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Delayed closure of the anterior fontanelle is often associ- S.D. is a 32-month-old African American boy who pre-
ated with significant disease entities. Range of normal sented to our clinic for a well-child physical examination.
closure of the anterior fontanelle is 4 to 26 months. He had no specific complaints at the time of presentation, and
Increased intracranial pressure, hypothyroidism, and his examination was essentially unremarkable except for the
skeletal anomalies are common etiologic factors. History, finding of an open anterior fontanelle measuring 3 cm 1.5
physical examination, and diagnostic testing rule out most cm. This was noted to be an unusual finding considering
disorders. Once these disorders have been ruled out, it is the patient’s age.
important for the physician to realize that a persistent S.D. was born to a 21-year-old gravida II para I African
open anterior fontanelle beyond the accepted ranges of American woman at term gestation. His mother’s prenatal
closure can be a normal outlier. course had been uncomplicated. Similarly, her previous preg-
(Key words: case report, pediatrics, family medicine, nancy was without complication. At birth, S.D. weighed 3019
open anterior fontanelle) g, was 49 cm long, and had a head circumference of 36 cm—
all appropriate for gestational age. Routine plotting on a
500 • JAOA • Vol 102 • No 9 • September 2002 Gaetano and Gaetano • Case report
work to include a screening urinalysis, complete metabolic toxicity can all be associated with a persistent open fontanelle.
profile, complete blood count, and thyrotropin was ordered These also are easily ruled out via a thorough history, blood
in an effort to rule out metabolic as well as endocrine etiologic levels, and thyroid function screening. All of the previously
factors. The CT scan revealed the open fontanelle; however, mentioned disease processes had been sufficiently ruled out
no pathologic intracranial processes were noted. Simultane- via the multiple histories, physical examinations, x-rays, and
ously, all laboratory work returned with normal values. laboratory studies that S.D. had undergone since birth. The
Potentially deleterious causes of persistent open anterior significance of a persistent open anterior fontanelle in an oth-
fontanelle were ruled out. S.D. was discharged after his par- erwise healthy 32-month-old child remained to be deter-
ents were reassured that his physical examination finding mined.
was a variant of normal and that closure would eventually A search of the literature yielded few results. One source1
occur. stated that in a healthy child with a persistent open fontanelle
Persistent open anterior fontanelle can be a common who has continued to plot accordingly on growth charts, the
finding in children. This normal variant must be recognized, finding is considered an outlier with no specific significance.
given the vast number of well-child examinations done annu- Yet another source4 stated that as long as a child’s head cir-
ally. cumference progresses along a normal curve and the neu-
In 1949, Milton Aisenson, MD, reviewed the records of rologic and ocular fundoscopic examinations yield normal
two child health stations of the New York City Department results, no further diagnostic studies are required.
of Health for over 10 years to determine the normal age at The persistent open fontanelle probably has a familial
which the anterior fontanelle closes. Examinations of 1677 inheritance pattern associated with it. Closure simply occurs
infants were recorded to note the age of closure of the ante- at a time beyond the accepted range of normal. The impor-
rior fontanelle. The range of closure was determined to be tance of recognizing the many etiologic factors responsible for
between 4 and 26 months.2 Ninety percent of the children’s a persistent open anterior fontanelle is crucial for physicians
fontanelles closed between 7 and 19 months, and 42% of the who care for children. Physicians must know the multiple syn-
fontanelles closed before 1 year.2 This study set the guidelines dromes, diseases, and toxic exposures that can cause a delayed
by which we examine our infant patients and counsel their closure.
parents about the closure of the anterior fontanelle. This case report and literature review demonstrate that
Delayed closure of the anterior fontanelle can be associ- once simple laboratory studies and x-rays are performed
ated with multiple diseases, most of which have dysmor- and the previously mentioned maladies are ruled out, a per-
phic features that should facilitate early recognition.3 Simple sistent open anterior fontanelle can be a normal finding. This
radiographic or laboratory studies rule out other common carries tremendous clinical relevance as we counsel our
causes. Increased intracranial pressure is the most common patients’ parents about the ramifications of this discovery. Par-
cause of delayed closure of the anterior fontanelle.3 Multiple ents can be told that there is probably a familial inheritance
etiologic factors are responsible for this phenomenon. Hydro- associated with delayed closure of the anterior fontanelle
cephalus, subdural hematomas, porencephalic cysts, and and that with time the fontanelle should close.
tumors are most frequently seen. All are easily and routinely
identifiable via plain skull series or CT scanning of the head. References
Many skeletal disorders are responsible for delayed clo- 1. Tunnessen WW Jr. Persistent open anterior fontanelle. JAMA. 1990;264:2450.
sure of the anterior fontanelle. Achondroplasia, osteogen- 2. Aisenson MR. Closing of the anterior fontanelle. Pediatrics. 1949;6:223-225.
esis imperfecta, vitamin D deficiency–rickets, and cleidocra-
nial dysostosis are the most common of these.3 These diseases 3. Tunnessen WW Jr. Signs and Symptoms in Pediatrics. Philadelphia, Pa: JB
Lippincott; 1988.
present with characteristic physical findings and are con-
firmed by associated laboratory and x-ray abnormalities. 4. Popich GA, Smith DW. Fontanelles: Range of normal size. J Pediatrics.
Chromosomal defects as well as dysmorphogenetic syn- 1972;80:749-752.
dromes also predispose infants to delayed closure of the
anterior fontanelle. Down, trisomy 13, trisomy 18, Russell-
Silver, Rubinstein-Taybi, and Robinow’s syndromes com-
monly encompass this physical finding within the constel-
lation of findings associated with that particular syndrome.
All are routinely identified early in child development as
the result of their significant dysmorphic features.
Endocrine disorders as well as drug and toxin exposure
are also associated with delayed closure of the anterior
fontanelle. Commonly, hypothyroidism, fetal hydantoin syn-
drome, aminopterin-induced malformations, and aluminum
Gaetano and Gaetano • Case report JAOA • Vol 102 • No 9 • September 2002 • 501