Chapter 2. Conditions Mistaken for Child Abuse and Sexual Abuse

Clinical Summary

Mongolian spots (also referred to as blue-gray macule of infancy) are the most frequently encountered birthmarks in neonates. Melanin-containing melanocytes in the dermis are present (migrational arrest) and the distinctive blue color, characteristic of dermal melanin, occurs as a result of the Tyndall effect (when light strikes the surface of the lesion, red wavelengths of light are absorbed and blue wavelengths are reflected back from the brown melanin pigment from the dermis). More than 95% of African American and 80% of Asian infants are born with Mongolian spots. The skin lesion is flat (macular), slate-gray, bluish-gray or brown, and consists of poorly circumscribed, single or multiple lesions ranging in size from a few millimeters to several centimeters. The most common location is the sacrum and buttocks (90%), but lesions may occur anywhere, including the back, shoulders, or flank. Mongolian spots fade gradually and are resolved by age 5 to 6 in about 96% of cases. Differential diagnosis of Mongolian spots includes accidental or inflicted injury and other forms of dermal melanocytosis (eg, nevus of Ito or Ota).

Emergency Department Treatment and Disposition

Mongolian spots do not lead to any symptoms and require no treatment. Family needs to be reassured about the benign nature of these lesions.

Pearls

1. Mongolian spots can be mistaken for ecchymosis resulting from inflicted injuries. Mongolian spots are nontender unlike ecchymosis, which may be tender.

2. An ecchymotic skin lesion undergoes sequential color changes and resolves within a few days, whereas Mongolian spots do not undergo similar color changes and fade spontaneously over a period of years.

Clinical Summary

Folk healing practices are used by various cultures to treat illness. Coin rubbing (also known as Cao Gio, scratch the wind, or coining) is practiced among Southeast Asians (Vietnamese and Cambodians) when a child is sick. Warm oil (eg, tiger balm or mentholated oil) is applied over the affected region of the body, and then it is vigorously rubbed in with the edge of a coin. This is an attempt to rid the body of “ill wind” to reduce fever and chills. Cupping (ventosa) is used in some Eastern European, Latin American, and Russian cultures to reduce congestions. Alcohol is applied to the inner rim of the cupping glass and ignited with cotton soaked in alcohol. The cup is applied to the skin after the flame is extinguished. As it cools, a vacuum forms in the cup causing an ecchymotic lesion at the site. Spooning (spoon rubbing or quat sha) is used among the Chinese to relieve pain and headache. The skin is scratched with a porcelain spoon until ecchymotic lesions appear. Moxibustion is also practiced among the Chinese. It is a form of acupuncture. Burning sticks of incense, yarn, cigarettes, or cones of the herb Artemisia are used to make small circular burns on the skin at therapeutic points. Maquas is used by Arabs, Jews, and Bedouins. Hot metal spits or coals are applied to areas of disease or over a traditional “draining point” to produce burns. Caida de mollera (fallen fontanelle) is used by Mexicans and Hispanics. An attempt is made to elevate the fontanelle in infants who are dehydrated by holding the infant upside-down. Retinal hemorrhages may be seen with this practice and it resembles shaken impact syndrome.

Emergency Department Treatment and Disposition

These practices are not followed to injure the child, but rather are performed with the belief that they will help the child heal or recover from an illness. Ignorance of these folk healing practices on part of the medical team results in allegations of physical abuse, and trust between the medical team and the family may be irrevocably damaged. Consultation with a child abuse specialist may help in difficult cases. Educate parents about the injurious nature of these practices and suggest alternative approaches to treating illness.

Pearls

1. Folk healing practices (also known as pseudo-battering) may be mistaken for inflicted injuries from child abuse.

2. Physicians should become familiar with folk healing practices in their community.

Clinical Summary

Cold panniculitis is an inflammation of subcutaneous fat after prolonged exposure to cold or prolonged application of a cold object to any area of the skin (eg, ice packs applied to the face of an infant to control supraventricular tachycardia or ice packs applied to the lower extremities after vaccination). It is believed to occur solely because of the inherent properties of infant body fat with a higher percentage of saturated fatty acids (compared with older children and adults) and an increased propensity to solidify with prolonged exposure to a cold object. The degree of fat necrosis is inversely related to the age of the patient. Popsicle panniculitis is caused by sucking on ice, seen especially in infants who while sucking on the popsicles or ice cubes do not move them around in the mouth (as done by adults) keeping the cold object in contact with buccal fat for a prolonged period.

