Outpatient Adolescent Surgical Problems

A significant percentage of adolescent referrals to a pediatric surgeon are caused by problems that result in outpatient or in- office surgical intervention. A few of these lesions, such as hidradenitis suppurativa, pilonidal cysts, and complications of body piercing or tattooing, are seen with the onset of puberty. Medical and surgical management of these problems are reviewed. Pigmented cutaneous and subcutaneous lesions and their management also are reviewed.

Hemorrhoids

Hemorrhoids affect 4% of the population, and approximately 33% of those affected need treatment for symptoms. Hemorrhoidal disease is more frequent among Caucasians, especially those of higher socioeconomic status, and individuals who live in rural areas. Hemorrhoids are seen with increased frequency in patients with portal hypertension [1] and inflammatory bowel disease [2].

Squamous epithelium covers external hemorrhoids, and cutaneous nerves innervate them. Internal hemorrhoids develop above the dentate line, have columnar epithelium, and have no sensory innervation, thus abrogating pain. The mucosal suspensory ligaments anchor internal hemorrhoids to the underlying muscle that become lax to allow prolapse, particularly with the less elastic tissue of the older patient. Hemorrhoids have three cushions positioned in the left lateral, right posterior and right anterior areas of the anal canal.

Straining with defecation because of small caliber stools from low-fiber diets causes hemorrhoidal enlargement. Patients with hemorrhoids have a high anal canal resting tone that lowers after operation. In fact, botulinum toxin has been used to decrease the spasm within the internal sphincter and decrease pain after hemorrhoid surgery [3].

Many adolescents with perianal symptoms are referred for possible hemorrhoids. Hemorrhoids are symptomatic when they become engorged, inflamed, thrombosed, or prolapsed. Internal hemorrhoids cause bleeding and prolapse, leading to perianal sphincter spasm. Pain is relieved by reduction of hemorrhoidal tissue. Acute pain also happens with incarceration and necrosis. External hemorrhoidal thrombosis causes intense cutaneous pain. Pain peaks at 48 to 72 hours, and then improves as the thrombus organizes. Pain remits as the thrombosis involutes over 1 to 2 weeks. The stretched anoderm may persist as skin tags. The tags potentially can cause trouble with hygiene by inhibiting adequate cleansing by mechanical interference. The clotted inflammatory mass can erode the skin to cause bleeding. Hemorrhoidal bleeding is arterial, not venous. Pruritus ani results when mucus and stool are delivered onto the perianal tissue with prolapse.

Adequate visualization requires gentle spreading of the buttocks. The best position is in left lateral decubitus with knees toward chest. Five percent lidocaine ointment to the anal area minimizes distress during the examination. The examiner should note the presence of redundant tissue, skin tags, prolapse, fissures, fistulae, infection, mucoid discharge, or blood (Fig. 1). The underlying rectal tone should be elicited. Digital examination may reveal polyps, masses, occult blood, and indurated or ulcerated areas. Internal hemorrhoids are usually not palpable, as they are soft vascular structures.

Internal hemorrhoids are seen and graded best by anoscopy. Grade I hemorrhoids protrude in the anal canal to cause minimal bleeding or may be asymptomatic but do not prolapse. Grade II hemorrhoids prolapse with straining or defecating then reduce spontaneously afterwards. Grade III hemorrhoids require manual replacement, and grade IV hemorrhoids are permanently prolapsed. Flexible sigmoidoscopy is performed to exclude proximal disease. Physicians at The University of Calgary, Alberta, Canada [4] found all serious lesions with bleeding lay within 60 cm of the anus, which is within the reach of the fiberoptic sigmoidoscope. They felt that sigmoidoscopy is an appropriate initial investigation. Conversely, Baylor University Medical Center [5] doctors warn of potential misdiagnosis using only flexible sigmoidoscopy for bright red blood per rectum. Ten percent of adult patients with bright red hematochezia had lesions that were more proximal in location.

Fig. 1. Prolapsed hemorrhoid in a 13-year-old boy.

Conservative treatment usually alleviates symptoms. These measures include increasing fiber and liquid intake, decreasing straining, and minimizing time spent sitting on the toilet. Ice often relieves the pain of acute thrombosis. In the office setting, acute thromboses maybe managed within the first 48 to 72 hours after onset of symptoms. One percent lidocaine with epinephrine is used for local analgesia. An elliptical incision is used to excise the vein with overlying skin. The incision should not extend beyond the anal verge. A pressure dressing is applied that the patient can remove after several hours. Those who present late with decreasing pain should be left alone. Usually, hemorrhoids resolve spontaneously.

