Cutaneous back lesions

 

When performing a physical exam on a newborn, the midline back should be examined carefully for a cutaneous back lesion. Because these lesions are associated with neural tube defects (NTDs)/spinal dysraphism, and are sometimes the only indication that one exists, it is important to look for them, as serious deficits can be prevented with early intervention. These lesions include:

  1. Sinus tracts: can connect cysts – that look like 1-4 cm rubbery nodules – to the bone, CNS, or nasal sinuses, and when they connect to the CNS, can cause meningitis with non-classical organisms
  2. Lipomas: can be in the dermis or spinal canal and pass through vertebral defect into the intraspinal space, which can be a cause of tetehered cord; small connections may be missed
  3. Hairy tufts (hypertrichosis): hair can be any color or texture but is often “silky” (“faun tail nevus”), V-shaped, and not well circumscribed. If prominent, this is a strong marker of an NTD, but it can also be normal if not localized and prominent.
  4. Hemangiomas, telangiectasias, and other capillary malformations: proliferative, common vascular tumors. Those that are associated often have a defect within it, pass the midline, are large, and associated with another lesion (e.g. lipoma).
  5. Dimples: Some dimples are benign, such as those over the coccyx with a visible base that is intact and most of those in the gluteal crease. Others are more worrisome, such as those that are deep and greater than 5 mm, are more than 2.5 cm from the anus, or are associated with other NTD markers. The gluteal fold should be separated and examined carefully for these.
  6. Aplasia cutis: absence of skin, rarely reported in lumbosacral region
  7. Skin tags/acrochordons/pseudotails: nodules extending from skin
  8. Skin hypertrophy/atrophy or hyperpigmentation/hypopigmentation

In addition to observation, the spine should be palpated to feel for incomplete fusion of the bony spine, and legs should be examined for equal length and sensory abnormalities. If the patient is slightly older, a history should be taken regarding bladder abnormalities (e.g. failure to urinate or empty bladder completely) and unexplained complication.

 

All of the above can indicate an underlying neural tube defect, NTD. (Notably, mongolion spots, which can also be mistaken for bruises, are benign.) With hypertrichosis, dimples, skin tags, lipomas, hemangiomas, aplasia cutis, or sinus tracts, there is a higher index of suspicion than with telangiectasias, hyperpigmentation, nevi, or teratomas.

 

If any cutaneous back lesion or abnormality is noted, an ultrasound of the lower back is the next screening tool, followed by an MRI (or CT if visualization of the bones are needed) and a neurosurgery consult.

 

What exactly is a neural tube defect? There are several kinds and can be either open or closed. Closed NTDs include spina bifida occulta, meningocele, and myelominingocele. In spina bifida, the vertebral arch is incompletely fused but no herniation of contents occurs – classically, this presents with a tuft of hair. In myelomeningocele, the meninges and neural tissue protrudes, and in meningocele, only the meninges protrudes. Open NTDs will not be discussed here, but includes anencephaly, which, together with spina bifida, is one of the most common NTDs.

 

Why do they occur? NTDs occur due to failure of closure of the neural tube, which happens by week 4 of gestation, before most women know they are pregnant. They can be prevented by supplementation of 0.4 mg of folic acid in the diet from conception to the end of the first trimester.

 

How common are they? In the United States, 1-2 of every 1000 newborns has a neural tube defect. It is the second most common congenital anomaly, and there are multiple factors that determine risk of an NTD, such as geographical location (Ireland and Wales high-risk), SES (poor high risk), maternal age (teenagers and older moms increased risk), and prenatal exposure to valproic acid (and other drugs) and maternal diabetes. If a first degree relative is affected that means another is more likely to have another affected.

 

Why screen? If a neural tube defect goes undetected, there can be serious consequences that can be prevented with early diagnosis and neurosurgical intervention. In meningocele and myelomeningocele, bowel and bladder sphincter dysfunction and sensorimotor loss below the lesion can result. The infant can be neurologically intact, but as the caudal end of cord is tethered to distal spine, when the spine grows, traction injury leads to issues with gait, sphincters, feet, and motor. Patients with spina bifida can get infectious complications, cortical malformations, and Chairi II malformations. In tethered cord, patients can be easily detected, as more than half of patients have a cutaneous lower back lesion while being otherwise asymptomatic until the child is walking or has neurological defects. This happens because as the spine grows, the cord cannot ascend as it normally does, leading to traction injuries and damage of the lower cord and conus. Symptoms that result are pain in legs, limp, atrophy, sensory deficits, incontinence, and bowel difficulties. They can have UTIs and enuresis. However, by the time symptoms arise, many are irreversible. Early surgery can prevent motor and sphincter deterioration in function and can partially reverse already-acquired defects. Fetal surgery may be possible in the future.

 

Thus, it is important to examine and palpate a newborn’s back carefully for cutaneous back lesions, as this is sometimes the only manifestation of a neural tube defect. Serious consequences can be prevented with early identification.

 

Quick case about a pseudotail: Benton TJ, Cheema R, Nirgiotis JG. A newborn with a skin lesion on the back. J Fam Pract Jan 2007; 56(1):E1-E3.\

Especially brief, helpful article: Drolet, Cutaneous signs of neural tube dysraphism

 

All references

Ackerman L. Spinal congenital Dermal Sinuses: A 30 Year Experience. Pediatrics Sept 2003

Albright AL, Gartner JC, and Wiener ES.  Lumbar cutaneous hemangiomas as indicators of tethered spinal cords.  Pediatrics. Vol 83 No. 6 pp977-980 June 1989

Ben-Sira L, Ponger P, Miller E, et al. Low-risk lumbar skin stigmata in infants: the role of ultrasound screening. J Pediatr 2009; 155:864.

Benton TJ, Cheema R, Nirgiotis JG. A newborn with a skin lesion on the back. J Fam Pract Jan 2007; 56(1):E1-E3.

Douvoyiannis M, Goldman DL, Abbott IR 3rd, Litman N. Posterior fossa dermoid cyst with sinus tract and meningitis in a toddler. Pediatr Neurol 2008; 39:63.Drolet BA. Cutaneous signs of neural tube dysraphism. Pediatr Clin North Am 2000; 47:813.

Gibson PJ, Britton J, Hall DM, Hill CR. Lumbosacral skin markers and identification of occult spinal dysraphism in neonates. Acta Paediatr 1995; 84:208.

Kriss Botoo l, and Moore C. et al. Neural Tube Defects NEJM Nov. 11, 1999. Vol 341 No 20. pp 1509-1519 Vesna, Kriss Timothy, Desai Nirmala, Warf Benjamin.  Occult Dysraphism in the Infant.  Clinical Pediatrics Dec. 1995

Kriss VM, Desai NS. Occult spinal dysraphism in neonates: assessment of high-risk cutaneous stigmata on sonography. AJR Am J Roentgenol 1998; 171:1687.

Robinson AJ, Russell S, Rimmer S. The value of ultrasonic examination of the lumbar spine in infants with specific reference to cutaneous markers of occult spinal dysraphism. Clin Radiol 2005; 60:72.

Sneineh AK, Gabos PG, Keller MS, Bowen JR. Ultrasonography of the spine in neonates and young infants with a sacral skin dimple. J Pediatr Orthop 2002; 22:761.

Weprin and Oakes Cocygeal Pits Pediatics  Electronic pages e69 May 2000

Zywicke,H and Roxxelle C.  Sacral Dimples.  Pediatrics in Review March 2011

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