Infantile Perianal Pyramidal Protrusion with Coexisting Perineal and Perianal Hemangiomas: A Fortuitous Association or Incomplete PELVIS Syndrome?
A 3-month-old female child was seen by one of us (SV) in a multi-specialty hospital upon the request of the pediatrician. She was born after a normal full-term vaginal delivery and had no history of consanguinity in her parents. The child was thought to have hemorrhoids with a large hemangioma in the perineal area present since birth. Upon examination the child had a skin-colored perianal protrusion that was clearly located posterior to the anus []. There was in addition a large midline hemangioma that started from the gluteal cleft all the way to the medial root of the thigh skirting around the vulva []. The presence of inflammation around the pyramidal protrusion could not be assessed because of the presence of the large hemangioma. The parents said the child was born with a ‘red patch’ in the area of the hemangioma which evolved over the past 3 months into a hemangiomatous red plaque. The child also had a large hemangioma on the contralateral side of the face with involvement of the lower lip []. There were no neurological complaints. There were also small hemangiomas found on the ventral aspect of the left forearm and dorsum of the left foot. The hemangiomas were explained in detail to the parents and they decided not to give any oral corticosteroid for the perineal hemangiomas and rather wait for a year. A head and neck as well as spine X-ray was performed that did not show any abnormality. CT scan/MRI was refused by patient's parents due to cost factor. A referral was made to the pediatric surgeon for biopsy of the pyramidal lesion which they refused. A provisional diagnosis of large perineal hemangioma with a perianal skin tag was made. The large facial segmental hemangioma was thought to be Sturge-Weber syndrome with no clinical evidence of internal involvement. The patient was prescribed topical clobetasol cream under monthly supervision after which the lesion had flattened a little and showed a whitish area with pronounced flattening.