Treatment of internal hemorrhoids does not require anesthetic because of lack of cutaneous innervation. Nonoperative measures should be the initial treatment for grade I and grade II hemorrhoids unresponsive to conservative treatment. These techniques, such as rubber band ligation, have a recurrence rate of 30% to 50%. Linares [6] compared the method of rubber band ligation with infrared photocoagulation. He found no significant differences regarding the effectiveness between the two treatment modalities for the treatment of grade I or II hemorrhoids, but ligation was more effective for grade III and IV hemorrhoids. Perez [7] studied the effectiveness of rubber band ligation in symptomatic hemorrhoids and factors related to relapse. He studied 232 patients who underwent placement of one to three bands per session and had up to three sessions per patient. The treatment was effective in grades I and II (100% and 97.4%), but effectiveness decreased for grade III and grade IV (69.8% and 0%, respectively). Most relapses (87%) occurred within the first 24 months and were related to the presence of preoperative prolapse. Other available techniques to treat low-grade bleeding hemorrhoids include radiofrequency coagulation [8]. This modality has not been used frequently in the United States.

After nonoperative treatment, the patient goes home with instructions for bed rest for several hours, sitz baths three times a day, stool softeners, and topical or systemic analgesics. The patient should return for wound check in 48 to 72 hours.

Indications for hemorrhoid surgery include nonsurgical treatment failure, repeated acute events, hygiene trouble from skin tags, or bleeding from internal hemorrhoids, Grade III and IV presentations with severe symptoms, and additional anorectal conditions requiring operation. An outpatient operation with proper anesthetic care results in good function in most patients [9]. The number of hospital hemorrhoidectomies is declining; 37 hemorrhoidectomies per 100,000 people were performed in 1987. A transanally inserted circular stapler used for treatment of symptomatic prolapsing hemorrhoids leads to no manometric or symptomatic change in anal sphincter function. The most common complication of hemorrhoidectomy is anal stricture, which occurs in 4% of patients approximately 6 weeks after surgery. Other problems occur less than 5% of the time and include bleeding, infection, recurrence, nonhealing wounds, and fistula formation.

Rectal prolapse

The etiology of rectal prolapse is unknown, but it often is associated with long-standing constipation, chronic straining during defecation, weak anal sphincter, redundant rectosigmoid, and mental or neural impairment. Some surgeons believe that rectal prolapse is a sliding hernia through a defect in the pelvic fascia. Others think that it starts as a circumferential internal intussusception of the rectum, which then progresses to full-thickness rectal prolapse with time and straining. Mucosal prolapse happens when connective tissue to the rectal mucosa stretches, permitting tissue to prolapse through the anus.

There are three different types of rectal prolapse: full- thickness rectal prolapse, mucosal prolapse, and internal prolapse. The treatment of these three entities differs. Full-thickness rectal prolapse involves the protrusion of the full thickness of the rectal wall through the anus. This is the most common entity. In mucosal prolapse, the rectal mucosa protrudes from the anus. Internal prolapse may be a full thickness or partial rectal wall disorder; however, the prolapsed tissue does not pass beyond the anal canal and does not pass through the anus.

Patients with rectal prolapse present with a mass protruding through the anus. The prolapse initially occurs only after stooling. The mass usually retracts when the patient stands up. The disease may progress until the rectum requires manual replacement or becomes incarcerated so the patient cannot restore the rectum to the normal position. Evaluation of rectal prolapse in the office requires a detailed history, including stooling patterns, presence of diarrhea or constipation, dietary habits, and history of neurologic or muscular impairment. A careful anal and digital rectal examination is mandatory to rule out rectal masses. Often, it is helpful to ask the patient to strain in a seated position. If the prolapse is not elicited with this maneuver, adminis\tration of a phosphate enema may help make the diagnosis. The protruding mass has a beehive appearance (Fig. 2). A contrast enema should be performed to rule out masses or polyps and usually shows a redundant colon.

Fig. 2. Prolapse in a 13-year-old girl with mental retardation. Note the beehive appearance.

Management of prolapse is dependent on its presentation. The first-line treatment for simple prolapse is manual reduction using gentle continuous pressure. After reduction, the patient should be instructed on the importance of a high-fiber diet, drinking adequate fluids, and lessening time on the toilet seat. Treatment with bulking agents, stool softeners, or suppositories may be helpful. Incarcerated rectal prolapse is rare. A perianal block with local anesthetic followed by placing 3% saline-soaked gauze to decrease swelling usually allows reduction. Inverting the middle of the prolapse with a sponge wrapped finger may be helpful. Operative resections may be necessary for irreducible prolapse or questionably viable bowel. Symptoms associated with prolapse vary. Pain may occur. Incontinence occurs, because the protruding rectum stretches the anus, and the trapped mucosa secretes excessive mucus that may feel like colonic contents.

