Wolff-Parkinson-White syndrome in children with pectus excavatum

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Clinical and laboratory observations

survive to adulthood, the effect of these schedules on the testis will need to be studied.

REFERENCES 1. Bloom HJG, Wallace ENK, Henk JM. The treatment and prognosis of medulloblastoma in children. Am J Roentgenol 1969;105:43-62. 2. Bloom HJG. Tumours of the central nervous system. In: Voute PA, Barrett A, Bloom HJG, Lemerle J, Neidhart MK, eds. Cancer in childreni clinical management. New York: Springer-Verlag, 1986:197-222. 3. Ahmed SR, Shalet SM, Campbell RHA, Deakin DP. Primary gonadal damage following treatment of brain tumors in childhood. J PEDIATR 1983;103:562-5. 4. Whitehead E, Shalet SM, Morris Jones PH, Beardwell CG, Deakin DP. Gonadal function after combination chemotherapy for Hodgkin's disease in childhood. Arch Dis Child 1982;47:287-91. 5. Tanner JM. Growth at adolescence, 2nd ed. Oxford: Blackwell, 1962. 6. Shalet SM, Hann IA, Lendon M, Morris Jones PH, Beardwell CG. Testicular function after combination chemotherapy in childhood for acute lymphoblastie leukaemia. Arch Dis Child 1981;56:275-8. 7. Conte FA, Grumbach MM, Kaplan SL, Reiter EO. Correlation of luteinizing hormone-releasing factor-induced luteinizing hormone and follicle-stimulating hormone release from infancy to 19 years with the changing pattern of gonadotropin secretion in agonadal patients: relation to the restraint of puberty. J Clin Endocrinol Metab 1980;50:163-8.

The Journal of Pediatrics June 1988

8. Salbenblatt JA, Bender BG, Puck MH, Robinson A, Faiman C, Winter JSD. Pituitary-gonadal function in Klinefelter syndrome before and during puberty. Pediatr Res 1985;19:826. 9. Kulin HE, Santner SJ. The assessment of diminished testicular function in boys of pubertal age. Clin Endocrinol i986;25:283-92. 10. Lee IP, Dixon RL. Effects 0f procarbazine on spermatogenesis determined by velocity sedimentation cell separation technique and serial mating. J Pharmacol Exp Ther 1972;181:219-26. 11. Sieber SM, Correa P, Dalgard DW, Adamson RH. Carcinogenic and other adverse effects of procarbazine in nonhuman primates. Cancer Res 1978;38:2125-34. 12. Sherins R J, Olweny CLM, Ziegler JL. Gynecomastia and gonadal dysfunction in adolescent boys treated with combination chemotherapy for Hodgkin's disease. N Engl J Med i978;299:12-6. 13. Takihara H, Cosentino M J, Sakatoku J, Cockett ATK. Significance of testicular size measurement in urology, ii. Correlation of testicular size with testicular function. J Urol 1987;137:416-9. 14. Brown IH, Lee TJ, Eden OB, Bullimore JA, Savage DCL. Growth and endocrine function after treatment for medulloblastoma. Arch Dis Child 1983;58:722-7. 15. Hahn EW, Feingold SM, Simpson L, Batata M. Recovery from aspermia induced by low-dose radiation in seminoma patients. Cancer 1982;50:337-40. 16. Watson AR, Rance CP, Bain J. Long-term effects of cyclophosphamide on testicular function. Br Med J 1985; 291 : 1457-60.

Wolff-Parkinson-White syndrome in children with

pectus excavatum Joon M. Park, MD, a n d Alfred R. Farmer, MD From the DeDartment of Pediatrics, Texas Tech University Health Sciences Center, Lubbock

Cardiac dysrhythmias, particularly paroxysmal supraventricular tachycardia and atrial fibrillation, have occurred in patients with pectus excavatum? ,2 These have been attributed to cardiac impingement by a depressed sternum. 1,2 Resolution of PST was observed in some patients after surgery for pectus, but persistence was noted in other patients despite adequate surgical correction of the sternal deformity. 2,3 Although Wolff-Parkinson-White syndrome predisposes to dysrhythmias4:6 and has been demonstrated

Submitted for publication Oct. 5, 1987; accepted Jan. t 1, 1988. Reprint requests: Joon M. Park, MD, Pediatric Cardiology, Department of Pediatrics, Texas Tech University Health Sciences Center, 4th and Indiana, Lubbock, TX 79430.

in patients with pectus, 3'7' s the mechanism by which these dysrhythmias are brought about in patients with pectus is LGL MVP PST WPW

Lown-Ganong-Levine (syndrome) Mitral valve prolapse Paroxysmal supraventricular tachycardia Wolff-Parkinson-White (syndrome)

purely speculative? A prospective study was therefore undertaken to determine the incidence of W P W syndrome in pectus excavatum.

