0
Special Feature |

Picture of the Month—Diagnosis FREE

[+] Author Affiliations

Section Editor: Samir S. Shah, MD, MSCE

More Author Information
Arch Pediatr Adolesc Med. 2010;164(6):578. doi:10.1001/archpediatrics.2010.68-b.
Text Size: A A A
Published online

Magnetic resonance imaging revealed a 1.5-cm cystic scalp lesion in the midline just superior to the cerebellum. Its enhancement paralleled the cerebrospinal fluid enhancement on all sequences, and it overlaid a skull defect. Craniectomy and excision revealed a meningocele.

Most scalp lesions are benign; however, up to 37% of solitary, nontraumatic scalp nodules extend intracranially to the dura mater or brain.1A midline location suggests embryologic abnormalities with potential for intracranial communication. At 3 to 5 weeks of development, the cranial neural tube fuses at the midline, followed by separation of the surface ectoderm away from the neural tube.2Defective midline fusion results in dysraphism. The differential diagnosis for a congenital midline scalp nodule includes cephaloceles, dermoid cysts, and heterotopic brain tissue in addition to more common entities such as hemangiomas and lipomas. Similarly, a midline scalp ulceration raises concern for aplasia cutis congenita (ACC) and underlying embryologic malformation.

The term cephaloceledescribes herniation of intracranial structures through a scalp defect and includes meningocele, encephalocele, and meningoencephalocele. These defects appear as pink or blue compressible nodules that pulsate or fluctuate with activity. They are frequently located on the parietal or occipital midline of the scalp.3Cephaloceles vary in size, with some lesions presenting as small plaques and others affecting a significant portion of the scalp. The hair collar sign, a band of thick, dark, coarse hair encircling the scalp nodule, is common with cephaloceles.24It is also seen with heterotopic rests and ACC. While not specific, its presence mandates radiologic workup for intracranial communication.4,5

Dermoid cysts are common childhood scalp nodules and are also a consequence of abnormal fusion.1They are composed of disorganized dermal and epidermal tissue, are typically 1 to 4 cm, and are noncompressible. Often they are located along fusion lines, including the glabellar region, the posterior scalp, and the lateral brow, although the latter rarely shows intracranial extension.1Midline location or an overlying dimple or sinus ostia are particularly concerning for an intracranial communication. Intracranial communication is present in 11% of midline cysts, is found almost exclusively in midline cysts, and most commonly communicates to the posterior fossa.6Conversely, intracranial dermoid cysts in the posterior fossa may communicate to a posterior scalp nodule via a sinus tract. Serious complications such as infections, aseptic meningitis, and mass effect are associated with the latter condition.7

Heterotopic brain tissue is a sequestrated rest of brain or meninges most commonly located on the occipital or parietal area of the scalp.8It presents similarly to cephaloceles, including a hair collar sign, but lacks intracranial communication.

In contrast to a scalp nodule, ACC is a congenital absence of skin that most commonly presents as a small area of ulceration or full-thickness skin loss on the vertex scalp. Larger ACC lesions are more commonly associated with underlying defects in the skull or meninges,9and 20% of vertex ACCs will have an associated underlying bone defect.10They may also present with a hair collar sign.4

The patient presented with a pink, hairless, glistening scalp nodule that expanded with maturity. The lesion was atypical for more common entities such as hemangioma, port-wine stain, ACC, or sebaceous nevus.

This case emphasizes that congenital scalp midline lesions are associated intracranial communication. Nodules in the midline that fluctuate with activity, have an overlying dimple, or have a hair collar sign are particularly concerning. High-risk lesions of ACC are often larger, irregularly shaped, and situated on the cranial vertex.11These features warrant a thorough physical examination, neurological examination, magnetic resonance imaging,12and usually a neurological surgery consult. Identification is critical as delayed diagnosis is associated with a higher incidence of neurological complications.13This patient's scalp nodule at the posterior midline prompted investigation and emphasizes that regardless of age or symptoms at presentation, a midline scalp lesion deserves medical attention.

Return to Quiz Case.

Correspondence:Maria Garzon, MD, Department of Dermatology, Herbert Irving Pavilion, 12th Floor, 161 Fort Washington Ave, New York, NY 10032 (mcg2@columbia.edu).

Accepted for Publication:December 16, 2009.

Author Contributions:Study concept and design: Daly, Barnett, and Garzon. Acquisition of data: Daly and Garzon. Analysis and interpretation of data: Feldstein and Garzon. Drafting of the manuscript: Daly and Garzon. Critical revision of the manuscript for important intellectual content: Barnett, Feldstein, and Garzon. Study supervision: Barnett, Feldstein, and Garzon.