Patients who fail first-line therapy may need surgical intervention. There are many surgical procedures described in children and adolescents. Linear cauterization or sclerotherapy with various agents is beneficial to most children [10]. A mechanical bowel preparation and perioperative intravenous antibiotics are used. A Thiersch wire consists of a circumferential subcutaneously placed wire or other nonabsorbable materials around the anal opening. The anal lumen is restricted to allow soft stool to pass, but not prolapsed tissue. Complications include obstruction, fecal impaction, and fistula from erosion of the wire. In a Ripstein procedure, a nonabsorbable material is wrapped part way around the rectum. This wrap is sutured to the presacral fascia under tension, suspending the redundant rectum within the pelvis. The Frykman- Goldberg procedure combines anterior rectosigmoid resection and rectopexy; this is considered a good option for those children who have significant constipation. The redundant sigmoid colon is removed, and the descending colon is anastomosed to the proximal rectum. The lateral ligaments or the rectal fascia are fastened to the presacral fascia with tension. In the Delorme mucosal sleeve resection, a circumferential incision through the mucosa of the prolapsed rectum near the dentate line initiates removal of the mucosa from the prolapsed rectum to the apex. The suture reefs the denuded circular muscle, and the mucosa is approximated. In the Altmeier perineal rectosigmoidectomy, the excision is full thickness. All redundant bowel is pulled down, the bowel transected and anastomosed to the anus. A laparoscopic approach using mesh suspension of the rectum to the presacral fascia has been described [11].

Pilonidal disease

Hodges coined the term pilonidal (“pilus” meaning hair and “nidal” meaning nest) in 1880. At operation, only half of patients have a hair nest. Pilonidal disease occurs because of widening hair follicles in the skin ingesting cutaneous debris producing inflammation. Resultant swelling occludes the orifice, and the continually expanding purulence bursts into the surrounding tissue, causing folliculitis. Prepubertal presentations do not occur for pilonidal disease, as a mature pilosebaceous apparatus is requisite for evolution of the process. The sex hormones cause thickening of the secretions and distension of the pilosebaceous glands with keratin.

Presentations range from asymptomatic pits in the natal fold to watering pot multiple abscesses. Hairs in the skin surrounding pilonidal opening tend to keep the wound matted and dirty. There are reports of malignant change to squamous and verrucous carcinoma [12].

Pilonidal disease occurs in 26 per 100,000 people, and is twice more common in men than in women. The average age at presentation was 21 years for men and 19 for women. Patients usually have 2 years of disease history before referral for treatment. Most are hirsute. Thirty-eight percent of patients have a family history. Caucasians have the disease more frequently. Fifty percent of patients have normal body weight, and 37% are overweight. Obesity is not an important factor in the etiology of pilonidal sinus [13], although patients with a high body mass index have increased risk of recurrence after surgery. Patients with repeated local trauma and occupations that require prolonged sitting have a higher prevalence of having pilonidal disease. Preoperative cultures do not influence the complication rate, because bacterial isolates from infected wounds are mostly aerobes. Siaphylococcus uureus is the most common bacteria cultured. The most common anaerobes are Bacteroides species.

Eighty percent of presentations are exacerbations of chronic disease. Physical findings are midline edema and pits with signs of infection. An endoanal pilonidal sinus may affect the perianal skin or the skin of the anal verge. Mildly symptomatic cysts need hygiene measures, including careful washing, drying, and regular depilatory mechanisms.

Most surgeons opt to excise all pilonidal sinus tracks down to the sacrococcygeal periosteal level. This is performed when the sinuses are not infected. Excision of the sinus tracts during an acute infection is impossible to accomplish. For patients who present with pain and discharge, an incision lateral to the midline to drain the abscess, curette the hair and granulation tissue, and remove the infected material usually are required [14]. Some surgeons have emphasized that a lateral incision is needed for abscess drainage in the acutely infected patient. The wound is left to heal secondarily using dressing changes or wound-VAC (Kinetic Concepts Incorporated, San Antonio) therapy [15]. Sinus tract excision is performed after the wound has healed completely to avoid further episodes of reinfection.

Oncel et al [16] has found sinus excision technique requires a shorter operation time, hospital stay, and off-work period than excision and marsupialization in the treatment of limited, chronic pilonidal disease. Others have confirmed this finding in adolescents.

There is a documented recurrence rate of 9% to 27%. Operations for recurrent disease include advancement flaps, rotational flaps, marsupialization, grafting, and z-plasty. The Karydakis procedure removes the sinuses en bloc with an ellipse of the involved skin [17]. A full-thickness flap is mobilized for primary wound closure. The recurrence rate after Karydakis is 1.3%.

Hidradenitis suppurativa

Hidradenitis suppurativa (HS) is a suppurative folliculitis of apocrine sweat gland-bearing skin of the perianal, periumbilical, axillary, and genital areas, producing abscesses, sinuses, or mucopurulent discharge. The condition occurs when apocrine gland follicular epithelium becomes blocked by perspiration and cannot drain normally because of incomplete gland development. Perspiration and bacteria extrude into surrounding tissue, causing subcutaneous comedo-like follicular occlusion, inflammation, and infection. Apocrine glands occlude with periodic acid Schiff-positive extracellular polysaccharide substance, which may originate from Staphylococcus epidermidis. Many bacteria have been associated with infected HS.

