An Infant’s Isolated Lumbosacral Hemangioma: What Might It Signify?
A 2-month-old boy was referred to a neurology clinic for an isolated, superficial strawberry hemangioma over his lower back (Figure 1).
He had been born at 36 weeks of gestation and had been treated for group B streptococcal sepsis; otherwise, his perinatal history was unremarkable, and he was thriving.
Neurologic examination findings were completely normal for his age, and he had good tone and movement in all limbs. His age prevented assessment of bowel and bladder dysfunction, but he had a brisk anal reflex.
Lumbosacral ultrasonography and magnetic resonance imaging were performed, the results of which are shown in Figure 2 and Figure 3, respectively.
Why were these imaging studies ordered? What do they show?
(Answer and discussion on next page)
Answer: Occult Spinal Defect With Isolated Superficial Lumbosacral Hemangioma
A 2-month-old boy was referred to a neurology clinic for an isolated, superficial strawberry hemangioma over his lower back (Figure 1). He had been born at 36 weeks of gestation and had been treated for group B streptococcal sepsis; otherwise, his perinatal history was unremarkable, and he was thriving.
Neurologic examination findings were completely normal for his age, with good tone and movement in all limbs. His age prevented assessment of bowel and bladder dysfunction, but he had a brisk anal reflex.
Ultrasonography of the lumbosacral spine appeared to show an intraspinal lipoma at the level of S1 but with no evidence of tethering (Figure 2). Lumbosacral magnetic resonance imaging (MRI) showed tethered spinal cord and conus at the level of L5, in addition to the intraspinal lipoma at S1 (Figure 3).
The boy underwent surgery to release the tethered cord. The procedure was performed without complications, and the boy has been doing well, without neurologic deficits.
Discussion
Skin changes in the lumbosacral area in conjunction with other cutaneous alterations such as dermal sinus tracts, hair tufts, and subcutaneous lipomas are known to be associated with underlying spinal dysraphism.1,2 This is a case of a newborn with an isolated superficial hemangioma in the lumbosacral region and occult spinal cord lipoma and tethering.
Occult spinal dysraphism (OSD) refers to skin-covered lesions with no exposed neural tissue and is the most prevalent spinal axis malformation. OSD can cause distortion or partial absence of neural tissues and can lead to damage from compression or traction, which increases as the child grows.2 Patients with OSD can present with incontinence, deformity or weakness of the feet, impaired gait, and other difficulties, which appear late. They eventually may develop progressive neurologic deficits; thus, early prophylactic surgery is advised upon diagnosis of the condition.
Historically, cutaneous stigmata representative of OSD have included subcutaneous lipomas, dermal sinuses, tails, and tufts of hair or localized hypertrichosis.1,3 Researchers in one prospective study found that approximately 35% of patients with isolated lumbosacral infantile hemangiomas had concomitant spinal anomalies.4
Although ultrasonography is a quick and noninvasive tool for evaluation of possible OSD in a newborn, discordance has been reported in the interpretation of ultrasonography and MRI results. The sensitivity of ultrasonography for detecting spinal anomalies in this high-risk group reportedly is poor at approximately 50%.1,4 MRI is a noninvasive and nonirradiating examination that is now considered the first choice for the detection of OSD.4,5
Children with isolated cutaneous hemangiomas in the lumbar region may have occult spinal defects, although such defects are more common in combination with other cutaneous lesions. Moreover, these patients otherwise may be asymptomatic and neurologically normal. Early surgical intervention can reduce the risk of delayed or progressive loss of function.3,5
Apeksha Sathyaprasad, MD, is a third-year resident in the Department of Pediatrics at Lincoln Medical and Mental Health Center in the Bronx, New York.
Lisa Bouma, MD, is a third-year resident in the Department of Pediatrics at Lincoln Medical and Mental Health Center in the Bronx, New York.
Magda Mendez, MD, is the pediatric residency program director and an attending physician in the Department of Pediatrics at Lincoln Medical and Mental Health Center in the Bronx, New York.
Sergey Prokhorov, MD, is an attending physician and pediatric neurologist in the Department of Pediatric Neurology at Lincoln Medical and Mental Health Center in the Bronx, New York.
References
1. Tubbs RS, Wellons JC III, Iskandar BJ, Oakes WJ. Isolated flat capillary midline lumbosacral hemangiomas as indicators of occult spinal dysraphism. J Neurosurg. 2004;100(2 suppl pediatrics):86-89.
2. Liptak GS, Dosa NP. Myelomeningocele. Pediatr Rev. 2010;31(11):443-450.
3. Guggisberg D, Hadj-Rabia S, Viney C, et al. Skin markers of occult spinal dysraphism in children: a review of 54 cases. Arch Dermatol. 2004;140(9):1109-1115.
4. Drolet BA, Chamlin SL, Garzon MC, et al. Prospective study of spinal anomalies in children with infantile hemangiomas of the lumbosacral skin. J Pediatr. 2010;157(5):789-794.
5. Albright AL, Gartner JC, Wiener ES. Lumbar cutaneous hemangiomas as indicators of tethered spinal cords. Pediatrics. 1989;83(6):977-980.