Defects of Tooth Structure in Congenital Cytomegalovirus Infection

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646 PEDIATRICS Vol 69 No. 5 May 1982 the mesenchymal anlage ofthe dura and periosteum in the region of the anterior fontanel led to delayed

ossification, as membranous bone formation ap-pears to be directed by these contiguous structures.

Defects in membranous bone formation occur at

other sites in the developing calvarium and have

included almost total agenesis of the parietal

bones2’ and anterior transorbital meningoencepha-loceles.

A

more closely analogous defect, however, can

form in the region of the posterior fontanel.Z ?fl

occurs in familial distribution and is usually

unas-sociated with other skeletal abnormalities. This

anomaly may originate as a persistent large

poste-rior sagittal fontanel, which then is divided in the midline by mesial growth of membranous bone

resulting in paired parietal foramena. These fora-mena may be the site through which emissary yes-sels course and their persistence may be evident in

adulthood. Anterior parietal foramina occur

rarely.’ A relationship may exist between the

per-sistent posterior fontanel destined to become pan-eta.! foramena and the anterior fontanel defect noted in our patient.

ROBIN

I.

DAVIDSON,

MD

P.

KLINEMAN,

MD

Departments of Surgery and Radiology

University of Massachusetts Medical School

Worcester

REFERENCES

1. Acheson RM, Jefferson E: Some observations on the closure of the anterior fontanelle. Arch Dis Child 29:196, 1954 2. Aisenson MR: Closing of the anterior fontaneile. Pediatrics

6:223, 1950

3. Bai K, Subrahmanyam MJG, Rao KVS: Fontanels: Range

ofnormal size. Indian Pediatr 10:667, 1973

4. Boryslawski K, Kornafel D: Pr#{244}baokreslenia normy rozwo-jowej dia wielkosci i szybkedci zarastania duIego ciemia#{231}zka (fonticulus anterior) u dzieci Wroclawskich. Pediatr Pol

53:345, 1978

5. Davies DP, Ansari BM, Cooke TJH: Anterior fontanelle size

in the neonate. Arch Dis Child 50:81, 1975

6. Katiyar GP, Sen 5, Agarwal KN: Anterior fontanelle during infancy. Indian Pediatr 12:1253, 1975

7. Popich, GA, Smith PW: Fontanels: Range of normal size. J

Pediatr 80:749, 1972

8. Segali HD, Mikity VG, Rumbaugh CL, et al: The radiology

of the normal anterior fontanelle. Radiology 107:105, 1973 9. Tan KL: Wide sutures and large fontanelles in the newborn.

Am JDiS Child 130:386 1976

10. Lemir RJ, Loeser JD, Leech RW, et al: Normal and

Abnor-mal Development ofthe Human Nervous System. New York, Harper & Row, 1975

11. Reader MM, Felson B: Gamuts in Radiology. Cincinnati, Audiovisual Radiology of Cincinnati Co., 1975

12. Smith DW: Recognizable Patterns of Human Malforma-tion. Philadelphia, WB Saunders, 1970

13. Matson DD: Neurosurgeiy of Infancy and Childhood.

Springfield, IL, Charles C Thomas, 1969

14. Milhorat TH: Pediatric Neurosurgery. Philadelphia, FA Davis Co. 1978

15. O’Brien BM, Drake JE: Congenital defects of the skull and scalp. Br J Pleat Surg 13:102, 1960

16. Gibson NF, Ball, MM, Kelsey DS, et a!: Anterior fontanelle herniation. Pediatrics 56:466, 1975

17. Adeloye A, Odeku EL: Congenital subgaleal cysts over the anterior fontanelle in Nigerians. Arch Dis Child 46:95, 1971 18. Girdany BR, Blank E: Anterior fontanelle bones. Am J

Radiol 95:148, 1965

19. Mehra 5, Mohan M: Anterior fontanelle bone. Indian Pe-diatr 16:925, 1979

20. Trosc V, Frasin G, Ardeleanu D: Os fontanelar anterior La

nou-nascuti. Rev Med Chir Soc Med Nat lasi 75:221, 1971 21. Sela M, Sahar A, Lewin-Epstein J: Agenesis ofparietal bones

with restoration of the cranial vault. J Neurosurg 50:674, 1979

22. Davidson RI, Klineman PK: Anterior transorbital menin-goencephaloceles: A defect in the pars orbitalis of the frontal

bone. Am J Neuroradiol 1:579, 1980

23. Pendergrass EP, Petter OHP: Observations on the process ofossification in the formation ofpersistent enlarged parietal foramena. Am J Radiol 41:343, 1939