In the United States, the prevalence of HS appears to be 1% to 2% in the general population. HS does not present until apocrine glands are triggered by a surge in sex hormones at puberty. There is a familial HS with an autosomal dominant inheritance. There is an increased incidence in females, in African American patients, and individuals with HLA-A1 and HLA-B8. Hirsutism and obesity also predisposing factors. Flare-ups have been associated with menstruation, which may be explained by the effects of androgens and estrogen on apocrine sweat glands. A hot, humid environment creates a milieu favorable the development of HS. Hidradenitis may be observed in association with arthritis, Crohn’s disease, Down syndrome, Graves’ disease or Hashimoto’s myroiditis, herpes simplex virus, irritable bowel syndrome, and Sjgren’s syndrome. Arthropathy associated with HS may present with asymmetric pauciarticular arthritis to a symmetric polyarthritis or polyarthralgia syndrome.

Patients present with painful, firm, and nodular lesions in areas where apocrine glands are found. The nodules may drain spontaneously. Regional lymphadenopathy is characteristically absent. Nodules often heal within a month, but may recur several times a year. Perianal HS may be difficult to distinguish from cutaneous manifestations of Crohn’s disease, which may coexist with perianal HS in 39% of patients. Anal fistula formation is common in perineal HS. In severe cases, the patient may have new lesions form as the older condition heals. Excessive heat, perspiration, and obesity seem to aggravate the condition. Remissions may last months or years [18].

First-line treatment consists of enhanced hygiene measures, instructions to wear loose-fitting clothes, and stop smoking, and lose weight for obese patients [19]. When infection occurs, drainage aids the resolution of the acute inflammation. The drainage incisions should parallel the skin folds. Injections of povidone- iodine into the draining fistula may enhance the recognition of the subcutaneous fibrotic tissue and its complete removal. Cellulitis, fever, or toxicity mandates short-course antibiotic use. Topical clindamycin and systemic tetracycline have been used with success [20]. Other adjunctive therapies to decrease inflammation in HS include the use of retinoids (acetretin) [21], intralesional injection of triamcinolone acetonide suspension [19], and antiandrogen cyproterone acetate combined with ethinyloestradiol [22\]. Patients should be warned of the potential existence of metachronous disease.

Operative excision, with wide margins and excision of the subcutaneous tissue down to the deep fascia is the preferred treatment for chronic or recurrent disease [18]. Coverage of the wound may be achieved by secondary intention, split thickness skin grafts, or by musculocutaneous flaps. The carbon dioxide (CO2) laser procedure has been described patients with severe perianal HS to ablate lesions in layers until all macroscopically abnormal tissue is removed. Musculocutaneous flaps are used for procedures to reconstruct areas of large resection. Skin grafting is not recommended for closure of inguinoperineal disease. There is a recurrence rate of 2.5% after wide surgical excision. Up to 90% of patients with HS may continue to have symptomatic disease years after initial presentation. There is a 3% prevalence of squamous cell carcinoma among patients with long-standing perianal HS [23].

Nail problems

Ingrowing toenails (onychocryptosis) occur in the early to mid- adolescent period in great numbers. The male-to-female prevalence is 3:1. It tends to occur during periods of rapid foot growth when shoes tend to get too tight (Fig. 3). A precipitating event is usually cutting the nail at an angle to the sulcus. Subsequent growth causes a spicule of the nail, usually in the lateral sulcus, to penetrate the skin. This spicule introduces bacteria and infection into the surrounding skin. The foreign body response to the nail produces a paronychia, with formation of purulent discharge and granulation tissue. Ingrown toenails initially are treated with the use of open-toed or wider footwear, cutting the nail straight across with avoidance of the sulci, elevation of the advancing nail edge, and placement of a cotton wisp to maintain nail elevation. Soaking the affected foot for 10 to 15 minutes in warm, soapy water or an Epsom salt solution may also help. When these measures fail, a partial unguectomy may be performed. A digital block is performed and allows the physician to lift the nail near the sulcus to the proximal nail fold. Granulation tissue is removed, and the lateral nail sulcus is removed to provide a surface level with the nail matrix. Direct pressure and application of silver nitrate control bleeding. The patient is instructed to wear open-toed shoes, elevate the foot for several days, and soak the foot for 10 to 15 minutes starting on the day after surgery. Matricectomy or complete excision of the viable nail matrix with loss of the nail plate is reserved for the ingrown nail, which is refractory to usual treatment. Sometimes, the nail bed is cauterized using electrocautery or chemically with phenol (0.8%). Phenol is caustic and is left in place only for 20 to 30 seconds. It needs to be deactivated using isopropyl alcohol.

Fig. 3. Ingrowing great toenail in a 15-year-old girl. Note the medial sulcus of the nail is very curved. The lateral sulcus is configured similarly with overlying granulation tissue.

Paronychia, an inflammation of the periungual area, can start because of trauma or an ingrowing toenail. Onychomycosis also may predispose to the infection. Use warm soaks and antibiotic therapy directed toward skin flora for 7 to 10 days. Silver sulfadiazine is usually effective as a local agent.

