Cutis Verticis Gyrata
Cutis Verticis Gyrata (CVG)
Cutis verticis gyrata is a rare morphological condition characterized by convoluted folds and deep furrows of the scalp resembling cerebral gyri.
Key Points
- Rare condition affecting scalp morphology
- Can be primary or secondary
- Male predominance (male:female ratio 5:1)
- Usually presents during puberty or early adulthood
- May indicate underlying systemic conditions
Epidemiology
- Prevalence: 1 in 100,000 males
- Rare in females
- Peak onset: puberty to 30 years
- Higher prevalence in certain syndromes
- Geographic and ethnic variations reported
Classifications
Primary Forms
- Primary Essential:
- No underlying condition
- Male predominance
- Post-pubertal onset
- Normal scalp thickness
- Primary Non-Essential:
- Associated with neurologic/ophthalmologic conditions
- Earlier onset
- Mental retardation common
- Cerebriform changes more pronounced
Secondary Forms
- Inflammatory Conditions:
- Eczema
- Psoriasis
- Folliculitis
- Impetigo
- Neoplastic Conditions:
- Cerebriform intradermal nevus
- Neurofibroma
- Giant congenital melanocytic nevus
- Cutaneous lymphoma
- Systemic Diseases:
- Acromegaly
- Amyloidosis
- Graves' disease
- Diabetes mellitus
Pathophysiology
Primary CVG
- Genetic Factors:
- Autosomal dominant inheritance
- Possible X-linked inheritance
- Gene mutations
- Developmental abnormalities
- Structural Changes:
- Dermal thickening
- Collagen bundle reorganization
- Elastic fiber changes
- Connective tissue proliferation
Secondary CVG
- Inflammatory Mechanisms:
- Chronic inflammation
- Tissue remodeling
- Fibrosis
- Scar formation
- Hormonal Influences:
- Growth hormone excess
- Androgen effects
- Thyroid dysfunction
- Insulin resistance
Histopathological Features
- Primary Forms:
- Normal skin architecture
- Thickened dermis
- Increased collagen
- Normal elastic fibers
- Secondary Forms:
- Variable findings
- Disease-specific changes
- Inflammatory infiltrates
- Specific tissue alterations
Clinical Features
Morphological Features
- Scalp Characteristics:
- Symmetric folds and furrows
- Soft, spongy consistency
- Non-inflammatory appearance
- Cannot be flattened by pressure
- Distribution Pattern:
- Predominantly parietal and occipital
- Anteroposterior orientation
- Symmetric involvement
- Variable extent
Associated Features
- Primary Essential:
- Normal hair growth
- No scalp tenderness
- No systemic symptoms
- Normal intelligence
- Primary Non-Essential:
- Neurologic abnormalities
- Ophthalmologic defects
- Intellectual disability
- Seizures
Associated Conditions
Neuropsychiatric Associations
- Neurological Conditions:
- Seizure disorders
- Microcephaly
- Cerebral palsy
- Brain tumors
- Psychiatric Conditions:
- Schizophrenia
- Intellectual disability
- Behavioral disorders
- Autism spectrum disorders
Syndromic Associations
- Known Syndromes:
- Turner syndrome
- Noonan syndrome
- Tuberous sclerosis
- Ehlers-Danlos syndrome
- Endocrine Disorders:
- Acromegaly
- Diabetes mellitus
- Thyroid dysfunction
- Growth hormone excess
Diagnosis
Clinical Assessment
- History Taking:
- Age of onset
- Family history
- Associated symptoms
- Progression pattern
- Physical Examination:
- Scalp morphology
- Hair pattern
- Associated features
- Systemic examination
Diagnostic Studies
- Essential Tests:
- Skin biopsy
- Photography documentation
- Neuroimaging
- Endocrine evaluation
- Additional Studies:
- Genetic testing
- Ophthalmologic examination
- Psychiatric evaluation
- IQ assessment
Management
Treatment Approaches
- Primary CVG:
- Surgical correction
- Scalp care
- Psychological support
- Regular monitoring
- Secondary CVG:
- Treatment of underlying condition
- Symptomatic management
- Prevention of complications
- Multidisciplinary approach
Surgical Management
- Surgical Techniques:
- Total scalp excision
- Partial tissue removal
- Tissue expansion
- Reconstructive procedures
- Considerations:
- Timing of surgery
- Patient selection
- Surgical risks
- Post-operative care
Special Considerations
Pediatric Aspects
- Growth Monitoring:
- Regular assessment
- Developmental screening
- Early intervention
- Family support
- Quality of Life:
- Psychosocial support
- School adaptation
- Peer relationships
- Family counseling
Long-term Care
- Follow-up Care:
- Regular monitoring
- Complication prevention
- Growth assessment
- Psychological support
- Prevention Strategies:
- Scalp hygiene
- Infection prevention
- Early intervention
- Regular screening
Disclaimer
The notes provided on Pediatime are generated from online resources and AI sources and have been carefully checked for accuracy. However, these notes are not intended to replace standard textbooks. They are designed to serve as a quick review and revision tool for medical students and professionals, and to aid in theory exam preparation. For comprehensive learning, please refer to recommended textbooks and guidelines.
