Fascinomas- Fascinating Medical Mysteries

by Clifton K Meador

  On admission to University Hospital, the workup by the medical resident revealed the patient to be short of breath and sitting upright in the bed, preferring to sit on the edge of the bed letting her feet hang freely. Blood pressure was 140/60 and her pulse rate was 92 per minute. She was breathing 24 breaths a minute and appeared frightened and acutely ill. Temperature was normal.

  Both legs were swollen to the knees with a large amount of edema (fluid in the tissues). The lungs were moist to listening. There was a loud murmur over the left side of the heart, suggesting mitral valve leakage. The working diagnosis was Congestive Heart Failure due to mitral valve rupture.

  The attending physician agreed with the resident and immediately moved Mrs. Sanderford to the cardiac catheterization lab. Both left and right sides of her heart were catheterized. Surprisingly all heart valves were functioning normally with no sign of rupture or leakage. The measured cardiac output of blood was very high at 8.0 liters per minute. Normal is around 3.5 liters per minute.

  The attending physician and medical resident were completely mystified to find such a high cardiac output in the face of congestive heart failure. In other words, the heart was failing to pump adequate blood to the body even though it was pumping huge quantities of blood. Something in the body was putting a demand on the heart for more blood than it could pump, i.e. “high output congestive heart failure.”

  The attending physician called for a cardiac consultation from Dr. Rand Frederiksen. Dr. Frederiksen listened to the story and sequence of events following back surgery. He listened to Mrs. Sanderford’s heart and heard the loud murmur. He then noted, as he suspected, that the murmur became louder and louder the lower in the abdomen he moved his stethoscope. The murmur, now a loud swishing sound, was loudest over the right groin, far away from the heart.

  Frederiksen turned to the medical resident, “There it is. The arterio-venous or AV fistula is causing the high output failure.”

  Arteriograms of the aorta and leg vessels showed a connection (fistula) between the right iliac artery and the right iliac vein (vessels moving blood in and out of the right leg). Surgery later that day closed the connection. Mrs. Sanderford recovered completely over the next 48 hours, with no more swelling of her legs or shortness of breath. There were no murmurs heard anywhere in her body.

  There are only a few causes for high output heart failure: hyperthyroidism (high thyroid hormone levels), anemia, beri beri from malnutrition and Vitamin B1 deficiency, psoriasis, Paget’s disease of Bone, certain forms of liver or kidney disease and arteriovenous fistulas.

  The right iliac AV fistula in Mrs. Sanderford’s case came from an accidental penetration by the surgeon with a sharp instrument through the disc space from her back into that artery and into the iliac vein, creating a connection. This complication occurs rarely in spine surgery, but it is well known and easily corrected. The surgeon erroneously thought the leg swelling was due to deep vein clotting with later blood clots moving into the lungs, causing the shortness of breath.

  The combination of knowing about the particular vascular complication of spine surgery combined with careful listening with the stethoscope lead to the solution of this mystery.

  *This case was shared by :

  Rand T. Frederiksen, M. D.

  Clinical Assistant Professor of Medicine (Retired)

  Vanderbilt University School of Medicine

  Chapter Twenty Five

  Labels That Stick *

  Josephine Rudolph was referred to Dr. Sidney Warrier with a diagnosis of hypoglycemia, which means recurring low blood sugar. Dr. Warrier was a well known and respected endocrinologist.

  There are some diagnoses that most physicians dread to hear, among them hypoglycemia, fibromyalgia and chronic fatigue syndrome. Hypoglycemia leads the list, and Mrs. Rudolph’s case is a good example why.

  Dr. Warrier had developed his own detective method for dealing with patients who carried a diagnosis of hypoglycemia. The year was 1974. He insisted that Mrs. Rudolph be admitted to the hospital where he could closely observe her over several days. Hospital admissions were easy to obtain in those days; it is too bad that managed care regulations no longer permit such periods of direct observation.