Popsicle panniculitis is commonly seen in the summer. Typically there are no systemic signs like fever or leukocytosis. The skin lesion is red to purplish indurated, discrete nodules or plaques with mild tenderness and sometimes itching. Lesions are seen in the perioral area (adjacent to the corners of the mouth) and may be unilateral or bilateral. Panniculitis lesions resolve spontaneously in 2 to 3 weeks without scarring. Differential diagnosis includes child abuse, subcutaneous fat necrosis from other etiology (eg, hypercalcemia, trauma), buccal cellulites, giant urticaria, contact dermatitis, or frostbite.

Emergency Department Treatment and Disposition

Cold panniculitis is self-limiting and does not require specific therapy except reassuring caregivers. Recurrence is common and parents must be educated about the condition. Dermatology consultation and a skin biopsy (nonspecific lobular adipocyte necrosis at the dermal–epidermal junction with a surrounding mixed inflammatory infiltrate seen) may aid in doubtful cases.

Pearls

1. A history of skin exposure to a cold object followed by a skin lesion supports the diagnosis of cold panniculitis. (Important: Panniculitis arises within hours to 1 to 2 days after the exposure to a cold object).

2. Popsicle panniculitis lesions due to sucking on ice are seen adjacent to the corners of the infant’s mouth.

Clinical Summary

Nevus of Ota and Ito arise from dermal melanocytes and are present at birth in about 50% of cases. A second peak occurs during puberty. The skin lesion is a light to dark brown, blue-black, or bluish-gray macule with a mottled or specked appearance. It increases in intensity and size with time and persists for life. They are more common in Asians (about 75% of cases; most commonly found in the Japanese), in African Americans, and in girls. Nevi of Ota occur on the face, forehead, zygomatic region, periorbital area, cheek, nose, or eye. Ipsilateral eye involvement (ocular melanosis) is commonly seen in moderate to severe cases and may involve the sclera (most common), conjunctiva, cornea, iris, retina, and optic nerve. Other mucous membranes and tissues (ear canal, tympanic membrane, pharynx, hard palate, nasal mucosa, buccal mucosa) may be rarely involved. Nevi of Ito occur on the supraclavicular region, shoulder, upper arm, and neck. Differential diagnosis of these nevi includes postinflammatory hyperpigmentation, café-au-lait spot, or Mongolian spots.

Emergency Department Treatment and Disposition

The diagnosis is made by typical clinical appearance. These nevi do not require any intervention in the ED. Patient can be referred to a dermatologist for evaluation and therapies that may include laser surgery or masking with cosmetics. If ocular melanosis is present, glaucoma may occur (melanocytes in the ciliary body of the anterior chamber may impede normal flow of fluid), and patients also need periodic funduscopic examinations by an ophthalmologist.

Pearls

1. Nevi of Ota and Ito may be mistaken for ecchymoses due to inflicted child abuse injuries. However, these nevi are nontender unlike ecchymoses, which may be tender.

2. An ecchymotic lesion undergoes sequential color changes and resolves within a few days. These nevi do not undergo similar color changes and are permanent lesions.

3. These nevi are unilateral, present in a specific anatomic location and have a speckled or mottled appearance unlike Mongolian spots, which are uniform in appearance and fades spontaneously over the years.

Clinical Summary

A port-wine stain (PWS), also known as nevus flammeus, is a congenital vascular malformation of dermal capillaries present at birth. Most commonly they are seen on the face or neck but may occur anywhere. The lesion is pink to purple, varied in size and shape, and sharply circumscribed. Large lesions follow a dermatomal distribution and rarely cross the midline. During infancy and childhood the lesion is macular; as age increases, it may become darker in color, papular or nodular, causing disfigurement. Elevated skin lesions may bleed spontaneously.

PWS may occur as an isolated birthmark (majority of patients) or be associated with a syndrome, including Sturge-Weber syndrome (PWS in the distribution of the V1-V2 branch of the trigeminal nerve, intracranial calcifications, seizures, glaucoma, etc) and Klippel-Trenaunay syndrome (PWS over an extremity, hemangiomas, varicosities, and limb hypertrophy of the involved extremity). Differential diagnosis of PWS includes capillary hemangioma and other vascular malformations (eg, salmon patch).

Emergency Department Treatment and Disposition

PWS is diagnosed clinically and does not require any intervention in the ED. Refer the patient to a dermatologist for evaluation for laser therapy. Radiologic studies (as clinically indicated) may be ordered to evaluate patients with Sturge-Weber syndrome presenting to the ED with seizures and may include CT scan and/or MRI. Such patients will need appropriate referrals to neurologist and ophthalmologist.

Pearls

1. PWS can be confused with inflicted bruises from child abuse.

2. An ecchymotic skin lesion undergoes sequential color changes and resolves within a few days. PWS does not undergo similar color changes; however, PWS may become darker in color and more nodular over the years.

