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Aside

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.

A Puzzling Abdomen

 
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Posted by on September 7, 2012 in Congenital Anomalies, neonates

 

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My Bargain With The Almighty

In commemoration of the Lenten Season, I’d like to share one of my Lenten experiences. This happened last year though.

I received a call of referral to attend to the delivery of a baby who was initially diagnosed  as a case of hydrops fetalis (baby is swollen all over his body).

Hydrops fetalis (not the actual case; photo courtesy: http://www.medadteam.org)

My case is that of a baby who is term, who was worked up (as mother is considered high risk, >35 years old primigravid –first time pregnant ) and initial ultrasound during middle of  pregnancy revealed hydrops fetalis. A repeat ultrasound again after one month described the baby to be hydropic. With this, parents were advised not to expect much, that the baby has a slim chance of surviving. Parents were then preparing (emotionally and psychologically) that baby will not survive for a long time after birth. Mother was admitted when labor started but cervix remained at 9cm for several hours. Because of this, a second opinion was obtained from another obstetrician, who repeated the ultrasound. The result was… UNEXPECTED! Baby had no hydrops, instead he has a mass (tumor) at the abdomen. So this then made us rattled and shift paradigm. Parents, who were resigned to the feeling of hopelessness, had to be informed that the initial consideration was wrong and the baby might still have a chance of making it in indeed this is a tumor. (A tumor can just be removed and then presto! Chemotherapy might also be done, if indicated). From the initial state of unpreparedness (emotionally, psychologically, financially) for a longer baby’s stay in the neonatal ICU, the parents now had to. (Mother is 40, a teacher, while father is a contractual laborer). After all, this is going to be their first baby. An emergency cesarean section was thus scheduled.

The baby was born weighing 3.6kgs, with noticeable swelling at the abdominal area. When baby was stabilized and brought to the NICU, ultrasound of the abdomen and surgical consult was in order. The ultrasound of the abdomen revealed a complex mass with calcifications, and an x-ray done afterwards was suggestive of meconium peritonitis. An emergency operation of the baby was scheduled but the parents (who were not expecting this) didn’t consent. I went back later in the day and tried to explain the findings of the surgeon, and that I think convinced them to sign the consent. (Meconium peritonitis means that a part of the intestine ruptured while the baby is still in the uterus, and thus meconium — baby’s stool, leaked into the entire abdominal cavity. This can cause the other segments of the intestines become irritated and develop a reaction against the meconium). The operation was successful, and the point of rupture was located, cut and then the intestines were then re-attached/re-anastomosed. A drain was in place that will be removed when baby will show signs of healing.

(Meconium peritonitis; Not the actual case. Photo courtesy: http://www.scielo.br)

Baby was brought back to the ICU immediately after the operation, stabilized. Antibiotics were given to cover for possible/ongoing infection. However, just as when we decided to begin feeding baby, he started having fever. Initially, I thought perhaps the antimicrobials were not doing their job so I had to switch to stronger kind. But the fever was unrelenting, while I kept on changing antibiotics every after five to seven days. I even referred the baby already to an Infectious Disease specialist to no avail. Also, from being able to breath on his own, baby now had to rely on continuous positive airway pressure. Platelets were also dropping to alarming levels. Blood was also coming out of the mouth and from the endotracheal tube. I suggested to the mother, (not to insult their financial capability but out of concern because I considered their sources of income and the baby’s mounting expenses), that I will refer the baby to social service so that they will be classified charity case, they agreed with great sigh of relief. Their emotions and mental status were already on roller coaster.

After almost one month from birth did I think that perhaps the baby might have contracted a tuberculosis from the mother during the time of pregnancy and this is the manifestation. It was as this point that I was bargaining with the almighty, asking Him to give the baby to his mother (as she is already high-risk with her old age). And in exchange I will not charge them professional fee as long as baby goes home alive. But exactly one month from birth, baby succumbed on Good Friday. I failed… but stood true to my bargain with the Lord, I did not ask the parents to pay me… It was painful. But on the other hand I thank the Lord for relieving the parents already of the pain they had been going through. They accepted baby’s death with ease. In fact before baby died, parents already signed a “do not resuscitate” order…

 
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Posted by on April 22, 2012 in Personal

 

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