METHODS During the study period of 84 months, beginning in mid-1979, pectus excavatum was identified in 76 patients

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Clinical and laboratory observations

927

at the pediatric services of Texas Tech University Health Sciences Center. Because of our mail requests for the referral of these patients, 59 were referred to us by pediatric practitioners after informed consent was obtained from their parents. Seventeen were recruited from the pediatric cardiac service, where the pectus was recognized during cardiac evaluation for a variety of cardiac complaints; five of these were referred to us primarily because of PST. Patients were evaluated for W P W syndrome with standard electrocardiography. Also included for each patient were physical examination and M-mode two-dimensional and Doppler echocardiography. The severity of the pectus deformity was determined by measuring the depression of the sternum from the surface of the anterior chest wall to the deepest point of the pectus, and was considered to be mild for sternal depression less than 2.0 cm and severe if 2.1 cm or more. The electrocardiographic criteria of W P W syndrome were a short PR interval for age and a QRS complex with initial slurring (the "delta wave"). 9 Rosenbaum's classification9 was used (type A, upright delta wave and QRS complex in lead VL; type B, inverted delta wave and QRS complex in lead V j). The M-mode and two-dimensional echocardiograms were obtained with a Hoffrel model 201A ultrasonoscope, (Hoffrel Instruments, Inc., South Norwalk, Conn.) interfaced to a Honeywell model LS-6AHS strip chart recorder (Honeywell Test Instruments, Denver, Co.) or an IREX system (Technicare Ultrasound, Ramsey, N.J.) using a 5.0 mHz transducer. The examination was performed at the standard transducer location and at interspaces above and below to ensure optimal recording of the mitral valve from annulus to free edge. Particular care was taken to avoid recording with inferior transducer angulation. Mitral valve prolapse was considered present when the mitral valve leaflets exhibited midsystolic, late systolic, or pansystolic posterior motion and returned to a normal position at end systole?~

Table. Comparisons of the incidences of W P W syndrome among the patients with and without pcctus and cardiac complaints

RESULTS

DISCUSSION

The mean age of the 76 patients was 5.0 years, with a range from 1 month to 18 years. Fifty-eight were male. Three (4%) had electrocardiographic evidence for W P W syndrome, including type A in 1 and type B in 2. Two other patients possibly had WPW, and three others had Lown"Ganong-Levine syndrome (short PR interval without a delta wave on electrocardiogram in the presence of PST).5 Of the five patients with PST, there were three With LGL syndrome, one with WPW, and one with possible WPW. The Table summarizes the incidence of W P W syndrome among the patients with and without pectus and cardiac complaints. The incidence of W P W syndrome was 4% in the pectus patients and was 2.8% when no cardiac problem was suspected. In contrast, patients in the cardiac clinic

No. of patients

Patients with pectus Patients without pectus Patients with cardiac complaints Pectus patients without cardiac complaints

Total

WPW syndrome

p

76 1871 1888

3 8 9

<0.001

71

2

<0.01

had a 0.5% (9/1888) incidence of W P W syndrome, and this incidence was 0.4% when pectus patients were excluded. Therefore the incidence of W P W syndrome in the pectus group was significantly higher than in the general cardiac clinic population (Table). Patients with W P W had a 27% (3/11) incidence of pectus. Of the 76 patients, the degree of the pectus deformity was mild in 53 and severe in 23. Although we demonstrated both W P W and PST in the patients with mild pectus, neither of these was demonstrated in any of the patients with severe pectus. Mitral valve prolapse is reportedly common in patients with pectus 7 and W P W 4,1~, with an incidence of 18% and 10%, respectively. The association of WPW, MVP, and pectus has been reported. 7'8'1z We demonstrated a 25% incidence of MVP in the patients with pectus, and one of the three patients with W P W had MVP. Of the 19 patients with MVP, 15 patients had auscultatory findings of nonejection click, late systolic murmur, or apical pansystotic murmur related to mitral regurgitation. Unavoidable transducer angulation might have given a false impression of MVP, particularly in patients with severe pectus. Five of the six patients with severe pectus and MVP had auscultatory findings of MVP.