3. PWS does not regress spontaneously and persists for a lifetime.

Clinical Summary

Phytophotodermatitis (PPD) is an acute phototoxic eruption following contact with certain plants, fruits, or vegetables containing photosensitizing compounds and exposure to sunlight. Examples of plants causing phototoxic reactions include limes, lemons, celery, parsnip, fig, fennel, parsley, dill, mangoes, carrots, and meadow grass. PPD occurs only with contact with a photosensitizing furocoumarin (psoralens) compound that readily penetrates the epidermis and subsequent exposure to UV radiation (wavelengths greater than 320 nm). PPD is common among food handlers (eg, salad makers and grocery workers) and the most common compounds are limes and lemons used in mixing drinks or making lemonade. A history of helping parents to make such drinks or spilling juice on the body during outdoor activities may be present. In temperate countries, cases are often seen among children playing outdoors during summer, when psoralens are most abundant in wild and garden plants.

Berloque dermatitis refers to dermatitis that results from application of a psoralen-containing perfume (eg, oil of bergamot), followed by UV exposure. The patient develops a rash at the exact areas where the perfume was applied.

Acute presentation of PPD after inflammation can be a mild reaction with erythema or a severe reaction that includes tingling of exposed skin and erythema followed by edema and vesiculation (after 24 hours; similar to severe sunburn). Bullous formation lasting for days may occur. Presentation after resolution of inflammation includes desquamation followed by hyperpigmentation. Configuration of lesions may be puzzling or unusual patterns like streaks (eg, from brushing against a plant), drip marks or haphazard lines (eg, from spilling celery juice), hand prints (eg, child touched with hands soaked with lemon juice), or crisscrossing linear streaks of erythema, vesicles, and bullae (eg, due to meadow grass rubbing on the skin). The lesions of PPD are sharply limited to areas of sun exposure, at the sites of contact (eg, mouth, face, arms, legs) and seen during hot and humid sunny days. Ecchymoses from inflicted bruises due to child abuse is an important differential diagnosis.

Emergency Department Treatment and Disposition

Symptomatic relief may be required for acute presentations, including baths with colloidal oatmeal (eg, Aveeno), emollients (eg, Aquaphor or Vaseline), and topical steroids. Systemic therapy as indicated may include analgesics, antihistamines (eg, hydroxyzine), and systemic steroids (eg, prednisone for 5-14 days). Patients presenting with hyperpigmentation do not require any treatment and it will fade over a period of weeks to months.

Pearls

1. Phytophotodermatitis is an example of photo-irritant contact dermatitis.

2. Commonest produce containing the offending compounds are limes and lemons used in mixing drinks or in making lemonade.

3. Phytophotodermatitis may be easily overlooked when a patient presents with erythema and vesicles following contact with plants or foods thought by parents to be irrelevant. Diagnosis can be easily made by taking a thorough history.

4. Phytophotodermatitis may be mistaken for child abuse because of its unusual patterns of cutaneous lesions.

Clinical Summary

“Raccoon” eye refers to periorbital ecchymosis and results from subgaleal bleeding, bleeding from a fracture site in the anterior portion of the base of skull (basilar skull fracture) or from a blow to the forehead. In an upright child, bleeding from the subgaleal space or forehead leaks ventrally into the facial soft tissues alongside of the nose and into the periorbital space. Darkening of the soft-tissue swelling and eyes (ecchymosis) are seen about 1 to 3 days after the trauma because of degradation of blood products. Because of the loose connection of the eyelid skin and underlying subcutaneous tissues, dramatic ecchymosis can be seen with mild blunt trauma. The initial subgaleal hematoma may be masked by the child’s hair, while a forehead hematoma may be seen or palpated. Raccoon eyes from basilar skull fracture are usually seen 4 to 6 hours after the traumatic event. The other signs of basilar skull fracture that may be seen are retroauricular ecchymosis (Battle’s sign), hemotympanum, cerebrospinal leaks (otorrhea or rhinorrhea) or VII nerve palsy. Raccoon eye can be bilateral (eg, forehead hematoma, subgaleal bleeding, basilar skull fracture, LeFort II and III fractures) or unilateral or bilateral (eg, direct eye trauma). Other differential diagnosis of raccoon eye includes coagulopathy and metastatic neuroblastoma with orbital involvement (see Figure 8-4).

Emergency Department Treatment and Disposition

A raccoon eye due to accidental forehead hematoma or subgaleal bleeding is usually a benign finding and only requires reassurance. Neurosurgery consultation will be required for raccoon eye due to basilar skull fracture (see management of basilar skull fracture).