  Warrier’s plan was quite simple: Every time Mrs. Rudolph had one of her “hypoglycemia” symptoms, she was to call the nurse and have blood drawn for a blood sugar measurement. She was also to record in a bedside diary an exact description of her symptoms and the time of day. This plan would allow direct correlation of Mrs. Rudolph’s symptoms with the level of her blood sugar.

  When Mrs. Rudolph arrived in her hospital room, she was accompanied by her husband, Frederick Rudolph. Mr. Rudolph carried a large Styrofoam ice chest under each arm, returning with a total of five such chests. After Mrs. Rudolph gave him specific instructions where to place the chests along the wall of the room, she explained to the nurse.

  “You see, dear, I have this dreadful allergy to all domestic meats – pork (especially bacon), chicken and beef. I also cannot eat most fish, particularly if raised in ponds. So what do I have to do? I must bring my own meats. I have most of it here for your chef to cook – rabbit, buffalo, pheasant, and quail. My favorite is elk, but it is difficult to find.”

  Mr. Rudolph made no effort to make social contact and stood by saying nothing. He quietly left the room.

  Over the next five days, Mrs. Rudolph reported her symptoms several times a day. By the fifth day, there were 13 entries and 13 blood sugar measurements, occurring at all times of the day.

  Some of the recorded symptoms were dizziness, itching all over, extreme weakness, nausea, rapid heartbeat, lightheadedness, blurred vision and pelvic pain.

  Dr. Warrier had waited patiently day by day, seeing Mrs. Rudolph each day, listening carefully to her many symptoms but saying nothing except, “Well, let’s wait and see what the blood sugars show.” He withheld the blood sugar results until he had all of the numbers. On the fifth day, Dr. Warrier pulled a chair to the side of Mrs. Rudolph’s bed and placed the results sheet on his lap.

  “Mrs. Rudolph, I am glad to tell you that you do not have hypoglycemia. We have these measurements at the height of your symptoms and all the blood sugars are normal – normal being 80 to120. The lowest you showed was 85. I know you must be relieved to see that you do not have hypoglycemia.”

  Dr. Warrier tilted back in his chair and smiled faintly, visibly satisfied with his plan and presentation.

  Mrs. Rudolph paused for several moments, considering all she had just heard. “Well, Dr.Warrier,” she began, “I appreciate your thoroughness. But this morning I woke with this terrible itching and scratching feeling in my throat. What do you suggest I take to relieve it?”

  Warrier rose from his chair. “Oh, just get one of those over-the-counter gargles or mouthwashes at the drugstore. Use it several times a day, if you need to.”

  “I can’t do that. All those mouthwashes are full of glucose and that would trigger my hypoglycemia,” Mrs. Rudolph shot back, raising her nose in the air like she just won the national chess contest.

  Dr. Warrier nodded his head slowly, stunned at this woman’s denial of the facts and with the failure of his plan to persuade her. He said calmly, “I’ll send a full report to your family doctor. He’ll know what to do for you.” He then headed to the doctors’ lounge to tell his colleagues another hypoglycemia story.

  The case of Josephine Rudolph is an extreme, but not uncommon, example of patients who carry a diagnosis of hypoglycemia. True hypoglycemia is a very rare condition usually caused by tumors of the pancreas secreting insulin. (See Chapter 14. An Uncommon Cure for a case report.) However, despite the rarity, the false diagnosis or label of hypoglycemia is very common. Many people with the label are also insistent about having the condition. No amount of reassuring or measuring normal blood sugars will dissuade many of these patients or remove the diagnosis.

  Over many years somehow the diagnosis began to be applied widely and erroneously t
o people who had a myriad of vague symptoms even when the blood sugar levels were not low. The problem with any diagnosis, even when false, is that it is nearly impossible to remove the label. One often hears, “But Doctor, so-and-so told me I have hypoglycemia. He must know something that you don’t.”

  Dr. Michael Balint in the 1950s followed and observed a group of family doctors in Scotland and wrote a book called The Doctor, The Patient, and His Disease. Balint made the observation that for patients with chronic and unexplained symptoms any label is liable to be permanent, even when the named disease is not present. Diagnoses are difficult to remove.