Onychomycosis is a fungal infection of the fingernails or the toenails. It occurs in 2% to 13% of the population. It affects men more than women and is more common in adults. About 3% of children have Onychomycosis. Onychomycosis may be caused by Candida species, dermatophytes, or nondermatophyte molds. Candida infection tends to infect the surrounding soft tissue of the nail or paronychia and then invade the nail itself. In contrast, dermatophyte infections infect the nail or nail bed. Candida infection is seen most commonly in the fingernails, but dermatophyte infections are more commonly found in the toenails. Onychomycosis is divided into distal, lateral subungual Onychomycosis (OM) (DSO), white superficial OM (WSO), proximal subungual OM (PSO), endonychomycosis OM (EO), or several combinations. DSO presents as a whiteto-brown thick and opaque nail, with nail bed hyperkeratosis and erosion. EO has a white discoloration of the nail without hyperkeratosis or erosion. WSO has small, white patches on the surface of the pitted, friable nail plate. PSO has a white proximal nail fold. Total dystrophic OM involves the whole nail and imparts a dense, deep tan appearance to the nail; this is the end stage of any type of Onychomycosis.

Risk factors for Onychomycosis include exercise, poor health, tight nonporous shoes, diabetes, immune compromise, family history, advanced age, local trauma, and warm climate. A laboratory diagnosis must confirm Onychomycosis before initiating therapy. Nail clippings are tested using a potassium hydroxide (KOH) in dimethyl sulfoxide to identify the fungi. The rate of recurrence remains high, even with newer agents. The topical agents such as the imidazoles and the allylamines are adjunctive to oral therapy. Antifungal agents such as itraconazole and terbinafine have greater effectiveness because of early nail penetration. The success rate with terbinafine therapy is 35% to 50%, and with itraconazole therapy, the success rate is 25% to 40% regardless of dosing schedules. The use of appropriate footwear and avoidance of high-exposure areas like health clubs can help with better long-term results [24,25].

Piercing and tattoos

Piercing and tattoos have been used to alter human appearance for thousands of years. These have become increasing popular among young Americans recently, however. An adornment is inserted into earlobes, eyebrows, navels, tongues, lips, eyebrows, nipples and genitals using a clamp-like device. The most common problems encountered with piercings involve infections, imbedded foreign bodies, and puncture enlargement. Most infections are caused by Staphylococcus species and usually are treated successfully with oral antibiotics and removal of the foreign body. Removal of an imbedded adornment may be performed with local analgesia with or without sedation. Enlarged local stomas occur when the rings or studs are too large or heavy. Removal of the jewelry may cause some closure. For ears with enlarged holes, an operative procedure using rotation flaps may be necessary. Piercing also may result in keloid formation. The management of keloids is discussed later in this article.

A tattoo results from repeated injections of pigments into the dermis. Superficial bacterial infections occur less that 5% of the time. Nonsterile technique or the use of inadequately sterilized needles or dyes may transport more serious infections such as tuberculosis, syphilis, leprosy, tetanus, human papilloma virus and hepatitis C. Hepatitis C is present in 3.5% of people with no tattoos and in about 33% of the people with tattoos. Superficial bacterial infections usually are treated with antibiotics.

In many states, a commercial parlor may not tattoo or pierce a person younger that 18 years of age without consent from the individual’s guardian unless a new tattoo is used to cover a preexisting tattoo that contains obscenities, drug inferences, or gang-related markings. Despite increasingly complex and stringent state laws, multiple problems continue to occur with piercings and tattoos. Adolescents frequently have friends perform tattooing or piercing or perform the process themselves.

Adolescents with tattoos may seek surgical consultation for tattoo removal. Complete removal of all pigment without scarring is virtually unattainable. Q-switched lasers have been used to disrupt the pigment in the tattoo. Bits of pigment may be absorbed into the skin. Blue and black dyes resolve easier than orange, yellow, and green. Dermabrasion uses a sander-like instrument in an anesthetized area to remove tattoo dyes. This usually leaves scarring and residual pigment. Salabrasion uses a salt slurry to remove tattoos. Surgical excision may be used to remove the tattoo completely or serially.

Tattoo pigments may cause pruritus and pain, which may be difficult to distinguish from an early infection [26]. This reaction usually occurs several weeks after injection and has a similar mechanism with poison ivy dermatitis. Red tattoo pigments are the most allergenic. Yellow or red dyes may contain cadmium that may cause phototoxic reactions of edema and erythema with light exposure. When a pink or purple nodule occurs within a tattoo, delayed hypersensitivity tattoo-induced pseudolymphoma should be suspected. The appearance of the lesion may be indistinguishable from cutaneous B-cell lymphoma; histologic analysis may be required. Reactions to dye may respond to steroids, but removal of the tattoo may be necessary.

Keloids

The first description of abnormal scar formation in the form of keloids was in the Smith papyrus in approximately 1700 BC. Hypertrophic scars and keloids occur only in people and in 10% of wounds. The gender prevalence is equal, but more women seek treatment. Keloids begin to occur in the preadolescent stage.