Pearl

1. Raccoon eye of accidental trauma can be mistaken for inflicted trauma from child abuse; however, the orbital rim and ecchymotic shiner is nontender in raccoon eye and there is evidence of earlier forehead trauma or basilar skull fracture.

Clinical Summary

Hair-tourniquet syndrome (HTS) occurs when fibers of hair or thread are wrapped around an appendage producing tissue necrosis and is frequently an accidental injury. A constricting hair or thread decreases lymphatic drainage, and lymphedema subsequently impedes venous drainage, leading to more edema and eventually preventing arterial flow to the appendage. This obstruction leads to necrosis and tissue loss if not promptly relieved.

Common sites of HTS include toes, fingers, penis, clitoris, or labia. Involvement of toes or fingers is seen in young infants (birth to 2 years). Penile-tourniquet syndrome is seen in infants up to 6 years, and clitoral-tourniquet syndrome is seen in older girls (8 years to adolescence). Symptoms may include inconsolable crying or irritability (young infants) and odd gait or genital pain (older children). The physical exam may show erythema and swelling of the involved area (early presentation) to gangrene and amputation (late presentation). Complications are variable depending on duration of strangulation and include amputation of the digit, clitoris, or penis and partial or complete transection of the urethra. Differential diagnosis of HTS include child abuse or self-inflicted (eg, older girls with clitoral-tourniquet syndrome).

Emergency Department Treatment and Disposition

As the encircling hair dries out, it shrinks, cutting through the skin and becoming enveloped, making diagnosis difficult. Prompt removal of the constricting hair or fibers is required to prevent tissue loss. Sometimes the end of the hair or fiber is visible and can be used to facilitate its removal; otherwise the wound needs exploration to dissect and remove all the strands. A meticulous hunt to remove every single strand is required before discharging the patient.

Pearls

1. Consider HTS in any infant presenting with irritability or inconsolable crying or swelling of an appendage including the penis or clitoris.

2. Even though most cases of HTS are unintentional, the possibility of child abuse must be considered, especially if there is a delay in seeking medical care.

3. Deliberate wrapping of hair around the penis to stop wetting has been reported to be a form of punishment used by some care-takers.

4. A cursory examination of digits or other appendages may fail to identify the problem in an infant presenting with irritability or inconsolable crying. A thorough physical examination of all digits (after removal of the socks and mittens) and genitalia is required.

Clinical Summary

Rickets is caused by undermineralization of the cartilaginous epiphyseal growth plate (present only in childhood), resulting in excessive accumulation of unmineralized matrix (osteoid). The normal product of serum Ca × PO4 concentration (each measured in mg/dL) is 40; rickets occurs when the Ca × PO4 product drops below 30. Nutritional vitamin D deficiency rickets (NR) results from a deficiency of vitamin D metabolites, including “Sunshine deficiency” (inadequate exposure to sunlight, or factors preventing UV light penetration like industrial pollution, darkly pigmented skin, abundant clothing), dietary vitamin D deficiency (eg, exclusive breast-feeding without vitamin D supplementation, strict vegan diet), and fat malabsorption (eg, celiac disease, extrahepatic biliary atresia).

Rachitic deformities that are seen on physical examination include bow-legs, prominent wrists and ankles, rachitic rosary, Harrison groove (weakened ribs pulled by muscles, producing flaring over the diaphragm), craniotabes, frontal bossing with craniosomatic disproportion, knock-knee (genu valgum), or kyphoscoliosis. Generalized muscular hypotonia, failure to thrive, hypocalcemic seizures or tetany (latent or manifest) are other features of NR.

Emergency Department Treatment and Disposition

Laboratory tests suggestive of NR include either low (early) or normal serum Ca (secondary hyperparathyroidism), invariably a low PO4, and an elevated alkaline phosphatase value. Liver enzymes, serum albumin, blood urea nitrogen, and creatinine values help in excluding underlying liver or kidney disease as a cause of rickets. Additional tests for confirmation of NR include an elevated serum parathyroid hormone (PTH) and a decreased serum calcidiol (25-hydroxycholecalciferol) values.

A single anteroposterior (AP) view of the knee in children <3 years of age (femur and tibia being the most rapidly growing bones in infants leading to accentuated rachitic changes) or a single AP view of the wrist in older children is obtained. Radiographic changes suggestive of rickets include cupping of metaphyses (concave deformity of the end of the long bone shaft, instead of the normal convex or flat appearance), fraying of metaphyses (indistinct, shaggy borders), widening of metaphyses, generalized osteopenia, thinning of the cortex, pseudofractures, and bowing deformities of long bones. Endocrine consultation may be requested, if diagnosis is uncertain.

