Sudden infant death syndrome and barometric pressure

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B r i e f cfinical and laboratory observations

immediate gain in hearing. As with any medical or surgical intervention, there are occasional complications from either the operative procedure or from the anatomic or physiologic effects of the tubes themselves. These complications have been previously summarized. 2 An infrequent complication is the development of a cholesteatoma at the site of insertion. These iatrogenic implantation cholesteatomas have an approximate incidence of 0.09%, based on a questionnaire answered by 320 practicing otolaryngologists? We report an example of a documented iatrogenic implantation cholesteatoma occurring in an unusual location (anterosuperior quadrant) and to stress the need for periodic, systematic otologic examinations following tubal extrusion. Abbreviation used OME: otitis media with effusion CASE REPORT

A 2~A-year-oldboy had a bilateral myringotomy with aspiration of thick mucoid secretions and insertion of collar-button ventilation tubes because of persistent (four months) bilateral OME. The procedure was performed under an operating microscope in the anterosuperior quadrant of the tympanic membrane. During the 15 months that the tubes were functioning, there were no episodes of otorrhea, after which time the tubes spontaneously extruded. Two months later, during a routine annual pediatric physical examination, a small cholesteatoma was visualized behind the anterosuperior quadrant of the left tympanic membrane at the site of the previously inserted tympanostomy tube. The cholesteatoma was completely removed through an endomeatal approach. A small tympanic membrane defect which resulted from the surgery was closed employing a Gelfoam patch. One month later the tympanic membrane was intact, and the audiogram and tympanogram were normal. No persistence or recurrence of the cholesteatoma has been noted after an llmonth follow-up period.

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DISCUSSION A cholesteatoma is an actively enlarging cystic structure lined by keratinizing stratified squamous epithelium containing a soft mass of concentric layers of cast-off epithelial cells which often undergo diffuse inflammatory changes. The etiology and classification of middle ear cholesteatomas have recently been reviewed? A middle ear cholesteatoma (keratoma) is a dangerous, locally invasive, nonneoplastic growth which must be completely excised to preserve middle ear function and to prevent possible intracranial sequelae. After the reintroduction of ventilation tubes in 1954, it was feared by some that the tubes would promote the development of a cholesteatoma. However, with the passage of time it seems clear that patent tympanostomy tubes, rather than serving as a stimulus for the development of a cholesteatoma, in fact probably prevent the formation of secondary acquired cholesteatomas by insuring long-term continuous middle ear ventilation. Implantation cholesteatomas are a real, though fortunate.ly rare, complication of ventilation tubes. Careful otologic follow-up would appear to be a reasonable suggestion for all patients who have had previous extrusion of the ventilation implants. Only in this way will a persistent attic retraction pocket or implantation cholesteatoma be detected. REFERENCES 1. Paradise JL: On tympanostomy tubes, Pediatrics 60:86, 1977. 2. Hughes LA, Warder FR, and Hudson WR: Complications of tympanostomy tubes Arch Otolaryngol 100:151, 1974. 3. Armstrong BW: What your colleagues think of tympanostomy tubes, Laryngoscope 78:1303, 1968. 4. Bluestone CD, Cantekin ELJ, Berry Q, and Stool SE: Function of the eustachian tube related to surgical' management of acquired aural cholesteatoma in children. Laryngoscope 88:1155, 1978.

Sudden infant death syndrome and barometric pressure Susan Heaney, B.S., and Matilda S. Mclntire, M.D.,* Omaha, Neb.

EPIDEMIOLOGIC STUDIES have been done correlating the incidence of sudden infant death syndrome with age, sex, and race of the infants, with seasonal variation, and with meteorologic factors. The purpose of this study was to investigate the relationship between daily barometric pressure and the incidence of SIDS. A literature review From the Creighton University School of Medicine. *Reprint address: 601 N. 30th St., Omaha, NE 68131.

0022-3476/79/300433 + 03500.30/0 9 1979 The C. V. Mosby Co.

revealed only one previous investigation by Fedrick 1 concerning monthly mean barometric pressure and the incidence of SIDS. METHODS

AND MATERIALS

The dates of infant deaths were provided by the Nebraska Chapter of the National SIDS Foundation in the Greater Omaha Metropolitan area from 1973 through 1976. Incidence rates were ascertained from statistics for

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Brief clinical and laboratory observations

The Journal of Pediatrics March 1979

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Daily Lowest Barometric Pressure Readings (t --0,0365) Figure. Lowest barometric pressure on the day of sudden death and distribution of lowest pressure for all days of the study. live-births in Douglas County over the same time period. The barometric pressure reading for each hour of the day, measured at the Omaha weather station, was graphed for the day of each infant death and then used to determine the lowest barometric pressure on the day of death, and to ascertain if the pressure was rising, falling, or holding steady. The lowest barometric pressure reading on the day of death was used to evaluate a relationship between barometric pressure and the incidence of SIDS. The exact time of death of each infant was not known so the barometric pressure at that time could not be determined. As a control measure the lowest barometric pressure recorded on each day of the four-year study was determined. The values for the lowest barometric pressures determined for each of the days on which a SIDS occurred and for the control days were distributed over five arbitrary groupings of pressure measurements. A comparison was made between the distribution of lowest barometric pressure readings in the five groupings for all the days of the study, and the distribution of lowest pressure readings over the same groups for days on which the sudden deaths occurred. RESULTS The Figure shows the distribution of sudden deaths in five barometric pressure groups according to the lowest barometric pressure recorded on the day of each death, al~d the distribution of all days in the four-year study period in the same pressure groupings according to the lowest pressure reading on each day. No statistical differ-

