I first met Baby Boy Hoff about eight years ago.
But meeting is not really the right word for our first encounter.
I couldn’t find him in the computer, my secretary had said as she handed me the consultation request slip. In the corner that usually bore an imprint of the patient’s name and room number, I saw Baby Boy Hoff handwritten in large, clear letters. He must be over in the newborn intensive care unit, she added. Probably born just a couple of hours ago and no time to make up an id card yet.
I walked over to the newborn intensive care unit and asked Margo Dobbins, the charge nurse, where to find Baby Boy Hoff. As an otolaryngologist, a specialist in disorders of the ear, nose, and throat, I often visited Margo’s unit to examine newborns.
No one here with that name, Norm, she replied. Let me look it up in the computer. . . . The only ‘Hoff’ it shows is Eleanor Hoff up in ob, admitted yesterday.
I called Tom Theodore, the icu’s director. A stereotypically compulsive physician, he knew almost everything about every sick newborn in the hospital--whether the child was under his care or not.
Tom, where is the Hoff baby you asked me to see? He seems to have gotten lost somewhere in the system.
Oh, he’s not lost--he’s just not born yet. But I want you to have a look. His mother’s name is Eleanor Hoff, and she’s going to have a C-section tomorrow. The baby’s in trouble. He’s got a large cystic hygroma and could die during delivery. Debbie Gray has the sonogram on videotape for you.
A cystic hygroma is a tangled mass of lymph vessels most often seen in young children. Although it is not cancerous, the abnormal growth can encroach on otherwise normal organs.
Downstairs in the radiology department, Debbie pointed out the hygroma in the sonogram image with a chewed ballpoint pen. This big black mass on the side of his neck is the cystic hygroma. I’ve been following it over the past few weeks, and it’s gotten as big as an orange. Tom is worried that a lot of it might be diving inside the neck and wrapping around the trachea like a tourniquet. If it is, it could choke him as soon as he’s born.
Debbie Gray was probably the best ultrasound doctor in the state. Where my untrained eye saw smudged ink blots in sonogram images, Debbie saw fetuses, livers, lungs, and malformations. Sometimes it was difficult to believe that sonographers could accurately interpret what looked like vague shadows. But Debbie had said she was absolutely certain about what she’d seen.
The lymph-filled hygroma wasn’t doing any harm right now, because the fetus was getting oxygen through the umbilical cord. As long as he remained in the womb, he didn’t need to breathe, but he couldn’t stay put much longer.
Lymph is the forgotten second fluid that circulates in the body. Capillaries--the smallest and most fragile of blood vessels--normally leak water, proteins, and salts into the spaces between the cells in all tissues. If this ooze, called lymph, weren’t returned to the blood, the cells would wind up in a sea of leaked fluid, and the blood, depleted of liquid, would congeal into a thick sludge. To prevent that, lymph vessels-- short segments of tiny, flexible pipe--hook together, forming a long one- way sluice to carry lymph back into the blood. But sometimes the nascent vessels in an embryo don’t connect with the main pipeline and end instead in blind alleys. These truncated vessels fill with lymph, then dilate and twist until they resemble a tiny cluster of balloons.
A cystic hygroma growing from the side of the neck or face can form a disfiguring bulging mass. But it does its real dirty work when it infiltrates deep into the body. If the cystic hygroma had burrowed into Baby Boy Hoff’s neck, it might be choking off his airway or encircling his voice box with its fluid-filled fingers. Or worse, it might have invaded one of these structures, blocking it like some diabolical cork.
The first tip-off I got that something was seriously wrong, Debbie said, was the volume of amniotic fluid in Eleanor’s uterus. When I did a sonogram a few weeks ago, it showed much more than two quarts, which is above normal.
Although excess amniotic fluid is not in itself harmful, it can be a symptom of muscular dystrophy, an undeveloped brain, or a growth in the chest or neck. Because these conditions all affect the fetus’s ability to swallow, they disrupt the normal recycling of amniotic fluid, causing it to build up in the amniotic sac.