Patients presenting with symptomatic or asymptomatic hypocalcemia require hospitalization for correction of hypocalcemia and initiation of vitamin D therapy. Asymptomatic normocalcemic patients can be referred to a primary care physician or endocrinologist for the treatment of rickets.

Pearls

1. Metaphyseal changes of rickets can be mistaken for skeletal injuries of abuse. Presence of rachitic changes on the physical examination and lack of evidence of abusive injuries will help to differentiate the two.

2. Lucent epiphyseal-metaphyseal junctions seen in rickets may resemble the “bucket-handle” fractures of child abuse. However, widening of the metaphyseal plates distinguishes rickets radiographically from abuse-related fractures.

Clinical Features

Osteogenesis imperfecta (OI) is a genetic disorder of connective tissue seen in all racial and ethnic groups. It results from an abnormal quantity or quality of type I collagen, a primary component of the extracellular matrix of the bone and skin. Seven clinical subtypes of OI (type I to VII) are seen with variable manifestations. Genetic inheritance is autosomal dominant in the majority of the subtypes. The triad of OI includes fragile bones, blue sclerae, and deafness (conductive hearing loss seen in adolescents and adults). Other features include easy bruising, fragile skin, growth retardation with short stature, defective dentinogenesis, hyperlaxity of skin and ligaments of joints (hands, feet, knees), and wormian bones (small, irregular bones seen along the cranial sutures) and spine changes (scoliosis, kyphosis, codfish vertebrae). Fractures in OI occur following a slight trauma or spontaneously in an active child.

Emergency Department Treatment and Disposition

Serum calcium, phosphorus and alkaline phosphatase values, and coagulation profile tests are normal in a child with OI. Radiographic features of OI include osteopenia, thin cortices, bowing, angulation of healed fractures and normal callus formation at the sites of recent fractures. Most fractures in OI are situated in the diaphysis. Consultation with a radiologist is valuable in a difficult case presenting with multiple fractures and when a diagnosis of child abuse cannot be excluded. Orthopedic consultation in the ED may be required depending on the type and location of the fractures. Goals of therapy include splinting or casting of fractures and maximizing comfort and function. Management of fractures in OI patients with moderate severity is no different from management of fractures in otherwise normal children. Protective orthotics may be needed in children with increased bone fragility. Osteotomies may be needed to straighten bone, and intramedullary rods may be inserted to maintain correct alignment and repair deformities arising both from fractures and from progressive bowing or bending of the skeleton. OI patients need a close follow-up by orthopedics and their primary care physicians. Multidisciplinary management with referrals as indicated to a dentist, a geneticist, an endocrinologist, and a social worker for family support are also needed. In doubtful cases, diagnosis of OI can be confirmed by collagen biochemical studies or a skin punch biopsy.

Pearls

1. Osteopenia is insufficient bone mass resulting from reduced bone production, increased breakdown of bone, or both. Osteoporosis is a syndrome resulting from osteopenia that can lead to increased bone fragility and susceptibility to fractures and skeletal deformities.

2. OI or “brittle bone disease” is the most common osteoporosis syndrome in childhood.

3. OI is less common than child abuse. Milder forms of OI often get misdiagnosed as child abuse. Multiple fractures with angular deformities of the long bones, wormian bones, osteopenia, dentinogenesis imperfecta, and a positive family history will help to differentiate the two.

4. A history incompatible with injury, the hallmark of abuse, may also be present in OI. Absence of other signs of abuse or fractures that occur in different environments point toward OI.

5. Spiral or transverse fractures of long bones are more common in OI than metaphyseal fractures; however, metaphyseal fractures resembling abuse are also seen.

Clinical Summary

Lichen sclerosus et atrophicus (LSA) is characterized by distinctive white lesions on the genitalia. It is predominantly seen in girls (F:M ratio 10:1) and in boys it is called balanitis xerotica obliterans. LSA occurs most commonly between 1 and 13 years of age. Most lesions appear spontaneously, but some may be induced by trauma. Borrelia burgdorferi or other similar strains may be involved in the development or as a trigger.

Pruritus, burning, constipation, dysuria, dyspareunia, vaginal discharge preceding vulvar lesions (girls), and phimosis and recurrent balanitis (boys) are common presenting symptoms. The skin lesion starts as ivory-colored shiny, indurated papules that become confluent irregular plaques, which may develop hemorrhagic bullae at their margins. Later lesions atrophy, becoming depressed plaques with a wrinkled surface. The location of lesion in girls is vulvar, perianal, and perineal skin (most common site). Atrophic plaques may lead to shrinkage of the labia and stenosis of the introitus. In boys, the lesion is on glans and undersurface of prepuce. Extragenital lesions appear as atrophic depigmented patches with cigarette paper–like wrinkling of the surface and sites include neck, axillae, trunk, periumbilical, and flexor surfaces of wrists and around eyes.

