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Case of the Week #519

Ho F (1), Cuillier F (2), Frade F (3)

Affiliations
1. Radiologist, private sector, 97400 Saint-Denis, Reunion Island, France.
2. Department of Obstetrics, Felix Guyon Hospital, Reunion Island, France.
3. Department of Pediatric Surgery, Felix Guyon Hospital, 97400 Saint-Denis, Reunion Island, France.

Posting Dates: June 4, 2020 - June 25, 2020

Case report:  This patient was referred to our hospital after abnormal 3rd trimester routine screening. First and second trimester ultrasound were normal. Down syndrome screening was negative. Previous personal, familial and obstetrical history is noncontributory. Our sonographic examination at 35 weeks of pregnancy revealed the following anomalies at the posterolateral side of the neck.

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We then performed fetal MR imaging.

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Answer

We present a case of Congenital Hemangioma.

Ultrasound revealed the following findings:

  • Images 1-4: Ultrasound exam revealed a solid, round, echogenic, homogenous soft tissue mass located on the posterolateral side of the neck, with clear margins, and peripheral vessels. One major high-flow arterial blood supply was also identified, probably a collateral of the external carotid artery.
  • Images 5-12: MRI showed also that the soft tissue mass had clear margins and was not locally invasive. Images 5-10 are T2 weighted, Image 11 is T1 weighted and Image 12 is T2* weighted. Flow-voids were depicted on the T2* image, which corresponded to flow-voids artifacts due to vascular flow.

In summary, we identified the following:

  • Mass developped in the 3rd trimester
  • Lateral location in the neck, far from midline and close to the ear
  • No aggressive features
  • Hypervascular, without cardiac failure (not shown)
  • Homogenous on both MRI and ultrasound

All these criteria led us to assume that this soft tissue mass was probably a congenital hemangioma, more specifically a rapidly involuting congenital hemangioma (RICH), which has a pseudotumoral appearance and is usually expected to involute spontaneously postnatally within the 1st year.  We performed a C-section at 38 weeks of pregnancy due to concerns for vaginal delivery with the neck mass. On postnatal exam, the mass’ diameter was 8cm and it began to slowly involute, measuring 5cm within the first postnatal month.

Congenital hemangiomas are a separate entity from Infantile hemangioma which appear later in infancy and then involute. Congenital hemangiomas are divided into 2 main subtypes:

  1. Rapidly Involuting Congenital hemangiomas (RICH): A pseudotumor differential diagnosis, often located on the scalp, on the neck next to the external ear, or on a limb next to a big joint such as the knee. Spontaneous involution is expected within 12-14 months. Some cases ulcerate and require percutaneous embolization. In some cases, a partial involution may occur and these hemangiomas are called Partially Involuting Congenital Hemangioma (PICH).
  2. Non Involuting Congenital hemangiomas (NICH): Masses are flat-shaped, telangiectatic, angioma-like. They are non-involuting and may increase in size as the child grows. Because they are flat, most of these masses are not identified on prenatal screening ultrasound.

References

Gorincour, G., Kokta, V., Rypens, F. et al. Imaging characteristics of two subtypes of congenital hemangiomas: rapidly involuting congenital hemangiomas and non-involuting congenital hemangiomas. Pediatr Radiol. 2005 Dec;35(12):1178-85.

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