ence was found between the distribution of lowest barometric pressure readings for all the days of the four-year study and the distribution of lowest barometric pressure readings for the days on which a sudden death occurred. This lack of statistical significance was determined by a two-tailed Student test. DISCUSSION The incidence of 2.55 per 1,000 live-births for the four-year period is similar to that determined in Douglas County over the years 1964 through 1969, 2.5 per 1,000 live-births.~ The seasonal distribution found, with 77% of the deaths occurring during the colder half of the year, has been noted frequently in the literature? The winter peak in deaths has been correlated with a possible role of viral infection in the ultimate mechanism of death and also with influenza epidemics.' Approximately half of infants dying of SIDS have had an upper respiratory infection. Viruses of upper respiratory infections may be more readily transmitted from person to person when cold weather forces people to stay indoors, especially when they live in crowded conditions. Our study has failed to show a significant correlation between SIDS and low barometric pressure.

REFERENCES 1. Fedrick J: Sudden unexpected death in infants in the Oxford record linkage area. An analysis with respect to time and place, Br J Prev Soc Med 27:217, 1973.

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Brief clinical and laboratory observations

2. Armitage J, and Hoffman BY: Sudden unexplained death in infancy, Nebr Med J 57:213, 1972. 3. Valdes-Dapena MA: Sudden and unexpected death in infancy: A review of the world literature, 1954-66, Pediatrics 39:123, 1967.

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Froggatt P, et al: Sudden death in babies: Epidemiology, Am J Cardiol 22:457, 1968.

Hypergonadotropic-hypogonadism in the Prader-Labhart- Willi syndrome L. E. Seyler, Jr., M.D.,* K. Arulanantham, M.D., and C. F. O'Connor, M.D., Newington and Farmington, Conn.

Hypogonadism is an almost invariable feature o f the Prader-Labhart-Willi syndrome 1 and is more severe and persistent in males than females. ~, ~ Although the exact cause for the hypogonadism in this syndrome is not known, previous studies have presented evidence for a hypothalamic deficiency.'-'. ~. ~ We report here studies of a 16,year-old boy with P L W syndrome whose responses to L R H and h C G show that the hypogonadism in this syndrome can be due to gonadal deficiency rather than to an incapacity to initiate or sustain pubertal hypothalamic maturation.

CASE REPORT The subject is a white boy born by cesarean section to a 37-year-old primigravida. Hypotonia, weak cry, flaccid muscles, sluggish Moro reflex, and fiat scrotum without palpable testes were noted at birth. In childhood, delayed motor development, internal strabismus, hypotonia, obesity, mental retardation, impaired articulation, and short, tapered fingers confirmed the diagnosis of PLW syndrome. At age 8, spermatic cord and blood vessels were traced to an extremely hypotrophic testicle, ]/2 • 1~/: cm, in the inguinal canal. Biopsy of this and a more proximal epididymal-like bulge revealed only a few ductal structures, resembling rete testes, without any seminiferous tubules or Leydig cells recognized. When studied at 16 years of age, he was 138 cm tall and weighed 45.5 kg. The penis was small (4 cm) and scrotum contained only cordlike material ending in soft, nontender, disclike masses, % cm in diameter on the fight and l/~ cm in

From the Department of Medicine, Veterans Administration Medical Center, and the Department of Pediatrics, Newington Children's Hospital, and the Departments of Medicine and Pediatrics, University of Connecticut. Supported by the Medical Research Service of the Veterans Administration. *Reprint address: Bldg. 5, VeteransAdministration Medical Center, Newington, CT 06111.

diameter on the left. There was no evidence of sexual development. Bone age was 13 years and T,, T~RU, and TSH were normal. Following 0.15 units insulin/kg iv, growth hormone concentration rose from < 1 to 9 ng/ml and cortisol from 12 to 26 p,g/dl. The patients' parents gave informed consent for the subsequent studies, which had been approved by the University of Connecticut Human Experimentation Committee. Abbreviations used PLW: Prader-Labhart-Willi LRH: luteinizing hormone-releasing hormone hCG: human chorionic gonadotropin TSH: thyroid-stimulating hormone LH: luteinizing hormone FSH: follicle-stimulating hormone After five days of treatment with hCG, 5,000 units/day, serum testosterone concentration was 47 ng/dl (normal adult male 300 to 1,200 ng/dl) and after six weeks of treatment with 3,000 units three times each week, serum testosterone was 58 ng/dl. Following these treatments, no sexual development was evident and no change in the intrascrotal tissues occurred. Response of LH and FSH (measured by radioimmunoassay using National Pituitary Agency materials) to iv LRH, 100/Lg (Ayerst Labs), was measured by comparing 10 plasma samples drawn in 90 minutes before injection with 8 plasma samples drawn at intervals after injection (Figure). Prior to LRH injection, plasma LH was in a normal adult range and FSH was in a hypergonadotrophic hypogonadal range. The maximal LH increment after LRH (119 ng/ml) was within the range measured in 21 men (40 to 1,180 ng/ml) and the maximal FSH increment (624 ng/ml) was greater than responses measured in 19 men (18 to 300 ng/ml). DISCUSSION Hypogonadism is an invariable feature of the PLW syndrome in males and cryptochidism is almost always present. '~ Late and incomplete sexual development does