28 August 2000

1. Sporadic Fever - Cause Unknown
2. Ask Your Doctor Questions
3. Applying for Disability Benefits
4. Pectus Excavatum - Dr. Warren, You Gave Bad Advice
5. Disclaimer

• Read Questions & Answers in Another Column

Sporadic Fever - Cause Unknown

9- month old girl, approx 22 lbs, high fevers every few days for one week (103.7, 104, etc.). Ther fever's come and go After giving tylenol once the fever disappears and returns a couple of days later. Blood count normal, initial urine screen normal but am waiting for culture results. If it's not an infection, than what could it be? It's very nerve racking.

Thank you.

-Mary

Dear Mary: Thank you for letting me know about the missing file. I have no idea what happened to it, so I reposted it.

Infection is the most common cause of fever in children. Sometimes the infection is caused by a virus which may not cause any findings on examination or any alteration in laboratory findings. If that is the case the illness should usually be resolved within a week, rarely longer. A normal urinalysis does not exclude the possibility of a urine infection, so you will need to know the results of the culture. Urinary tract infections may cause fever with no other obvious symptoms and there will not be any findings on physical examination to point toward that diagnosis or exclude it, so a urine culture must be done on any patient with prolonged or recurrent unexplained fever.

Other infectious causes of prolonged fever include Lyme disease, TB, and mononucleosis.

Fever may be a symptom of some other illnesses unrelated to infection. These include inflammatory diseases like inflammatory bowel disease, juvenile rheumatoid arthritis (JRA), and lupus. Fever may be seen as a symptom of childhood cancer. Since fever is such a common symptom, there isn't any reason for parents to worry about the rare non-infectious causes of fever until the fever has been prolonged for well over a week.

Sincerely,
Dr. Warren

Thanks

-MZ

Dear MZ: Regrettably, I cannot answer your question because I have no idea what procedure you are referring to when you talk about "cutting the membrane out." If your doctor has recommended a procedure, you should make sure you understand what the procedure is (including the actual name of the procedure in case you wish to do research about it such as send me a question), why it is recommended, and what the risks of the procedure are. It is your doctor's obligation to explain these things to you, but it is your responsibility to make sure he gives you this information before you consent to any procedure. Ideally, questions of this sort should be asked immediately when a doctor recommends a procedure, but it is understandable that sometimes patients are overwhelmed and forget to ask certain questions or ask and forget the answers. If that happens, don't be afraid to call your doctor and discuss it with him again. If possible, ask him to provide you with some literature about the procedure.

If you are able to find out just what it is your doctor has recommended and still have questions about it, I will be happy to answer your question if I can, but at this point, I don't know what procedure your question is about.

Sincerely,
Dr. Warren

I have a full time job, but the stress and strain seems to be worsening just getting up to go to work.

I also am receiving therapy twice a month, while seeing my physchrist once a month.

I am on Luvox, anxiety pills, blood pressure pills, ambien for insominia.

Is there a way I can find any means of disability or social security disability concerning mental health disease.

I am controlled at time on my medication, but the happiest I am is when I am at home, which I function ok.

What would I do or who would I contact about if I am eligible for any disabilities instead of my job.

Thanks for listening.

-SM

Dear SM: In order to get disability benefits you would have to apply to your state or county welfare agency or social security office depending on how disability is administered in your area. If you have personal disability insurance or disability insurance through work, you would have to contact the agency through which the policy was obtained. After applying for benefits you will need to follow the prescribed procedures to be certified for your disability. This may require documentation from your psychiatrist that you are unable to work or that working causes you undue hardship. You may be required by the disability agency to have an independent examination by one of their psychiatrists to certify your disability. If you are certified as disabled, you may be required to document that you are undergoing treatment. You may also be required to have your case periodically recertified in order to continue receiving benefits. Unless you can be certified as having a permanent disability (which would be unlikely for a psychiatric disorder), you would be expected to return to work as soon as you are able.

Sincerely,
Dr. Warren

-DC

Dear DC: You did not say what your personal experience with having pectus excavatum was. I am sorry you had difficulty as a result of it and that the medical profession did not serve you well.

I have not seen many cases of pectus excavatum as a pediatrician, but most were mild. Nonetheless, the parents were often quite alarmed by the appearance. By that I mean they thought the baby might be in some immediate danger or that there was something terribly wrong with their baby. Not to minimize the health problems associated with moderate to severe pectus excavatum or even the social and self image issues associated with less severe pectus, but most parents are relieved to find out that what they are seeing is not deadly.

