JOURNAL OF TROPICAL PEDIATRICS, VOL. 60, NO. 1, 2014
Case Report
Tiny Hair Tuft: A Clue for Occipital Cephalocele
by Nevin Hatipoglu, Özden Türel, Husem Hatipoglu, Nuri Engerek, and Rengin Siraneci
Pediatrics Department, Kanuni Sultan Suleyman Education and Research Hospital, Halkali, Istanbul, Turkey.
Correspondence: Nevin Hatipoglu, Pediatrics Department, Pediatric Infectious Diseases Specialist, Kanuni Sultan Suleyman
Education and Research Hospital, Halkali, Istanbul, Turkey. Tel: þ90 532 784 15 23. Fax: þ90 212 571 47 90.
E-mail <[email protected]>.
Summary
Recurrent meningitis is a rare problem and can be due to alterations in immune system, or craniospinal
defect. Any clue either in patient’s history or physical examination would be helpful for avoiding unnecessary and tiring tests. Here we present the case of a child with recurrent bacterial meningitis who
had an unnoticed hair tuft on the occipital region. The final diagnosis was occipital cephalocele with a
rare presentation of a tiny tuft of hair.
Key words: cephalocele, hair tuft, meningitis.
Congenital anomalies of the nervous system might be
related to recurrent meningitis, which can lead to
significant morbidity and mortality [1]. A detailed
history, thorough physical examination (especially
paying attention to midline structures) and targeted
investigations play a pivotal role in uncovering the
underlying etiology.
Case
A 2.5-year-old girl was admitted to our hospital with
high fever, vomiting and headache. She was normal
in neurological development. She was alert and had
neck stiffness with positive meningeal signs.
Laboratory examination revealed leukocytosis and
a high C-reactive protein level. A lumbar puncture
was performed, demonstrating cloudy cerebrospinal
fluid (CSF) with elevated pressure, pleocytosis (480
leukocytes/mm3, 80% polymorphonuclear cells),
protein 261 mg/dl and glucose 4 mg/dl. CSF culture
remained sterile, but Streptococcus pneumoniae grew
on blood culture. She was discharged after a 14-day
course of ceftriaxone therapy with full recovery.
Acknowledgements
All authors took care of the patient; all authors were
involved in the writing and final checking of the
manuscript. All authors work in the hospital named
‘Kanuni Sultan Suleyman Education and Research
Hospital, Pediatrics Department’ in Istanbul,
Turkey.
She was referred again to our unit after 4 weeks
with recurrent purulent meningitis. S. pneumoniae
was isolated on CSF culture. She had no serious bacterial infection or a sepsis attack other than past meningitis infection. Tests for immunodeficiency, HIV
status and splenic function were all insignificant. She
had no history of head trauma or a history of CSF
leakage. Otoscopic examination was normal. There
was no detectable skin lesion over the spinal cord. A
tuft of hair with a root of 1 mm in diameter over the
occipital region at midline was discovered with a more
detailed scalp examination (Fig. 1). A magnetic resonance imaging scan revealed a defect on the occipital
bone with minimal protrusion of infratentorial structures (Fig. 2). This finding was compatible with an
occipital cephalocele. Segmentation anomalies of the
cervical vertebrae, posterior arcus defect of the fourth
vertebral level and a narrow syringohydromyelic
cavity of the cervical spinal cord were also noted.
The patient underwent corrective neurosurgical procedure at the end of antibiotic therapy. Pathologic
diagnosis confirmed this lesion to be an encephalocele
containing cerebellar structures. The mentioned
cephalocele of this patient was the cause of two attacks
of pyogenic meningitis.
Discussion
Recurrent meningitis is defined as two or more episodes
of meningitis separated by a period of complete resolution of signs, symptoms and laboratory findings.
Recurrent bacterial meningitis is not only an emergency
but also a challenging situation for the physician, as a
variety of, sometimes exhausting, diagnostic tests have
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doi:10.1093/tropej/fmt073
Advance Access published on 27 August 2013
87
CASE REPORT
FIG. 1. A tiny tuft of hair on the occipital bone
(arrow).
to be performed to uncover the real cause. Bacterial
infections of the meninges will recur until the underlying cause has been treated [2]. Severe immunodeficiency disorders occupy one end of spectrum, whereas
anatomic defects constitute the other end [3].
Children with a compromised immune status often
have a history of recurrent bacterial and/or opportunistic infections anywhere in the body. Our patient
had suffered from only two episodes of bacterial
meningitis and no severe infection in any site of the
body. However, immunoglobulins A, M, G and E;
subgroups of G levels and total complement function
were within normal range.
Anatomic problems, either congenital or acquired,
constitute up to 59% of recurrent bacterial meningitis
[3]. Developmental anomalies or posttraumatic
pathologies of cranial and spinal structures should
be sought and eliminated by applying modern imaging
tools of the nervous system. Possible spinal anomalies
include neural tube closure anomalies, dermal sinus
tracts, intraspinal cysts, lipoma or other tumors [4].
Cephaloceles, also termed as encephaloceles, encompass all neural tube defects that result in abnormal
herniation of intracranial structures. The incidence of
encephaloceles varies between 0.8 and 3.0 per 10 000
live births [5–7]. Encephaloceles are located most frequently over the occipital bone [8]. Hydrocephalus,
corpus callosum or cerebellar anomalies and any
kind of other brain structure malformations as well
as extracranial malformations can accompany them.
Fortunately, minor anatomical malformations of the
cervical spine with no involvement of neurological
problem were detected in the presented case.
Hypertrichosis, sacral dimple, vascular marking,
sinus ostium, skin tag and subcutaneous lipoma are
well-known markers that suggest a spinal defect [9].
A cutaneous abnormality of spine can be easily seen
88
FIG. 2. A defect on the occipital bone with occipital
cephalocele (arrow) and cervical syringohydromyelia
(arrowhead).
even in a newborn when the back is examined carefully. But lesions located in the occipital region,
where they may be covered by hair, would be challenging to identify. A fine black hair can be due to a
dermoid cyst in the occipital region [10,11]. A hair
tuft has been described also as a presenting sign of
rudimentary meningoceles [12,13].
Occipital cephaloceles can be easily diagnosed, as
they are generally large enough to be seen. To our
knowledge, a tiny tuft of hair, like in this patient, is
rare for occipital cephalocele. This case was presented to emphasize the importance of careful physical examination to improve overall outcome. Early
recognition of such an anomaly would be very helpful for orientation of diagnostic tools. Any case of
recurrent bacterial meningitis deserves occult clues of
diagnosis.
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