Diane F. Merritt, MD

A 23-month-old girl was brought to the pediatrician's office by her mother who was concerned about "bulging down there." The child's mother reported that a "bump" had been present in the girl's diaper area since her birth and that it had been growing.

The child had a recent history of constipation. The mother had addressed this (with little success) by altering the toddler's diet. The child had no history of headaches, stomachaches, or nightmares and had not demonstrated any new or worrisome social behaviors. The child was happy and active, and she cooperated with the examination.

A smooth, sessile, rose-colored protrusion was present over the perineal region anterior to the child's anus. It did not pulsate or blanch with pressure, and it had a resilient, homogeneous texture. There did not appear to be any drainage of stool or pus from the lesion, and the rectal examination did not uncover any fissure or fistula. The lesion did not communicate with the vaginal vestibule or vagina.

Is this a normal finding--or does it point to sexual abuse?

This physical finding is known as infantile perianal pyramidal protrusion (IPPP).

 It is a normal variant of anogenital anatomy, most often found in young girls.

 The diagnosis can be made in early infancy through the preschool and early school years. The term "IPPP" was introduced in the medical literature within the past 10 years. It clearly describes findings previously classified as skin tags, or acrochordons, when describing prepubertal anogenital anatomy.

IPPP may be misinterpreted as a sign of sexual abuse when it is misdiagnosed as a condyloma or mistaken for healed trauma.

The cause of IPPP remains unclear. Most reporters of this finding have attributed it to an area of constitutional weakness of the perineum that either protrudes because of a congenital remnant of the urogenital septum or is aggravated by the pressure of chronic constipation. Three different variations of perianal protrusion have been proposed:

•Constitutional IPPP, which is present at birth and remains unchanged over years.

•Functional IPPP, which is related to constipation.

•Sentinel-type IPPP, which is associated with lichen sclerosus et atrophicus.

Regardless of type, IPPP lesions are sessile and have the same smooth texture and normal to rose-toned color. The majority of cases that have been described have occurred anterior to the anus overlying the perineal body, although a posterior location has been described in one series.

Most evaluations for IPPP are prompted simply by the discovery of an otherwise asymptomatic abnormality of the perianal region. Symptoms associated with IPPP may include itching, burning with urination, or swelling of an already discovered protrusion--as in this patient.

When the history includes constipation, the presence of an anal fissure is not uncommon.

 Itching and burning should prompt a careful search for signs of lichen sclerosus in the anogenital area as well as for the common hygiene challenges presented by the average female toddler.

Items in the differential diagnosis for IPPP include median raphe cysts, human papillomavirus (HPV) infection, and hemorrhoids. Close observation of the surface of the lesion should help differentiate among these diagnoses:

•Median raphe cysts look like a water blister.

•HPV lesions have a rough, speckled surface.

•External hemorrhoids have a different point of origin involving the anal verge, and they do not occur anterior or posterior to it.

Treatment of constipation has been shown to be helpful in some patients with functional IPPP. Many of the functional and sentinel-type cases self-resolve. This spectrum of outcome also helps to support the utility of the 3 subcategories of this finding.

The clinician can usually offer reassurance and some peace of mind to the parent of a child with IPPP. Perianal abnormalities may be present in sexually abused children; however, IPPP is a distinct clinical diagnosis of a normal variant of anogenital anatomy.

  Kayashima K, Kitoh M, Ono T. Infantile perianal pyramidal protrusion

 Konta R, Hashimoto I, Takahashi M, Tamai K. Infantile perineal protrusion: a statistical, clinical, and histopathologic study. 

 Merigou D, Labreze C, Lamireau T, et al. Infantile perianal pyramidal protrusion: a marker of constipation? 

 McCann J, Voris J, Simon M, Wells R. Perianal findings in prepubertal children selected for nonabuse: a descriptive study. 

 Patrizi A, Raone B, Neri I, D'Antuono A. Infantile perianal protrusion: 13 new cases. 

 Cruces MJ, De La Torre C, Losada A, et al. Infantile pyramidal protrusion as a manifestation of lichen sclerosus et atrophicus

  Fiorillo L. Therapy of pediatric genital diseases. 

 Fleet SL, Davis LS. Infantile perianal pyramidal protrusion: report of a case and review of the literature

Diane F. Merritt, MD

Articles

Perianal Finding--Sexual Abuse or Normal Variant?