Amniotic fluid does more than provide a warm, protective bath in which fetuses can move their arms and legs in a sort of prebirth workout. It also promotes the normal development of many organs by constantly circulating through the fetus. Most amniotic fluid originates as urine in the fetal kidneys. From there, it flows through the urinary tract and out into the amniotic sac, where fluids can be exchanged between mother and fetus across the amniotic membrane. But some amniotic fluid is continually reabsorbed by the fetus. By gulping the amniotic fluid surrounding him, Baby Boy Hoff could practice the swallowing he would need to nurse. In addition, amniotic fluid flooded his lungs, keeping them expanded and elastic enough to receive his first breath. Our worry was that if the hygroma was big enough to prevent him from swallowing, it might also prevent him from breathing.
I stopped by Eleanor Hoff’s room in the obstetrics ward to introduce myself. Another doctor, she said, stressing the first word with a mixture of surprise and exasperation. Are you mine or Andrew’s? We’ve decided on the name Andrew, by the way. Another doctor means another problem, right?
You know, I tried to do all the right things during my pregnancy. I didn’t drink or smoke, and I exercised regularly. I bought bottles of those expensive prenatal vitamins and consumed pounds of vegetables I didn’t like.
I knew all of this from Eleanor’s chart. Some things are nobody’s fault--only bad luck.
This is my first baby, she added, and I thought they called it ‘expecting’ because I was supposed to expect good things. I’ve seen the sonograms. The best I can expect for Andrew is that he makes it to the ICU.
Eleanor, I’m sure you know that we may have some trouble tomorrow, I began. The only information I have about Andrew is from the sonograms, and it’s pretty skimpy. We won’t be able to figure out his condition until you deliver him, and by then there won’t be time to do much figuring.
I told her what our options were. If Andrew can’t breathe, I’ll try to intubate him. This will involve guiding a slender, slightly curved tube down his throat and into the opening of the windpipe beneath the vocal cords. But that might be impossible if the hygroma has grown over these anatomical landmarks.
If you can’t intubate him, then you’ll have to put a hole in his neck, right? Eleanor had obviously been interrogating her doctors and nurses before I got there.
Yes, a tracheostomy would be our only other hope.
In an adult, piercing the trachea and inserting a plastic breathing tube is a straightforward procedure. The adult trachea is roughly the size of a garden hose, with cartilage so hardened that it is easy to find by probing within the soft, supple neck tissue. But Andrew’s immature trachea would be less than a quarter-inch wide. And it would be so soft and spongy that it could easily evade my probing finger. For that reason, newborns are usually intubated before undergoing a tracheostomy. The rigidity of the inserted tube makes the immature trachea easy to find. More important, it provides an airway so that the baby can breathe comfortably during the operation.
But if Andrew needed a tracheostomy, he wouldn’t be breathing at all, and he wouldn’t have a breathing tube in place. The operation would be a dreaded crash procedure--done with no time to spare and lots of shouting. Andrew would be getting bluer. His heart would race--perhaps reaching 200 beats a minute--in a desperate effort to circulate as much blood as possible to his oxygen-starved tissues. But the blood would be filled with carbon dioxide because his lungs couldn’t expel it through the blocked airway. Too much carbon dioxide makes blood acidic, which so poisons the heart muscle that it may fail to pump. Rule number one of CPR is that when there is too much acid in the heart muscle, the heart cannot be resuscitated.
Even if we cut into Andrew’s airway in time, the incision would probably slice through the hygroma. If the cysts around the incision became infected, the infection would spread to the cysts that were buried deeply in his neck and, possibly, down in his chest. We would have given Andrew an airway, only to have him succumb to the poisonous pus-filled abscesses locked inside him. No doubt about it, a tracheostomy was not a good option. Better to just intubate him.
I’m scared for him, Eleanor said and paused. Maybe this is all a big mistake. After all, doesn’t the sonogram sometimes get even the sex of the baby wrong? Maybe we’re getting ourselves excited over nothing.