  So it is with hypoglycemia, as well as fibromyalgia and chronic fatigue syndrome. Patients become very attached to their diagnoses, and no amount of evidence to the contrary fazes them.

  Dr. Warrier only thought he had a good plan to either substantiate or remove Mrs. Rudolph’s diagnosis of hypoglycemia. Obviously, he didn’t understand the permanent power of a label, be it true or false.

  *Case shared by:

  Alan Graber, M.D.

  Professor of Medicine (retired)

  Department of Medicine

  Vanderbilt University School of Medicine

  Chapter Twenty Six

  Learning to Speak

  the Language *

  Fred Harris, a second-year surgical resident, had just completed his workup on Lummy Jenkins. It was time to present the case findings to fellow residents and faculty surgeon Dr. Curt Tribble, Professor of Thoracic and Cardiovascular Surgery. The group was making rounds on their patients.

  When Harris mentioned the name Lummy Jenkins, Tribble interrupted. “So, Lummy is back again. I’m sorry to hear that. Not much left to repair on him. We have repaired or bypassed everything from his carotids to his aorta to his renal arteries. You name it, and Lummy has had it.”

  “Mr. Jenkins is not here this time for a vascular or cardiac problem,” Dr. Harris said, sounding somewhat excited. “He‘s got an enlarged breast. I think he has breast cancer.” The young doctor was obviously proud of his findings and eager for this complex case.

  As the group of residents headed into the room, Tribble asked if Harris had questioned Mr. Jenkins about all the drugs that can cause male breast enlargement. Harris had, and Jenkins denied taking any of the medications he named.

  Dr. Tribble paused in the hallway. “You did ask him how often he smoked marijuana, didn’t you?” Marijuana is a common cause of male breast enlargement (gynecomastia).

  Harris frowned. “Mr. Jenkins is an old man from a hollow back in the mountains,” he reasoned. “He doesn’t smoke marijuana.”

  Tribble led the group on into the room. “Hey, Lummy. How you been doing?” Jenkins stood by the bedside, smiled a toothless grin and shook Tribble’s hand, obviously glad to see him.

  “You got a wood shop at your place, right?” Tribble began his questions.

  “Yep.”

  “You have a machine shop too, right?”

  “Yep.”

  “You make a little shine for your own use, right?”

  “Yep, just for myself.”

  “You have a really good garden too, right?”

  “Yes, I do.”

  “Well, like most of the old folks around here, you grow a little of your own marijuana, just for yourself, right?”

  “Yep. How did you know that?”

  “Lummy, I have known you a long time and I know where you come from.”

  The two men laughed together.

  In the hallway, Tribble turned to Harris. “I suspect we now know the source of his breast enlargement — not by tests but by a simple, human conversation. I did several things in there with Lummy to get vital information. I let him know I knew his culture and some details of that. I also chose the first questions to make him comfortable and get positive answers. That let him know it was OK to say “yes.” Then I phrased the last question so he could answer honestly without worrying. I entered the world of Lummy Jenkins, and he let me into it.

  “Of course we will get an estrogen level just to be sure he does not have an estrogen secreting tumor somewhere,” Dr. Tribble continued. “Assuming those tests are negative, we can expect the breast to shrink on its own if he stops or reduces smoking marijuana. But at his age, I doubt if Lummy will do either. That’s up to him.”

  Editor’s Note:

  Dr. Tribble used several techniques to gain rapport. Entering the world of the patient is essential. Sometimes shifting voice tones to match the patient will help. When attempting to get sensitive or delicate information, it is necessary to give permission for an admission of a noxious habit. For example, when attempting to get an accurate laxative history in patients with low blood potassium levels and suspected laxative abuse, one might frame the question this way:

  “Mr./Ms. (name), we all use laxatives some time. I have patients who take only one bottle of milk of magnesia a day and I have some who take up to five bottles a day. How much do you take?”