Since Dr. Warren debuted on the Web 4 years ago, I have sought to provide comfort, advice, and information on routine pediatric (and even non-pediatric) matters, never claiming to be an expert on all these matters. Still, I realize the "MD" after my name carries a lot of weight and I'm careful to give the best advice I can. There is a real risk that I may misinterpret the focus of a question or that one of my readers may apply my advice to their situation in a way I never intended.

Since receiving your e-mail, I have reread my advice to Michelle regarding Pectus Excavatum. Just as I have with my own patients, I sought to reassure her that the mention of surgery did not imply that the child's condition was serious, but I see in so doing that I never answered her question about surgical procedures. And, as you've pointed out, some of my readers may take my "reassurances" that surgery is not necessary except for the most severe cases as advice that they should not consider surgical correction unless it is absolutely necessary. That is certainly not the case.

Pectus excavatum is a bona fide deformity which, in some cases, has a detrimental effect on heart and lung function. Even in the absence of physical infirmity due to pectus excavatum nobody should be expected to live with a deformity. It is not vanity to want to look normal. One's quality of life can suffer significantly from such a situation which is why insurance companies are beginning to recognize that the surgery should be covered without having to demonstrate cardiac or pulmonary complications.

While your criticism of my response to Michelle is justified, as a physician who has put himself out on the Web for free, solely for the purpose of doing good, I feel a bit hurt by your suggestion that I should "do a little research." While the improvement in the quality of your life is a testament to the benefits of surgery, it does not constitute proof that surgery is the answer for everyone, or even for the majority. The decision to have surgery when a life is not in the balance is a very personal one in which patients must weigh the benefits against the risks. Another person with your degree of pectus excavatum may decide against surgery.

In my defense, if I must defend myself, I should point out that there is not unanimity of opinion in the medical community, a factor which, unfortunately, makes it so much more difficult for patients to make informed decisions. In Behrman: Nelson Textbook of Pediatrics, Sixteenth Edition, Copyright © 2000, W. B. Saunders Company, it states,

Substantial pectus deformity rarely results in demonstrable restrictive pulmonary disease but usually has little or no functional effect.

Exercise testing has suggested an occasional link between pectus excavatum and exercise limitation. More commonly, exercise intolerance in children with pectus excavatum can be explained by limited habitual activity, related to the parents' fears.

In many children, the heart is shifted leftward, and in the rare patient, cardiac function may be adversely affected. Mitral valve prolapse (which may no longer be demonstrable by echocardiography after surgical correction of the pectus) and Wolff-Parkinson-White syndrome appear to be associated abnormalities. The clinical significance of these usually mild cardiac abnormalities is not clear.

Surgical correction of the pectus is not physiologically beneficial for most patients. However, improved exercise capability and normalization of lung perfusion scans and maximal voluntary ventilation have been reported. The functional importance of these findings is not clear. Some patients with severe deformities may seek repair for cosmetic or psychologic reasons.

Pectus excavatum, or funnel chest, is the most common of the congenital deformities of the chest wall, accounting for 90% of the defects and having an incidence of approximately 1 in 125 to 300 live births. It is characterized by a concave, posteriorly displaced sternum due to overgrowth of the costal cartilages.... The defect is a progressive deformity usually presenting at birth or early in life but can become manifest or exaggerated during growth surges. Pectus excavatum runs the gamut from a mild and scarcely noticeable deformity to those that are severe and symptomatic. In the most severe cases, posterior concavity of the sternum leads to a decreased anteroposterior diameter of the chest, loss of retrosternal space with displacement of the heart to the left, and reduction in volume of the left pleural space. Severe defects often have additional rotation or torsion of the sternum, most commonly to the right side. Pectus excavatum is more common in males than in females, with a male-to-female ratio of approximately 2:1 to 3:1. It is most commonly sporadic, although familial occurrence has been reported. Although this deformity can be associated with congenital heart disease, Marfan's syndrome, and other skeletal defects, it most commonly is an isolated finding.

... Patients most often present because of the cosmetic defect but are frequently found to have other symptoms. Although a mild defect may have few manifestations and minimal symptoms, symptoms are not always related to the degree of the sternal deformity. Older patients frequently note that they do not have the same respiratory reserve or exercise tolerance as their peers when they are engaged in strenuous physical activities. After surgical correction, patients frequently note an improvement in respiratory reserve, although preoperatively they may not have been aware of exercise limitations.