24. Rabinowitz JG: Pediatric Radiology Philadelphia, JB

Lip-pincott Co, 1978

Defects

of Tooth

Structure

in

Congenital

Cytomegalovirus

Infection

In most populations congenital cytomegalovirus (CMV) infections occur with a frequency of 0.5% to

Reprint requests to (5.5.) Department of Pediatrics, University of Alabama in Birmingham, Room 609, CDLD Bldg, University Station, Birmingham, AL 35294.

2% of live births. In the newborn period this infec-tion is largely clinically inapparent; only 5% to 10% of infected infants manifest ifiness.’ In this small

group of symptomatic babies, both the type and

severity of abnormalities have a wide spectrum.2 The most frequent manifestations are microceph-aly, hepatosplenomegaly, petechia, jaundice, and

growth retardation. Although signs of central

ner-vous system involvement are not usually evident at birth, they become apparent within the first few years of life in nearly 90% of children with

symp-torus at birth. The most common manifestations

are microcephaly (70%), intellectual impairment

(60%), neuromuscular disorder (35%), hearing loss

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EXPERIENCE AND REASON 647

(30%), and chorioretinitis or optic atrophy (22%). It is important to note that sequelae also occur in

approximately 15% of children who appear totally normal at birth. In this group, hearing loss is by far

the most frequent handicap whereas overt neuro-logic signs are rare.’ Intellectual and behavioral

problems have also been associated with

asympto-matic congenital CMV infections.

Teratogenic defects in congenital CMV infection

are infrequent.3’4 The most common structural

anomalies include inguinal hernias in males,

anom-alies of the first brachial arch, and structural defects of the central nervous system. The purpose of this

communication is to describe a specific anomaly of

tooth structure that affects mainly primary denti-tion.

RESULTS

The study population comprised 118 patients

with congenital CMV infection; all were older than

18 months at the time of dental examination. Typ-ical signs and symptoms of congenital CMV infec-tion were evident at birth in 25 patients whereas

the remaining 93 had a subclinical infection.

Con-genital CMV infection was documented in all pa-tients by isolation of virus from urine during the

first three weeks of life. All patients have been

longitudinally followed.

Tooth defects occurred in 40% (10/25) of those children with the more severe form of infection and in 5.4% (5/93) of those born with asymptomatic infections. One patient with tooth defects (from the asymptomatic group) also had nail-patella

syn-drome, making the origin of the tooth malformation

unclear. Of the children with dental defects, 11 received at least one course of penicillin therapy during the first year of life for conditions such as otitis media and pneumonia. One of these infants also received a course of gentamicin in conjunction with pemcfflin; however, none received tetracy-dines. There is no indication of excessive exposure to fluoride. Five of these infants were allowed to nurse from the bottle while in bed unattended, but only one of them had a combination of typical bottle canes and a defect of amelogenesis.

Clini-cally, the defect of tooth structure is generally more

severe in infants with symptomatic infection at birth inasmuch as when it occurs all, or nearly all, of the teeth are affected. The defect is characterized

by generalized yellowish discoloration. The enamel

is opaque and apparently hypocalcified inasmuch

as some children had incisors with fractured border (Figure). In many cases the enamel is simply absent, and affected teeth tend to wear down rapidly. The defect is less extensive and severe in infants with

asymptomatic congenital CMV infection. Rampant

Figure. Cytomegalovirus (CMV)-affected teeth. This

patient had clinically severe congenital infection. Note

fractured borders and opaque and hypocalcified enamel.

dental canes is also frequently seen in both groups

of affected children. Thus far, three deciduous in-cisors have been available for microscopic exami-nation. They showed generalized brown to yellow

discoloration with marked incisal attrition. This

initial observation will be further explored to assess a possible specific CMV-mediated effect on devel-opment and maturation of enamel.