Differential diagnosis include vitiligo, postinflammatory hypopigmentation, sexual abuse, bacterial vulvovaginitis, and intertrigo.

Emergency Department Treatment and Disposition

Topical steroid cream is the initial treatment and patient needs to be referred to a dermatologist or a primary care physician for ongoing treatment and follow-ups. LSA usually resolves spontaneously without sequelae in about two-thirds of children around puberty; about one-third will have persistence of LSA into adulthood.

Pearls

1. LSA is one of the most common dermatitides mistaken for sexual abuse, especially when it presents with purpura of the vulva.

2. LSA lesions are atrophic, porcelain-white plaque around the anus and vulva forming a figure-8 or hourglass configuration. This most distinctive pattern is seen in prepubertal females as well as in adults.

3. Nearly all affected boys with LSA present with phimosis in a previously retractable foreskin with an atrophic depigmented plaque at the tip of the penis.

Clinical Summary

Perianal streptococcal dermatitis (PSD) is a superficial bacterial infection caused by group A β-hemolytic Streptococcus (GABHS) in the perianal area. PSD is also referred as perianal streptococcal cellulitis. Chronic PSD may present without any signs of cellulitis; thus, the preferred term is PSD. It primarily occurs in children between 6 months and 10 years of age. About 70% of cases involve boys. Intrafamily spread can occur (eg, family members bathing together). Siblings may be either asymptomatic with only GABHS-positive cultures, or may develop similar dermatitis.

Perianal dermatitis and pruritus are the most common presenting symptoms followed by painful defecation with rectal pain, perianal pain, constipation, blood-streaked stools, and anal discharge. Systemic symptoms are usually absent. During the acute phase (<6 weeks), the perianal area may show bright red erythema (without induration) that is tender and confluent from anus outward. Size varies from a few to several centimeters. During the chronic phase, patients may present with painful perirectal fissures, dried mucoid discharge leading to irritation and excoriation, proctocolitis, or bleeding with little or no erythema. Psoriasiform plaques with yellow peripheral crust may also be seen. Infection may involve penis or vulva.

Differential diagnosis includes sexual abuse, candidiasis, contact, atopic or seborrheic dermatitis, chronic diaper dermatitis, psoriasis, pinworm infestation, inflammatory bowel disease, or local trauma (heavy wiping).

PSD is diagnosed by its characteristic distribution and confirmed by a culture obtained from the perianal area. A specific request should be made of the laboratory to look for GABHS. Culture the index patient and family members as indicated. A moderate to heavy growth of GABHS will confirm the diagnosis (children with asymptomatic perianal colonization have light growth of GABHS). Direct antigen studies for GABHS may also help (sensitivity about 90%; false-negative results seen early in the course). A throat culture may also be positive for GABHS. Rarely perianal dermatitis may be caused by Staphylococcus aureus.

Emergency Department Treatment and Disposition

PSD should be treated with systemic antibiotic therapy augmented by topical therapy. Drug of choice is penicillin given either orally or parenterally. Amoxicillin may be used instead of penicillin (better-tasting preparation). Erythromycin or clindamycin is used for patients who are allergic to penicillin. These alternative antibiotic treatments may also be helpful for patients who have not responded to a course of penicillin, or who are infected with S aureus. Topical therapy is with mupirocin ointment. The patient needs to be followed by the primary care physician for monitoring of response to therapy and to document bacteriologic cure posttreatment. Recurrences can occur.

Pearls

1. PSD is a bright-red, sharply demarcated perianal dermatitis caused by GABHS.

2. PSD often gets mistakenly diagnosed as sexual abuse or contact or atopic dermatitis.

3. Patients with PPD are subjected to treatments for a variety of different diagnoses without success. Failures to respond to prior therapy with topical antifungal or corticosteroids or therapy for pinworms are important clues in suspecting PSD.

4. Undiagnosed and inappropriately treated patients with PSD develop perianal fissures, bleeding, pain during defecation, and constipation.

Clinical Summary

Labial adhesion is fusion of the labia minora, usually extending from an area immediately inferior to the clitoris to the fourchette. The extent of the adhesion is variable and is thickest posteriorly. Labial fusion and labial or vulvar agglutination or synechiae are synonyms commonly used. This is an acquired condition and occurs as a result of labial abrasion that culminates in medial surfaces of labia minor adhering to one another followed by epithelialization. Fusion occurs secondary to local inflammation in the presence of the hypoestrogenic state of a preadolescent child (inflamed/injured epithelium is more likely to fuse in a low-estrogen environment). Nonspecific vulvovaginitis, poor perineal hygiene, diaper rash, and use of harsh soaps are common contributory factors. Other etiologies include lichen sclerosus, atopic or seborrheic dermatitis, herpes or pinworm infection, or sexual abuse.