Hypertrophic scars are smooth, raised, and confined to the borders of the original wound [27,28]. The hypertrophy is apparent by 6 weeks after the creation of the wound, and then it tends to stabilize or regress. In contrast, keloids present up to 1 year after wound formation and proliferate well beyond the initial site. The anterior skin surfaces of the upper trunk, extremities, neck, and ears are particularly prone to abnormal scar formation. Delayed wound healing increases the risk of abnormal scar formation. Compared with normal scars, keloids have a thickened epidermal layer, more vessels, higher mesenchymal cell density, increased mucinous ground substance, and mast cells. Keloids have decreased alpha smooth muscle actin fibroblasts and collagen cross-linking. Collagen fibrils in \keloids are thicker, with unidirectional fibers arranged in a highly stressed orientation. Keloids have increased prolyl hydroxylase activity, collagen synthesis, type III collagen, chondroitin 4-sulfate, histamine, tumor necrosis factor a, Interferon (IFN)-β, interleukin (IL)-6, glycosaminoglycan content, and fibronectin compared with normal scars.

Adolescents present because of the mass, pruritus, and occasionally burning pain. In planning operations in keloid-prone patients, it is wise to avoid wounds in areas of enhanced risk and minimize skin tension upon closure. Removal of keloids automatically predisposes the child to keloid recurrence. Adjuncts to decrease recurrent keloid formation include silicone pressure dressings with steroid cream, compression devices custom-made for the patient (regional pressures of 5 to 25 mm Hg), and repeated intralesional steroid injections (40 mg/mL of triamcinolone acetonide). When used as an adjunct to excision, steroid injection elicits an 85% to 100% response [29]. Test doses should be given before surgery to monitor potential skin atrophy, hypopigmentation, telangiectasia, and deposition of white agent in the scar. Cryotherapy and laser therapy also have been described for keloid regression. High-risk lesions require follow-up for 1 year to treat recurrences as expediently as possible.

Investigational therapy to prevent keloid formation includes intralesional injection of INF-α, INF-β, and INF-γ immediately postoperatively. These have been shown to normalize the increased collagen synthesis and glycosaminoglycan production by keloid fibroblasts, resulting in a reduction in the size of the keloid by approximately 50% [30].

Nevi

Nevi consist of an intradermal collection of pigmented cells. Pigmented lesions are brought to the attention of the pediatric surgeon because of perceived change in their appearance or size. In adolescents, most pigmented lesions are usually benign. In addition, most small nevi may be removed an outpatient basis. A working knowledge of these lesions is mandatory, however, to determine the appropriate course of action upon clinical presentation.

Congenital nevocytic nevi (CNN) may be small or giant in size. The largest of these lesions are called giant pigmented nevus, giant hairy nevus, or bathing trunk nevus. More than half contain long dark hairs. CNN is present in 1% of newborns; giant CNN (more than 5% of body surface area) is seen in 1 in 200,000 newborns. CNN is inherited in autosomal dominant fashion. There is a 6% to 10% risk of developing melanoma in CNN, especially in the larger lesions. Melanoma may develop in half of giant CNN lesions by age 5 [31]. Serial excision of large skin lesions should start by 6 months of age [31]. Reconstructive techniques include skin grafting, tissue expansion, and local rotation. Removal of smaller lesions may be deferred until adolescence, because these nevi have a less than 1% rate of malignant transformation [32]. Other nonsurgical techniques described for CNN include phenol chemical peel, dermabrasion, ruby laser, and high-energy pulsed carbon dioxide (CO2). These are designed to eliminate some of the nevus cells, theoretically decreasing the melanoma risk.

Speckled lentiginous nevus consists of a patch of darkly pigmented maculae and papules. It is considered by some to be a variant of congenital melanocytic nevus. Associations include facial features, anorexia, cachexia, and eye and skin anomalies (FACES) syndrome, ichthyosis, Ebstein’s syndrome, epidermal nevi, nevus sebaceous, scleral pigmentation, segmental neurofibromatosis type I, adult-onset hearing loss, corneal snowflake dystrophy, and hypertrophy of the underlying pectoralis major muscle. The prevalence of speckled lentiginous nevi is about 1.5%. Several cases of cutaneous melanoma developing within a speckled lentiginous nevus have been reported. At birth, they are lightly colored caf au lait maculae, which then become variably colored. The lesions occur most commonly on the trunk. Lentigines, junctional, compound, dermal nevi, Spitz nevi, and blue nevi may arise within the background patch. Biopsy may be needed to define atypia. The entire lesion must be excised to prevent recurrence [33]. The use of laser treatment is controversial.

Epithelioid and spindle-cell nevus (juvenile melanoma or Spitz nevus) consists of pigmented lesions that appear histologically like melanoma, but pursue a benign course. The characteristic lesion is a dome-shaped, scaly, pink-to-red nodule. The face or legs are the usual sites of occurrence. About 5% of cases diagnosed clinically as melanomas are Spitz nevi. They occur almost exclusively in fair- skinned individuals; 70% of all cases are diagnosed by age 20. Initial growth is rapid, and then slows. Color changes, and, rarely, bleeding and pruritus occur. One percent of childhood melanocytic nevi are Spitz nevi, either arising de novo or within a melanocytic nevus. Excision into the subcutaneous fat with good margins and histopathologic confirmation of adequate margins is indicated. The histological distinction between Spitz nevi and melanomas is blurred in as many as 8% of cases. Clinical data are invaluable for in reaching the diagnosis of Spitz nevus with reasonable certainty. Spitz nevi are more common in young patients; the lesion is usually symmetric and well defined. Pathologically, Spitz nevi contain mature melanocytes and epithelial hyperplasia. Recurrences or close margins should be treated with re-excision [34].

