title: The scalp hair collar and tuft signs: A retrospective multicenter study of 78 patients with a systematic review of the literature.
abstract: BACKGROUND
  Hair collar sign (HCS) and hair tuft of the scalp (HTS) are cutaneous signs of an underlying neuroectodermal defect, but most available data are based on case reports.
  
  
  OBJECTIVE
  We sought to define the clinical spectrum of HCS and HTS, clarify the risk for underlying neurovascular anomalies, and provide imaging recommendations.
  
  
  METHODS
  A 10-year multicenter retrospective and prospective analysis of clinical, radiologic, and histopathologic features of HCS and HTS in pediatric patients was performed.
  
  
  RESULTS
  Of the 78 patients included in the study, 56 underwent cranial and brain imaging. Twenty-three of the 56 patients (41%) had abnormal findings, including the following: (1) cranial/bone defect (30.4%), with direct communication with the central nervous system in 28.6%; (2) venous malformations (25%); or (3) central nervous system abnormalities (12.5%). Meningeal heterotopia in 34.6% (9/26) was the most common neuroectodermal association. Sinus pericranii, paraganglioma, and combined nevus were also identified.
  
  
  CONCLUSIONS
  The partial retrospective design and predominant recruitment from the dermatology department are limitations of this study.
  
  
  CONCLUSIONS
  Infants with HCS or HTS are at high risk for underlying neurovascular anomalies. Magnetic resonance imaging scans should be performed in order to refer the infant to the appropriate specialist for management.
authors.affiliation: Department of Dermatology, Saint-Eloi Hospital, Montpellier, France; Montpellier University Hospital and Institut National de la Santé et de la Recherche Médicale (INSERM) U1058, Montpellier, France. Electronic address: d-bessis@chu-montpellier.fr.
authors.forename: Didier
authors.initials: D
authors.lastname: Bessis
authors.affiliation: Department of Infantile Plastic Surgery, Lapeyronie Hospital, Montpellier, France.
authors.forename: Michèle
authors.initials: M
authors.lastname: Bigorre
authors.affiliation: Department of Dermatology, Saint-Eloi Hospital, Montpellier, France.
authors.forename: Nausicaa
authors.initials: N
authors.lastname: Malissen
authors.affiliation: Department of Infantile Plastic Surgery, Lapeyronie Hospital, Montpellier, France.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Captier
authors.affiliation: Department of Dermatology, Archet-2 Hospital, Nice, France.
authors.forename: Christine
authors.initials: C
authors.lastname: Chiaverini
authors.affiliation: Department of Dermatology, Brest University Hospital, Brest, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Abasq
authors.affiliation: Department of Dermatology, Hôtel-Dieu Hospital, Nantes, France.
authors.forename: Sébastien
authors.initials: S
authors.lastname: Barbarot
authors.affiliation: Department of Pediatric Dermatology, Necker-Enfants Malades Hospital, Paris, France.
authors.forename: Olivia
authors.initials: O
authors.lastname: Boccara
authors.affiliation: Department of Pediatric Dermatology, Robert-Debré Hospital, Paris, France.
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Bourrat
authors.affiliation: Department of Infantile Neurosurgery, Caremeau Hospital, Nîmes, France.
authors.forename: Hassan
authors.initials: H
authors.lastname: El Fertit
authors.affiliation: Department of Dermatology, Robert-Debré Hospital, Reims, France.
authors.forename: Catherine
authors.initials: C
authors.lastname: Eschard
authors.affiliation: Department of Dermatology and Infectious Diseases, Fréjus Hospital, Fréjus, France.
authors.forename: Thomas
authors.initials: T
authors.lastname: Hubiche
authors.affiliation: Department of Dermatology, Archet-2 Hospital, Nice, France.
authors.forename: Jean-Philippe
authors.initials: JP
authors.lastname: Lacour
authors.affiliation: Department of Neuroradiology, Gui de Chauliac Hospital, Montpellier, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Leboucq
authors.affiliation: Department of Dermatology, Argenteuil Hospital, Argenteuil, France.
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Mahé
authors.affiliation: Department of Dermatology, Timone Hospital, Marseille, France.
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Mallet
authors.affiliation: Department of Dermatology, Caremeau Hospital, Nîmes, France.
authors.forename: Myriam
authors.initials: M
authors.lastname: Marque
authors.affiliation: Department of Dermatology, Angers University Hospital, Angers, France.
authors.forename: Ludovic
authors.initials: L
authors.lastname: Martin
authors.affiliation: Department of Dermatology, National Center for Rare Skin Disorders, Larrey Hospital, Toulouse, France.
authors.forename: Juliette
authors.initials: J
authors.lastname: Mazereeuw-Hautier
authors.affiliation: Department of Dermatology, Saint-Eloi Hospital, Montpellier, France.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Milla
authors.affiliation: Department of Pediatric Dermatology, Femme-Mère-Enfant Hospital and Claude-Bernard Lyon 1 University, Lyonm, France.
authors.forename: Alice
authors.initials: A
authors.lastname: Phan
authors.affiliation: Department of Dermatology, Quimper Hospital, Quimper, France.
authors.forename: Patrice
authors.initials: P
authors.lastname: Plantin
authors.affiliation: Unit of Clinical Research and Epidemiology, Department of Medical Information, Antonin Balmes Hospital, Montpellier, France.
authors.forename: Marie-Christine
authors.initials: MC
authors.lastname: Picot
authors.affiliation: Department of Dermatology, Besançon University Hospital, Besançon, France.
authors.forename: Eve
authors.initials: E
authors.lastname: Puzenat
authors.affiliation: Department of Pathology, Montpellier University Hospital, Montpellier, France.
authors.forename: Valérie
authors.initials: V
authors.lastname: Rigau
authors.affiliation: Department of Dermatology, Bocage Hospital and Bourgogne Medical University, Dijon, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Vabres
authors.affiliation: Department of Pathology, Necker-Enfants Malades University Hospital, Paris, France.
authors.forename: Sylvie
authors.initials: S
authors.lastname: Fraitag
authors.affiliation: Department of Pediatric Dermatology, National Center for Rare Skin Disorders-Institut National de la Santé et de la Recherche Médicale (INSERM) U1035, Bordeaux, France.
authors.forename: Franck
authors.initials: F
authors.lastname: Boralevi
mesh_terms: D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging*; D001921:Brain / Q000002:abnormalities / Q000000981:diagnostic imaging*; D002828:Choristoma / Q000000981:diagnostic imaging*; D005188:False Negative Reactions; D005189:False Positive Reactions; D005260:Female; D006197:Hair / Q000002:abnormalities*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D008578:Meninges; D064847:Multimodal Imaging; D054258:Neural Plate; D059906:Neuroimaging; D011446:Prospective Studies; D012189:Retrospective Studies; D012535:Scalp / Q000473:pathology; D012886:Skull / Q000002:abnormalities / Q000000981:diagnostic imaging*; D014057:Tomography, X-Ray Computed; D018615:Ultrasonography, Doppler, Color; D014680:Veins / Q000002:abnormalities / Q000000981:diagnostic imaging*
keywords: cephalocele; hair collar; hair tuft; membranous aplasia cutis; meningocele; paraganglioma; sebaceous nevus; sinus pericranii; smooth muscle cell nevus