The W P W syndrome is an uncommon cardiac disorder, but often it predisposes the patient to PST? The incidence in the unselected childhood population is variously reported between 0.04% and 0.31%. 6 A higher incidence has been reported in two series of children from heart clinics? ,6 Selig3 reported that in 100 patients with pectus who were treated on a surgical service, W P W syndrome was recognized in two patients, both of whom had PST. Whether an electrocardiographic evaluation was done in each patient, however, is not known. It appeared that the incidence was higher in these patients than would be expected in otherwise normal individuals. However, this report may have underestimated the true incidence unless an electrocardiogram was routinely recorded, 6 because patients with W P W

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Clinical and laboratory observations

may be completely asymptomatic or have a normal electrocardiogram? Our study was designed to evaluate a large group of patients with pectus to determine the incidence of WPW syndrome. We demonstrated a 4% incidence of this syndrome in the patients with pectus and a 2.8% incidence when no cardiac problem was suspected. Of particular interest was the observation that patients with WPW syndrome had a 27% incidence of pectus. If the cause of PST were cardiac impingement by pectus, l,s one would expect the incidence of PST to be greater in the patients with a more severe pectus deformity, but none of our patients with severe pectus had evidence of cardiac dysrhythmia or WPW syndrome. Our findings indicate that WPW syndrome is the most important underlying mechanism for PST in patients with pectus. The explanation for our findings is not apparent. Because of the concept that a systemic connective tissue defect or abnormal embryologic development may be an underlying disorder for both MVP and pectus, 7,12,~3 our findings of the high incidences of MVP and WPW syndrome in patients with pectus may suggest that WPW syndrome is pathologically related to pectus. It is also possible that MVP may be present as the result of the altered sequence of ventricular activation, rather than as the result of a structural abnormality? The WPW syndrome tends to be a benign disorder, but some patients may develop a life-threatening dysrhythmia, for which an electrophysiologic study and drug therapy or surgical interruption of the reentry circuit may be indicated. Pectus excavatum is easily recognizable and should alert the physician to consider the possibility of WPW syndrome as an underlying condition when a cardiac dysrhythmia is present. It is currently our policy to include an electrocardiographic recording when a patient with pectus is evaluated.

The Journal of Pediatrics June 1988

We thank the faculties and house staffs of the Department of Pediatrics for referring their patients for this study. REFERENCES

1. Majid PA, Zienkociez BS, Roos JP. Pectus excavatum and cardiac dysfunction. Thorax 1979;34:74-8. 2. Wachtel FW, Raviteh MM, Grishman A. The relation of pectus excavatum to heart disease. Am Heart J 1956;52:12136.

3. Seling A. Trichterbrust und Wolff-Parkinson-White Syndrom. Med Welt 1969;21:1255-9. 4. Gallagher J J, Gilbert M, Svenson RH, Scaly WC, Kasell J, Wallace AG. Wolff-Parkinson-White syndrome. Circulation 1975;51:767-85. 5. Gillette PC, Garson A Jr. Pediatric cardiac dysrhythmias. New York: Grune & Stratton, 1981:162. 6. Roberts NK, Gelband H. Cardiac arrhythmias in the neonate infant and child. East Norwalk, Conn.: Appleton-CenturyCrofts, 1977:233. 7. Udoshi M, Shah A, Fisher V, Dolgen M. Incidence of mitral valve prolapse in subjects with thoracic skeletal abnormalities: a prospective study. Am Heart J 1979;97:303-11. 8. Drake CE, Hodsden JE, Sridharan MR, Flowers NC. Evaluation of the association of mitral valve prolapse in patients with Wolff-Parkinson-Whitetype ECG and its relationship to the ventricular activation pattern. Am Heart J 1985;109:836. 9. Garson A Jr. The electrocardiogram in infants and children: a systematic approach. Philadelphia: Lea & Febiger, 1983: 138. 10. Williams RG, Tucker CR. Echocardiographic diagnosis of congenital heart disease. Boston: Little, Brown, 1977:230. 11. Devereux RB, Perloff JK, Reiehek N, Josephson ME. Mitral valve prolapse. Circulation 1976;54:3-14. 12. Devereux RB, Kramer-Fox R, Brown WT, Shear MK, et al. Relation between clinical features of the mitral prolapse syndrome and echocardiographically documented mitral valve prolapse. J Am Coil Cardiol 1986;8:763-72. 13. Chan FL, Chen WWC, Wong PHC, Chow JSF. Skeletal abnormalities in mitral valve prolapse. Clin Radiol 1983;34:207-13.

Multiple telangiectases of the colon in childhood Harry A. C y n a m o n , MD, David E. Milov, MD, a n d Joel M. Andres, MD From the Department of Pediatrics (Gastroenterology), University of Florida College of Medicine, Gainesville

Submitted for publication Dec. 3, 1987; accepted Jan. 6, 1988. Reprint requests: Harry A. Cynamon, MD, Department of Pediatrics (Gastroenterology), University of Florida College of Medicine, Box J-296 JHMHC, Gainesville, FL 32610.

We have identified four children with hereditary hemorrhagic telangiectasia in whom bleeding of the large intestine developed in the first decade of life before the onset of skin or nasal lesions. The presenting manifestation of this