Halo nevi are common melanocytic nevi, with an inflammatory infiltrate causing surrounding depigmentation. The average age of onset is 15 years. Halo nevi have only cosmetic significance, but may mimic melanoma (a changing mole). Nonuniform lesions or those with an eccentric papular component should be biopsied. Often, however, the central nevus will disappear with time [35].

Atypical moles or dysplastic nevi are usually bigger than common nevi. Dysplastic nevi may lack pigment uniformity and may be clinically indistinguishable from melanoma. There is a syndrome of autosomal dominant inheritance, where dysplastic nevi are found in first- and second-degree relatives [36]. Melanoma can arise in from dysplastic nevi, but the risk is uncertain. Patients with many dysplastic nevi, especially those with a family history of melanoma, have at least a 10% risk of developing melanoma in their lifetime. Dysplastic nevi lesions start during childhood and develop typical features by puberty. Typical lesions are 5 to 15 mm in diameter with irregular boarders, and ill defined. The colors range from tan to dark brown to pink. They often are found on the back, the chest, the buttocks, the breasts, and scalp. Patients with dysplastic nevi must shun excessive sun exposure and use a sunscreen with a high SPF. These adolescents should be taught to examine their lesions at least monthly. Scheduled skin examinations, preferably by a dermatologist, with baseline and surreal color photographs are mandatory. Rapid and characteristic changes should prompt excisional biopsy to exclude melanoma. Prophylactic excision of all dysplastic nevi is not recommended. A suspicious lesion should be removed with a narrow margin. If histologic evolution confirms melanoma, wider excision may be needed.

Blue rubber bleb nevus syndrome (BRBNS) is manifested by multiple cutaneous venous malformations and visceral lesions, usually involving the gastrointestinal (GI) tract. BRBNS lesions are multiple, navy, small-to-large compressible rubbery nodules. The nevi have tortuous, blood-filled ectatic vessels with a single layer of endothelium. Central nervous system (CNS), thyroid, parotid, eye, oral cavity, musculoskeletal, oral cavity, lung, kidney, liver, spleen, and bladder lesions have been reported. A few cases have had autosomal dominant inheritance. BRBNS is rare entity, with only 150 cases reported. The nevi are usually present at birth, but later onset has been reported. It has potential for serious or fatal bleeding from the visceral lesions, usually during early adulthood. CNS tumors can be fatal. When the bone is involved, there may be complaints of joint pain, impaired ambulation, skeletal distortion, pathologic fractures, bony overgrowth, spinal cord compression, and vertebral collapse. Other symptoms include epistaxis, hemoptysis, hematuria, or menorrhagia. Thrombocytopenia, disseminated intravascular coagulation, intussusception, volvulus, and bowel infarction have been associated with BRBNS [37]. With suspected bone or joint involvement, a skeletal survey usually detects abnormalities. Radiographs screen for extracutaneous lesions and look for disease in asymptomatic family members. Cutaneous lesions should be removed for changes in appearance, bleeding, or ulceration. Mechanisms for removal include electrodessication and curettage, liquid nitrogen, and excision. The CO2 laser has less scarring and lower recurrence. GI bleeding often can be treated with iron supplementation and occasional blood transfusions. Endoscopic coagulation, Nd:YAG photocoagulation, and removal of the most involved intestine are all options for recurrent disease.

Epidermal nevi are hamartomas of embryonal ectodermal origin, and they may be sebaceous, apocrine, eccrine, follicular, or keratinocytic [38]. When involvement of other organ systems occurs, il is called epidermal nevus syndrome. The linear sebaceous nevus (LSN), linear nevus comedonicus (NC), linear epidermal nevus (EEN), and inflammatory linear verrucous epidermal nevus are types of nevi with systemic associations. LEN syndrome patients may have mental retardation, seizures, movement disorders, or intracranial or intraspinal lipoma. LEN has a female-to-male ratio of 4:1 and a linear, persistent, pruritic plaque with frequent left leg involvement. Linear sebaceous nevus (of Jadassohn) has lesions on the scalp, neck, and forehead.Drug-resistant seizures are present in 75% of patients within the first 6 months of life. Multiple abnormalities are found on neuroimaging studies. The LSN skin manifestations in infancy are smooth yellow-orange plaques. These lesions darken and become hyperkeratotic as the child gets older. Benign or malignant tumors are noted in 20% to 30% of patients. In this population, syringocystadenoma papilliferum is the most frequent benign neoplasm, whereas basal cell carcinoma, squamous cell carcinoma, and keratoacanthoma are the most malignant. Topical calcipotriol, not approved for children younger than 12 years in the United States, may be effective in decreasing the lesion. The nevus can be removed if desired by the patient.