DISCUSSION

A distinct defect of amelogenesis occurred in a significant number of children with congenital CMV

infections; thus, this infection may be considered as a new contributory factor to the etiology of dental defects. The group being observed at present is too young for clinical determination of whether enamel defects wi.ll occur in permanent teeth. Inasmuch as

congenital CMV infections are distinctively chronic,

anomalies of calcification of permanent incisors

in-cluding perhaps the first molars may occur, partic-ularly among those infants who remain ill in the first six months of life.

Patients with congenital CMV infections should

receive good dental preventive care as they have

high susceptibility to caries. In observing these patients, the initial impression is that many in the group may require intensive orthodontic therapy for two important reasons: problems of calcification of the teeth, to some extent, and possibly some defects of growth and development of the

dentofa-cial complex secondary to microcephaly and growth

retardation.

This anomaly of enamel in infants with

congeni-tal CMV infection is particularly common among those infants who, based on the clinical expression of disease at birth, seem to have suffered from a more severe infection in utero. The anomaly seems

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648 PEDIATRICS Vol 69 No. 5 May 1982

to derive from a defect in amelogenesis. Whereas

enamel hypoplasia has been related to many

sys-temic diseases, such as scurvy, congenital rubella and syphilis, nephrotic syndrome, and cerebral palsy of undefined etiology, none of these entities

occurred in these patients. With a prevalence of

congenital CMV infections ranging from 0.5% to

2.0% in most populations of the world, and the

relatively high frequency ofresulting dental defects,

it seems clear that preventive dental care should be

provided on a longitudinal basis to all these

pa-tients.

ACKNOWLEDGMENTS

This work was supported by grants from the National Institutes of Child Health and Human Development

(HD-10699), the General Clinical Research Center

(5-M01-RR32), and the National Institutes of Health

(NIDR-DE-02670).

SERGI0 STAGNO, MD

ROBERT F. PASS,

MD

JOE P. THOMAS, DDS

JUAN M. NAVIA, PHD MEYER E. Dwoisxy, MD

Departments of Pediatrics and Microbiology

School of Medicine

Institute of Dental Research, and School of Dentistry

University of Alabama in Birmingham Birmingham, Alabama

REFERENCES

1. Stagno 5, Reynolds DW, Amos CS, et al: Auditory and visual defects resulting from symptomatic and subclinical

congen-ital cytomegaloviral and toxoplasma infections. Pediatrics 59:669, 1977

2. Pass RF, Stagno 5, Myers GJ, et al: Outcome of symptomatic

congenital cytomegalovirus infection: Results of long-term longitudinal follow-up. Pediatrics 66:758, 1980

3. Reynolds DW, Stagno 5, Afford CA: Congenital

cytomega-lovirus infection. Teratology 17:179, 1978

4. Hanshaw JB: Developmental abnormalities associated with

congenital cytomegalovirus infection. Teratology 4:64, 1973 5. Curtis AC, Philpott OS: Prenatal syphilis. Med Clin North

Am 48:707,1964

6. Kraus BS, Ames MD, Clark GR: Effects of maternal rubella on dental crown development. Cliii Pediatr 8:204, 1969

7. Shusterman 5, Fellers FX: The prevalence ofenamel defects

in childhood nephrotic syndrome. J Dent Child 62:425, 1969 8. Witkop CF, Rao 5: Inherited defects in tooth structure.

Birth Defects 3:153, 1971

THE APPROPRIATE USE OF FUNDS

The prime minister of one of the underdeveloped countries recently asked

WHO

to provide a computer-assisted whole-body scanner. I pointed out that

this would absorb WHO’s entire budget for his country for two years: for the same amount of money his country could immunize all children against measles for 10 years, thereby saving the lives of perhaps 500,000 children. The president

of another country, concerned about malaria, wanted help for a campaign

against the mosquito vector. A vector-control campaign can be effective, but it

takes time: the thing to do right now to prevent the death of 100,000 children a year in his country is to get inexpensive malaria-control pills to everyone

suffering from the disease or exposed to it.

From H. Mahier, Scientific American, 243, September 1980.

Submitted by Student

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1982;69;646

Pediatrics

Sergio Stagno, Robert F. Pass, Joe P. Thomas, Juan M. Navia and Meyer E. Dworsky