Financial Disclosure:None reported.

Ruge  JRTomita  TNaidich  TPHahn  YSMcLone  DG Scalp and calvarial masses of infants and children. Neurosurgery 1988;22 (6, pt 1) 1037- 1042
PubMed
Drolet  B Cutaneous signs of neural tube malformations. Semin Cutan Med Surg 2004;23 (2) 125- 137
PubMed
Rogers  GFMulliken  JBKozakewich  HP Heterotopic neural nodules of the scalp. Plast Reconstr Surg 2005;115 (2) 376- 382
PubMed
Drolet  BAClowry  L  JrMcTigue  MKEsterly  NB The hair collar sign. Pediatrics 1995;96 (2, pt 1) 309- 313
PubMed
Harrington  BC The hair collar sign as a marker for neural tube defects. Pediatr Dermatol 2007;24 (2) 138- 140
PubMed
Crawford  R Dermoid cyst of the scalp. J Pediatr Surg 1990;25 (3) 294- 295
PubMed
Caldarelli  MMassimi  LKondageski  CDi Rocco  C Intracranial midline dermoid and epidermoid cysts in children. J Neurosurg 2004;100 (5) ((suppl pediatrics)) 473- 480
PubMed
Baldwin  HEBerck  CMLynfield  YL Subcutaneous nodules of the scalp. J Am Acad Dermatol 1991;25 (5, pt 1) 819- 830
PubMed
Frieden  IJ Aplasia cutis congenita. J Am Acad Dermatol 1986;14 (4) 646- 660
PubMed
Martínez-Lage  JFAlmagro  MJLópez Hernández  FPoza  M Aplasia cutis congenita of the scalp. Childs Nerv Syst 2002;18 (11) 634- 638
PubMed
Kos  LDrolet  B Developmental abnormalities. Eichenfield  LFrieden  IEsterly  NNeonatal Dermatology. Philadelphia, PA Saunders Elsevier2008;113- 130
Tracy  PTHanigan  WC Spinal dysraphism. Clin Pediatr (Phila) 1990;29 (4) 228- 233
PubMed
Ackerman  LLMenezes  AHFollett  KA Cervical and thoracic dermal sinus tracts. Pediatr Neurosurg 2002;37 (3) 137- 147
PubMed

Figures

Tables

References

Ruge  JRTomita  TNaidich  TPHahn  YSMcLone  DG Scalp and calvarial masses of infants and children. Neurosurgery 1988;22 (6, pt 1) 1037- 1042
PubMed
Drolet  B Cutaneous signs of neural tube malformations. Semin Cutan Med Surg 2004;23 (2) 125- 137
PubMed
Rogers  GFMulliken  JBKozakewich  HP Heterotopic neural nodules of the scalp. Plast Reconstr Surg 2005;115 (2) 376- 382
PubMed
Drolet  BAClowry  L  JrMcTigue  MKEsterly  NB The hair collar sign. Pediatrics 1995;96 (2, pt 1) 309- 313
PubMed
Harrington  BC The hair collar sign as a marker for neural tube defects. Pediatr Dermatol 2007;24 (2) 138- 140
PubMed
Crawford  R Dermoid cyst of the scalp. J Pediatr Surg 1990;25 (3) 294- 295
PubMed
Caldarelli  MMassimi  LKondageski  CDi Rocco  C Intracranial midline dermoid and epidermoid cysts in children. J Neurosurg 2004;100 (5) ((suppl pediatrics)) 473- 480
PubMed
Baldwin  HEBerck  CMLynfield  YL Subcutaneous nodules of the scalp. J Am Acad Dermatol 1991;25 (5, pt 1) 819- 830
PubMed
Frieden  IJ Aplasia cutis congenita. J Am Acad Dermatol 1986;14 (4) 646- 660
PubMed
Martínez-Lage  JFAlmagro  MJLópez Hernández  FPoza  M Aplasia cutis congenita of the scalp. Childs Nerv Syst 2002;18 (11) 634- 638
PubMed
Kos  LDrolet  B Developmental abnormalities. Eichenfield  LFrieden  IEsterly  NNeonatal Dermatology. Philadelphia, PA Saunders Elsevier2008;113- 130
Tracy  PTHanigan  WC Spinal dysraphism. Clin Pediatr (Phila) 1990;29 (4) 228- 233
PubMed
Ackerman  LLMenezes  AHFollett  KA Cervical and thoracic dermal sinus tracts. Pediatr Neurosurg 2002;37 (3) 137- 147
PubMed

Correspondence

CME
Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

See Also...
Articles Related By Topic
Related Topics
PubMed Articles