Labial adhesion occurs most commonly between 3 months and 6 years of age (peak: 13 to 23 months); however, it can occur at any age. It is rare in newborns (exposure to high levels of maternal estrogens) and in postpubertal premenopausal women. The majority of patients are asymptomatic and it is usually accidentally noted by a parent or by a physician during examination. Symptoms of urinary tract infection like dysuria and frequency may be present (related to pooling of urine in the vagina leading to recurrent vulvovaginitis). Other symptoms include urinary retention or altered urinary stream. Patients with labial adhesions have normal female genitalia; however, hymen and introitus are obscured because of adhesions. With complete adhesions, the urethra is usually not visualized. Urine may be seen dribbling at the anterior end (just posterior to the clitoris). Labial adhesions may also reoccur.

Differential diagnosis includes imperforate hymen, sexual abuse, ambiguous genitalia, congenital absence of the vagina, and scarring following female circumcision.

Emergency Department Treatment and Disposition

Spontaneous resolution (without therapy) occurs in the majority of children (>80%) over the course of a year. With endogenous estrogen production and the vaginal pH becoming more acidic at puberty, labial adhesions almost always resolve spontaneously. No treatment except reassurance is indicated for patients with asymptomatic adhesions. General measures include reassurance to the family about the presence of normal female genitalia, improving perineal hygiene (eg, regular changing of diapers and thorough wiping after each bowel movement from anterior to posterior), removal of irritants like caustic soaps, treatment of diaper dermatitis, loose-fitting cotton underwear, and sitz baths. Estrogen cream may be considered for patients with nearly complete adhesions with or without urinary symptoms or for patients with significant parental anxiety. Patients will need a follow-up with the primary care physician. A small quantity of estrogen cream is applied topically with gentle traction twice a day for 3 weeks, then at bedtime for 3 weeks. An additional few weeks of application of an inert ointment (eg, petroleum jelly) keeps the labia apart while healing is completed. Prolonged use of estrogen cream should be discouraged because of side effects (eg, breast enlargement and/or tenderness, vulvar pigmentation) and caregiver needs to be educated about this. If the patient presents with acute urinary symptoms, a gentle attempt to separate the labia with a Calgiswab can be made (only if adhesions appear to separate easily) after applying 5% lidocaine ointment or 2.5% prilocaine cream.

Labial adhesions can result from sexual abuse, but in the absence of other evidence, the presence of adhesions alone is not diagnostic of sexual abuse. Consider possibility of sexual abuse in girls (especially older) presenting with adhesions many years after the toilet training or without any prior history of adhesions or any predisposing skin condition or presenting with thick adhesions that are difficult to treat.

Pearls

1. A thin vertical raphe (line of adherence) in the midline where the labia are fused is pathognomonic for labial adhesions.

2. Labial adhesions should not be separated manually by force; it is painful and the resulting raw surfaces have a greater tendency to reagglutinate.

3. Patients with labial adhesions have normal female genitalia; however, hymen and introitus are obscured because of adhesions.

Clinical Summary

Urethral prolapse is an eversion of the distal urethral mucosa protruding through the urethral meatus and may be partial or complete. Predisposing factors include increased intra-abdominal pressure (eg, violent coughing, constipation) in a hypoestrogenic state and preceding trauma (eg, straddle injury or sexual abuse). Constriction of the prolapsed mucosa at the urethral meatus may lead to impairment of the venous blood flow with subsequent edema and erythema or purplish appearance of the mucosa. Thrombosis and necrosis of the mucosa may result if the process is not corrected.

Urethral prolapse is seen almost exclusively in premenarcheal girls, with a peak incidence between 4 and 10 years of age, and about 95% of cases are African Americans. The most common presenting symptom is painless bleeding or spotting on the underwear (90% of patients). Other symptoms may include dysuria and/or urinary frequency (due to urethral inflammation), difficulty voiding or urinary retention (prolapse may occlude urethral meatus) or perineal discomfort. Patient may also be completely asymptomatic and prolapse may be noted on routine examination.

Clinical signs of a prolapsed urethra include cherry-red doughnut or prolapsed cervix-like circular, nontender mass at the introitus. It may appear as a friable rosette of red or hemorrhagic tissue, may be ulcerated or gangrenous (necrotic) or infected. A centrally located urethral meatus may not be visible in the presence of severe edema or strangulation. Differential diagnosis of urethral prolapse includes hematoma of hymen from sexual abuse, straddle injury, hematometrocolpos, prolapsed ectopic ureterocele or urethral polyp, urethral cysts, condylomata acuminata, hemangioma, and sarcoma botryoides.