I desperately wanted to believe that nothing was wrong with Eleanor’s baby. But Debbie Gray had said there was no doubt.
The next day Eleanor was wheeled in for a cesarean section. The operating room was crowded, so I stood back, beside an incubator set up for Andrew. The obstetrician cut into the thick, bloody uterus, and as the amniotic fluid gushed out, all eyes focused on the spot where Andrew and the cystic hygroma would appear.
As for me, I didn’t care what Andrew’s neck looked like. It didn’t matter if Andrew had the biggest cystic hygroma known to medical science as long as he could breathe. I had made up my mind that if I could hear a cry, Andrew’s airway was probably clear. So I had no problem standing off to the side, where I couldn’t see the operating table. I could still hear, and hearing was all.
I finally did hear him. it’s not a very strong cry, said one of the OB nurses. But the cry was loud and long enough to permit a few seconds of relief and hope that the next cry would be stronger. But no more cries came. He can’t even grunt, she added, suctioning his mouth and nostrils with a blue rubber bulb. She immediately put Andrew down faceup and in front of me. His arms and legs were flailing, and his chest was heaving. But no air was moving.
From what I could see, Andrew’s hygroma wasn’t very big. His cheek looked a little chubbier than normal, and he had more of a football player’s bull neck than a protruding lump. Yet Andrew was getting bluer. More cystic hygroma must be diving inside Andrew than pushing out.
Doctors train hard for a moment like this, spending hours, sometimes days, learning a skill that occupies but a few seconds. Intubation is such a skill. CPR classes provide mannequins with plastic larynxes for medical students to intubate, but a plastic larynx doesn’t seem real, and the dummies never turn blue. Still, the drills allowed us to practice with the laryngoscope. The instrument, which looks like a bent letter opener with a miniature headlight on the blade’s tip, lights the surgeon’s path into the airway. But the most valuable experience came when a fledgling doctor would intubate a patient for surgery under the watchful eye of a veteran anesthesiologist.
I had done all that. Even more. I couldn’t be any more practiced. Except that in 20 years of patient care I hadn’t done this procedure under these conditions before. And Andrew needed help.
One Thanksgiving eve several years ago, while still a resident in training, I had rushed to operate on a young man’s mangled neck. He had been drunk when he ran his snowmobile into a strand of barbed wire strung three feet above the ground, probably by someone tired of trespassers. The dull wire guillotine had jumbled his neck tissue into sausagelike chunks. I remember marveling at how the emergency technician at the scene had managed to find the young man’s severed windpipe and insert a breathing tube.
Easy, he had said with a shrug. I just stuck the tube where the bubbles were. Of course. The only possible source of the bloody bubbles had to be the airway. Lessons like that seem to stick with you.
Andrew lay faceup in front of me, his face darkening. I maneuvered the lighted blade of the laryngoscope into his mouth, gently sliding it past his tongue. There, looking like tiny translucent grapes, lay a bunch of yellow cysts hopelessly hiding the airway. Somehow I had to find the one sure route through them. I recalled the snowmobiler--and pushed down sharply on Andrew’s chest with the flat of my right hand. His soft newborn rib cage gave way easily, like a small beach ball. A few tiny bubbles of saliva gently spat at me from between several of the cystic masses. This was probably the last bit of air left in Andrew’s lungs. I insinuated the laryngoscope’s blade through the area where the bubbles had been, and the tip suddenly lit up the inverted V of the vocal cords, the entrance to the trachea. I pushed the tube home. Andrew pinked up as air started moving easily in and out of his lungs.
Over the next few months, Andrew needed several operations to remove the bulk of the hygroma from his face and neck. The remaining cysts, which were too deep in his throat to remove, gradually dissolved. Today Andrew lives with his family on a farm in southern Minnesota and breathes the country air just like everybody else. After a while, Eleanor decided to get pregnant again, and Andrew now has a sister.
Medicine, like anything else, is full of unexpected connections, and sometimes the connections are crucial. The snowmobiler and Andrew will never know they were connected. I will never forget.