The most common reason for a patient with pectus excavatum to present to a physician is the significant cosmetic deformity. Fonkalsrud found that 64% of patients in his study complained of an unattractive physical appearance, whereas 50% of patients in Morshuis and associates' review paid conscious attention to the defect and took measures to hide it. Twenty-five percent of the patients believed that it altered their self-image. Even when no other impairment or abnormality can be identified, the emotional and psychological component can be significant and is an indication for operation. These patients may also have subtle and not so subtle exercise intolerance that routinely improves postoperatively. In addition to the cosmetic and psychological factors, other significant findings include impaired cardiopulmonary function and associated skeletal defects such as scoliosis.

Heart murmurs are present in approximately one third of patients. Systolic murmurs are most frequent and are probably caused by sternal compression on the pulmonary outflow tract. Congenital cardiac anomalies have been reported in 4% of patients, whereas mitral valve prolapse has been found in 8%.

The majority of patients with pectus excavatum report marked subjective improvement in exercise tolerance after repair. In addition, there are several studies that report objective evidence of cardiopulmonary dysfunction preoperatively and its subsequent improvement postoperatively....

Although some studies have failed to reveal hemodynamic changes in patients with pectus excavatum, this may be related to hemodynamic measurements being acquired while the patients were in the supine position. When patients with pectus deformities were studied while they were in the upright position, physical working capacity was significantly lower than when they were in the supine position. In this same study, patients who were subjected to exercise in the sitting position had a higher pulse rate during exercise at the same workload than when they were in the supine position. Oxygen uptake at rest is higher than predicted in patients with pectus excavatum and is even higher when sitting, while mixed venous oxygen saturation at rest or exercise was lower in the sitting position. Although these patients have a normal stroke volume at rest and exercise in the supine position, there is less of an increase in stroke volume with exercise in the sitting position when compared with normal controls. These changes while in the upright position are thought to be due to impairment in ventricular filling secondary to compression and displacement of the heart by the sternum.

...Pulmonary complaints are also common in patients with pectus deformities. In a recent review, 90% of patients younger than 6 years old who presented for surgical correction had functional pulmonary symptoms, including dyspnea or pulmonary infections. In Fonkalsrud and colleagues' review, over half of the patients reported mild to moderate exercise limitation and inability to keep up with peers in strenuous physical activity. In addition, recurrent pulmonary infections occurred in one third of patients, and asthmatic symptoms in 7%. Numerous studies have demonstrated restrictive alterations in chest wall mechanics and abnormalities in pulmonary function tests in patients with pectus excavatum including decreased vital capacity, decreased total lung capacity, decreased maximal ventilatory volume, and decreased maximal breathing capacity.

Improvement in pulmonary function postoperatively has been less clearly documented. In fact, several studies have documented worsening of the forced expiratory volume in 1 second, vital capacity, and total lung capacity postoperatively, suggesting further chest wall restriction after correction of the deformity. However, Cahill and colleagues from the University of Washington, in a prospective study of preoperative versus postoperative cardiorespiratory function, were able to show a significant improvement in maximal ventilatory volume, total progressive exercise time, and maximal oxygen consumption postoperatively. Most importantly, after surgical correction there was a consistent increase in maximal exercise capacity at every level of workload and a lower heart rate at every workload. Furthermore, in a Mayo Clinic study, the exercise duration and percentage of predicted work performed increased significantly after operation. Despite the potential for an increase in chest wall restriction postoperatively, there appears to be a functional and subjective improvement in exercise tolerance, dyspnea, and respiratory tract infections. As Morshuis and coworkers point out, factors other than changes in lung volumes may be responsible for the subjective physical improvement after surgery in most patients. Because of the satisfactory long-term results, surgical correction is justified in patients with pectus excavatum.

....Pectus excavatum itself has not been shown to be a contributing cause of death in those patients with the deformity, and therefore the long-term survival of these patients is not necessarily improved with surgical correction. However, because of the significant cosmetic and psychological improvement, subjective increase in exercise tolerance, documented changes in the cardiac and respiratory status, and prevention of the development of scoliosis after surgical intervention in these patients, surgical correction should be considered for all patients with a moderate to severe deformity. Because there may be spontaneous improvement in the pectus deformity in up to 40% of patients presenting in infancy, these patients should be observed through infancy and early childhood; spontaneous improvement becomes much less frequent after 3 years of age and usually there is no improvement after 6 years of age. Furthermore, long-term results are best in those patients operated on before age 6. Therefore, the optimal age for repair appears to be in early childhood (age 3 to 5 years). Successful repair with good results can also be obtained in the older patient, and adolescents and young adults who present with the deformity should likewise be considered for repair.

I do hope my response and the texts I've quoted provide the information you believe I should have provided to anyone seeking information about pectus excavatum.

Sincerely,
Dr. Warren

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