Emergency Department Treatment and Disposition

Urethral prolapse is a clinical diagnosis. Conservative therapy is used for mild cases without necrotic mucosa. This includes warm sitz baths and emollient cream. If indicated, topical antibacterial therapy or topical estrogen therapy (the distal urethra is estrogen dependent) twice a day for 10 to 14 days can be used. It usually resolves spontaneously or with the above therapy in 2 to 3 weeks. Urologic or surgical consultation is required for patients with necrotic mucosa or recurrent prolapse. Indications for surgery (excision and reapproximation of the mucosal edges or CO2 laser therapy) include prolapse with necrotic mucosa at the time of presentation, persistent prolapse with failed medical therapy, recurrent symptomatic prolapse, or urinary retention. Recurrences may be observed weeks to months after the initial resolution following medical therapy. Recurrence is uncommon after surgery.

Pearls

1. Urethral prolapse is the most common cause of apparent vaginal bleeding in premenarcheal girls.

2. Urethral prolapse presents as an interlabial mass and is the only lesion that has a circular mass of tissue surrounding the urethral meatus. If in doubt, catheterization of the bladder through the central dimple of the mass or observation of the child during voiding will aid in the diagnosis.

3. Clinical findings of urethral prolapse can be mistakenly attributed to sexual abuse, especially when prolapsed mucosa has become hemorrhagic and friable.

4. Urethral prolapse cannot be reduced manually.

Clinical Summary

Straddle injuries are the most common type of accidental injuries to the genitalia and rarely involve penetration. Soft tissues get crushed between the pubic bone (eg, pubic symphysis, ischiopubic ramus) and a hard object during impact, such as accidentally falling on a furniture arm, bed, bicycle crossbar, balance beam, fence, toilet seat, concrete wall, or playground equipment.

Straddle injuries are usually unilateral and involve the anterior portion of external genitalia in both girls and boys. Injuries to internal genital structures or the anus are rare (protected by soft tissues of the buttocks, bones of the pelvis, and labia in girls). The most common location in girls is the labia majora and labia minora. Other sites may include clitoral hood, clitoris, periurethral tissue, or posterior fourchette, but hymen or vagina are usually not involved (unless accidental penetrating injury). Typical injuries are linear abrasions, bruising, or hematoma of labia majora or minora. Small posterior fourchette tears may be seen. Swelling, pain, and bleeding are common symptoms. Findings in the boys include ecchymosis or minor laceration of scrotum and/or penis. Scrotal contents may be contused or crushed. Injury to anterior urethra is suggested by blood at the meatus, periurethral or perineal swelling, and difficulty voiding.

Differential diagnosis of straddle injuries includes self-inflicted injuries, which are exceedingly rare in children. Normal masturbation in girls is clitoral or labial and does not cause genital injury. Self-inserted foreign bodies also do not cause injuries to the hymen.

Emergency Department Treatment and Disposition

During the evaluation, other associated injuries including injury to the urethra must be excluded. Lacerations may be obscured because of swelling. Examination may be limited as a result of intense pain and fear, and the patient may need procedural sedation. If internal injuries cannot be excluded in girls, examination under anesthesia and evaluation by a pediatric surgeon or gynecologist is required. If clinically indicated, a scrotal ultrasound to assess testicular injuries and a retrograde urethrogram to assess urethral injuries (extravasation) may be ordered. Boys will need evaluation by urologist for urethral, testicular (eg, hematoma, rupture) or scrotal injuries (eg, lacerations extending through the dartos). Hematomas and superficial lacerations are managed with supportive care and resolution occurs spontaneously. Patient’s ability to void without any difficulty must be confirmed before the discharge. To minimize discomfort, patient can be advised to take sitz baths. Voiding in bath of warm water also may ease the dysuria. Local application of antibiotics may help in promoting healing. Child abuse specialist or child protective services may be consulted prior to discharge if the diagnosis is unclear.

Pearls

1. Straddle injuries are the most common type of accidental injuries involving the genitalia and may be difficult to differentiate from injuries due to sexual abuse. A plausible explanation and physical examination that supports the history are important in arriving at the correct diagnosis.

2. Suspect sexual abuse if there is a lack of correlation between history and clinical findings, evidence of injuries in other areas, genital injuries in nonambulatory children, extensive injuries, vaginal, perianal, rectal, or hymenal injuries without a clear history of penetrating trauma.