  By leading up to the mention of marijuana, Tribble was permitting “yes” answers to private information. The careful questioning saved Lummy from an unnecessary mastectomy.

  *Case shared by:

  Curt Tribble, MD

  Professor of Surgery

  Chief, Division of Cardiothoracic Surgery

  Vice Chair, Department of Surgery

  Medical Director of Transplantation

  University of Mississippi

  Chapter Twenty Seven

  Sometimes the Answer to

  a Single Question Solves

  the Mystery *

  Gilford Matthe was a drug rep for a national pharmaceutical company. He and his wife lived in Gainesville, Florida. He called on gynecologists mostly, introducing new drug products for women and promoting the company’s line of drugs and hormones. He periodically visited Dr. Madeline Simmons, a gynecologist with a large referral practice. Dr. Simmons was a professor of gynecology at the regional medical center and medical school. Her specialty included infertility.

  On one of his visits, Gilford asked if Dr. Simmons would see him and his wife for a fertility workup. They had been married for several years and had been unable to get pregnant. They had wanted a child since they first married, but hadn’t had a fertility workup to find out why they had not yet conceived.

  When his wife Janice came in for the appointment, Dr. Simmons explained the fertility workup and asked if there were any questions. Janice shook her head “no,” so the workup began. After an exhaustive set of questions about her menstrual history and general medical history, Dr. Simmons began a complete physical and pelvic exam. Both were normal. Samples of the cervical mucous and lining of the uterus were sent for microscopic evaluation. These, too, were normal.

  The next phase of the workup required a variety of hormone measurements in Janice’s blood. Ovarian function is controlled by the pituitary gland in the brain, so Dr. Simmons measured these controlling hormones, as well as thyroid hormone levels. All showed normal levels. The cause for infertility was still missing.

  The next phase required a detailed study of the anatomy of the uterus and the fallopian tubes that led to the ovaries. These tubes carry the mother’s eggs down from the ovaries into the body of the uterus for union with the male sperm. A dye is injected into the uterus for x-ray examination to see if the dye is transmitted into the fallopian tubes. A blocked fallopian tube is one common cause for failure to conceive; the egg cannot get to the body of the uterus. This particular test must be done either late or very early in the menstrual cycle when there is little chance of interfering with a pregnancy. The test has a long name: hysterosalpingogram. Janice’s test was normal, ruling out tubal blockage as the cause of infertility.

  The final evaluation included additional uterine tissue sampling and daily temperatures to see if ovulation was occurring. Both of these were also normal. The results of the complete workup revealed Janice Matthe to be a potentially fertile woman.

  Dr. Simmons then turned
her attention to Gilford Matthe. His detailed medical history revealed no serious illnesses and no surgery. There had been no injuries to his testicles, and he had never had mumps that might have infected his testicles. His physical exam profiled a normal adult male with no signs of testosterone (male hormone) deficiency. A sperm count revealed healthy and normally mobile sperm.

  At this point, Dr. Simmons was stumped. She called the couple in to lay out her plans for the next, most complicated phase of a fertility program that involved timed copulation tied to daily temperature measurements. As she began the lengthy description, a question popped into her head.

  “How often do you have sexual intercourse?” she asked.

  “Oh, we haven’t had sex in several years,” Gilford answered. Janice said nothing.

  The answer floored Dr. Simmons — and still does.

  After several awkward moments waiting some further comment from either Gilford or Janice, Dr. Simmons finally said, “Well, then, I think we have our answer to your infertility.” With that, the couple rose and walked out of the exam room. She never saw them again.

  Dr. Simmons sat puzzling over the entire encounter and the unnecessary fertility workup. She asked herself why a well-informed man would seek an expensive and lengthy fertility workup in the face of a long period of sexual abstinence. She raised a multitude of unanswered questions that might explain the bizarre behavior: Was Janice frigid and Gilford thought this might lead Dr. Simmons to discover and address the problem? Was Gilford impotent and embarrassed to discuss it? Why didn’t the wife raise any question? Could there be some sexual deviancy that Gilford wanted the doctor to unearth and resolve?