Instead, he was dumping them and thus the resultant situation. (This is a condition known as short bowel syndrome).
I referred him back to the surgeon for further management. Finally, we agreed to have baby operated on again, to reunite the intestinal segments that were disconnected before to create an ileostomy. After preparing baby for the said procedure, the operation was finally carried out. It took about 72 hours when we noted that baby has patent intestinal segments, he was having regular passage of stools, hence we started feeding baby, initially minimally, then gradually increasing. After he was able to achieve his full enteral feeds, the venous line was discontinued. We even documented this by doing a fluoroscopy, to really check if the intestines are patent. And yes, the dye passed through without impediment through the intestines, no retention whatsoever. This made us then confident that baby’s problem has been resolved. So, for the remaining days of baby in the hospital, he was being fed, he was moving his bowels regularly, his weight noted to be increasing daily. And we were happy to send him home.
He was asked again for follow-up return after a week. And again, baby surprised me. His abdomen was now bloated like a balloon. Again, this puzzled us once more. He seemed to be having Hirschsprung’s disease and this just contradicts how he behaved during the last hospital stay. Well, we had to abide by the baby’s presentation. The surgeon again created an ileostomy, repeated fluoroscopy and obtained biopsy specimen. Then baby was back to his problem of short bowel syndrome. It was not a challenge how to resolve this but with the help of gastroenterologist, we were able to minimize him dumping watery stool. Baby was discharged once more with confidence that his problem has been resolved. Our aim then is to nutritionally build baby and then when bigger enough, will do the corrective surgery.
For now, baby is stable, with a slow weight gain… I really hope it works this time so baby will recover fully before his next surgery.