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PubMed This is a summary of 21 peer-reviewed journal articles Updated
Pediatrics

Warning Signs and Potential Risks

At a Glance

Most cases of aplasia cutis congenita heal safely, but caregivers must watch for red flags. The 'hair collar sign' or midline lesions may indicate underlying skull defects requiring an MRI. Seek emergency care immediately for active bleeding, thick pus, or signs of extreme lethargy.

While most cases of Aplasia Cutis Congenita (ACC) heal safely with simple care, certain clinical “red flags” and potential complications require immediate medical attention. Understanding these risks is essential for keeping your baby safe during the healing process [1][2].

The Hair Collar Sign: A Major Red Flag

One of the most important warning signs is called the hair collar sign [3]. This is a ring of longer, thicker, or darker hair that surrounds a scalp lesion [4][5].

If your baby has this sign, it suggests that the skin defect may not be isolated. It is often a marker for underlying issues, such as a gap in the skull bone or a connection to the brain’s protective layers [3][6]. Because of this risk, the presence of a hair collar sign usually means your doctor will recommend imaging—most often an MRI—to see what is happening beneath the skin [5][7].

Life-Threatening Risks

In rare cases where the ACC lesion is very large or deep, particularly on the top of the head, it can expose the superior sagittal sinus [8][9]. This is a major vein that drains blood from the brain. If this area is unprotected by bone or thick skin, two serious complications can occur:

  • Hemorrhage: Severe, life-threatening bleeding from the sinus can happen if the area is bumped or injured [8][10].
  • Thrombosis: A blood clot (thrombosis) can form in the sinus, which is a medical emergency that can lead to seizures, lethargy, or other neurological symptoms [8][11].

Any active bleeding from the lesion or signs of extreme sleepiness or seizures in your baby require immediate emergency care.

Identifying Infection vs. Normal Healing

As the ACC lesion heals, it will form granulation tissue. This is healthy, new tissue that is typically shiny, moist, and pink or red [12][13]. It is often mistaken for a problem, but it is actually a sign that the body is repairing itself [14].

However, you must watch for a secondary infection, which can be dangerous if it spreads to the deeper tissues [15][16].

Feature Healthy Granulation (Normal) Infected Wound (Warning)
Color Bright pink or red Dark red or dusky
Drainage Clear or slightly pink fluid Thick, yellow, or green pus
Surrounding Skin Normal color Spreading redness and warmth
Odor None Foul or unpleasant smell
Baby’s Temperament Feeding and sleeping normally Fever, irritability, or lethargy

When Imaging is Necessary

Not every baby with ACC needs a brain scan. However, doctors will typically order an MRI (the gold standard for this condition) or a cranial ultrasound in the following situations [17][18]:

  1. Hair Collar Sign: As mentioned, this is the primary reason for imaging [3].
  2. Midline Location: Lesions located exactly in the center of the scalp or over the soft spot (fontanelle) are higher risk [4][3].
  3. Large Size: Lesions larger than 2 centimeters are often evaluated more cautiously [19].
  4. Atypical Findings: The presence of lumps, fluid-filled sacs, or unusual blood vessels around the lesion [3][4].

Early imaging allows the medical team to rule out serious complications and ensures that your baby’s treatment plan is as safe as possible [20][21]. Learn how doctors tackle wound care on our Treating ACC: Wound Care and Surgery page.

Common questions in this guide

What is the hair collar sign in aplasia cutis congenita?
The hair collar sign is a ring of longer, thicker, or darker hair surrounding a scalp lesion. It is a major warning sign indicating that the skin defect might be connected to underlying skull or brain abnormalities, which usually requires an MRI to evaluate.
How can I tell if my baby's ACC lesion is infected?
Healthy healing tissue is typically shiny, bright pink or red, with clear drainage. An infection may cause thick yellow or green pus, a foul odor, spreading redness around the wound, and fever or extreme irritability in your baby.
When does a baby with aplasia cutis congenita need an MRI?
An MRI or cranial ultrasound is typically recommended if your baby has a hair collar sign, a large lesion over 2 centimeters, atypical lumps, or a lesion located exactly in the center of the scalp over the soft spot.
What happens if a large ACC scalp lesion exposes a vein?
If a large or deep lesion exposes the superior sagittal sinus on the top of the head, there is a serious risk of life-threatening bleeding or blood clots. Any active bleeding, extreme sleepiness, or seizures require immediate emergency care.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Does my baby have a 'hair collar sign,' and does that mean we need an MRI?
  2. 2.Is the superior sagittal sinus exposed, and what are the specific emergency signs of bleeding I should look for?
  3. 3.How can I tell the difference between healthy pink granulation tissue and a secondary infection in this wound?
  4. 4.At what size or depth does a lesion go from 'safe for home care' to 'requiring a specialist consult' or imaging?
  5. 5.Is a cranial ultrasound sufficient for screening, or do we need the detail of an MRI to rule out skull defects?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (21)
  1. 1

    Adams-Oliver syndrome, intestinal obstruction and heart defects: a case series of aplasia cutis congenita.

    Baroudi I, Alakhras O, Douri T, Alkhani N

    Oxford medical case reports 2022; (2022(1)):omab141 doi:10.1093/omcr/omab141.

    PMID: 35083055
  2. 2

    Aplasia cutis congenita: a report of two cases from National Hospital Abuja, Nigeria and review of the literature.

    Mukhtar-Yola M, Mshelia L, Mairami AB, et al.

    The Pan African medical journal 2020; (36()):291 doi:10.11604/pamj.2020.36.291.24523.

    PMID: 33117485
  3. 3

    Low risk of clinically important central nervous system dysraphism in a cohort study of 69 patients with isolated aplasia cutis congenita of the head.

    Kuemmet TJ, Miller JJ, Michalik D, et al.

    Pediatric dermatology 2020; (37(3)):455-460 doi:10.1111/pde.14117.

    PMID: 32053222
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    Aplasia cutis congenita: Evaluation of signs suggesting extracutaneous involvement.

    Patel DP, Castelo-Soccio L, Yan AC

    Pediatric dermatology 2018; (35(1)):e59-e61 doi:10.1111/pde.13340.

    PMID: 29178194
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    The scalp hair collar and tuft signs: A retrospective multicenter study of 78 patients with a systematic review of the literature.

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    Journal of the American Academy of Dermatology 2017; (76(3)):478-487 doi:10.1016/j.jaad.2016.08.046.

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    Two cases of aplasia cutis congenita with hair collar signs and macrophage hyperplasia.

    Takayama E, Harada A, Ikura Y, Seto H

    The Journal of dermatology 2019; (46(8)):734-738 doi:10.1111/1346-8138.14946.

    PMID: 31180149
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    [Cutaneous signs of occult cranial and spinal dysraphism].

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    [Aplasia cutis congenita: Update and management].

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    Large aplasia cutis congenita of the vertex conservative management.

    Bouali S, Charfeddine SH, Ghedira K, et al.

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    PMID: 26023367
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    [Diagnosis and treatment of five neonatal cerebral venous sinus thrombosis].

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    Aplasia cutis congenita of the trunk in a newborn: a rare case report.

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    Amniotic membrane dressings for treatment of aplasia cutis in newborns.

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    The Role of MRI in Diagnosing Agenesis of the Corpus Callosum (ACC): A Rare Disorder Present at Birth.

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This page discusses warning signs of aplasia cutis congenita for educational purposes only. Always seek immediate medical attention if your baby shows signs of infection, bleeding, or unusual lethargy.

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