Connective tissue nevi are hamartomas [39]. Collagenomas have predominant collagen background; elastomas have increased elastin, and the nevus mucinosis has abnormal dermal glycosaminoglycan present. Buschke-Ollendorf syndrome has an increase in elastin messenger RNA. The lesions are rare. Collagenomas and elastomas generally present during the postpubertal period. In Buschke- Ollendorf syndrome, the skin changes may be delayed until adulthood. Nevus mucinosis may present at birth, during childhood, or in adolescence. Collagenomas have associations with multiple endocrine neoplasia type 1, Down syndrome, chronic myelocytic leukemia, syphilis, and encephalocraniocutaneous lipomatosis (ECCL). Familial cutaneous collagenoma occurs in early adolescence. A Shagreen patch is a type of collagenoma found with tuberous sclerosis. Flesh- colored plaques of variable size, usually on the lower back, can occur singly or asymmetrically in limited numbers. Operative excision usually is performed for cosmetic indications.

Nevus flammeus (NF) or port wine stain, a common congenital malformation of the superficial dermal blood vessels, is present at birth and grows with the child. Nevus flammeus occurs in 0.3% to 0.5% of newborns. Ninety percent are on the head and neck. The color transforms from pink at birth to red in adolescence to a deep plum in the fourth decade. The surface of the lesion thickens by adulthood. Glaucoma happens in around 10% of patients. NF may be found in association with other syndromes. Sturge-Weber syndrome involves vascular malformations involving the upper facial skin supplied by the ophthalmic branch of the trigeminal nerve, the ipsilateral leptomeninges, and the ipsilateral cerebral cortex. Klippel-Trenaunay syndrome consists of congenital varicosities and musculoskeletal overgrowth usually affecting a unilateral lower limb. Patients may present with unlimited varicose veins, venous thrombosis, pulmonary embolism, variceal bleeding, hypcrhidrosis in the area, and recurrent infections. If an arteriovenous malformation is present, the syndrome is termed Parkes-Weber syndrome. These fistulae are usually diffuse and difficult to manage. Complications include ulceration and severe lymphedema. In Cobb syndrome, the skin overlying the spine has angiomas; in addition, there may be vascular malformations in the adjacent meninges with potential for neurologic damage from compression of the spinal cord or nerves. Wyburn-Mason syndrome has facial NF associated with unilateral arteriovenous malformation of the retina and the intracranial optic pathway. NF over the lumbar spine is often an indicator of adjacent skeletal or neurological anomalies. There are more neural defects if skin tags, abnormal, lipomas, a distorted natal cleft, or a pit are present.

Tattooing with skin-colored pigment or opaque makeup camouflages NF without lasting effect. Flashlamp-pumped pulsed dye laser uses photothermolysis with ultrashort pulses of monochromatic yellow light (585 to 600 nm), tuned to the oxyhemoglobin spectrum, causing intravascular coagulation and destruction of some smaller vessels while limiting surrounding damage [40]. Some advocate treatment in the neonatal period, although the age for optimal response is not known. The best response usually is seen in small nonconfluent lesions the neck, torso, face, and hand; a lesser response is seen in the hand and arm.

Spider angiomas have a central arteriole with spokes. They present in up to 15% of normal children. Underlying conditions may accompany multiple angiomas. Laser ablation systems usually will eradicate the lesion, although some lesions will recur. Application of topical anesthetic allows most lesions to be removed painlessly.

Basal cell carcinoma (BCC) arises from basal cells, rarely metastasizes, but can grow to cause severe disfigurement. This neoplasm is found with increased frequency in male adults with sun exposure. Other associations are chemical contacts, radiation, burns, xeroderma pigmentosum, vaccinations, and tattoos. BCC can develop in teenagers [41]. The lesion is a pale pink nodule that may have central ulceration. Gorlin syndrome, an autosomal-dominant inherited condition, is characterized by multiple BCCs and commences at puberty. A biopsy of the affected area and the surrounding healthy tissue frequently is curative. Radiotherapy is contraindicated in young patients. Mohs surgery shaves off layers until there is histological confirmation of complete tumor eradication. Chemoprevention with systemic retinoids for those patients at high risk is under evaluation.

Malignant melanomas have proliferated over the last 20 years. They originate from melanocytes and are induced by sun exposure, especially acute, intense, or intermittent blistering sunburns. These lesions are relatively uncommon in the adolescent population. Five percent of skin cancers are melanomas, but they cause three times as many deaths each year as nonmelanoma skin cancers. The evaluation of pigmented lesions starts by asking questions about the duration of the lesion and changes in size, borders, and color. A history of severe or recurrent sunburns as a child should be elicited [42]. Examination of the mass should note the regularity of the border, uniformity of pigment presence of underlying mass, and history of ulceration or bleeding. The whole body, including the hands, feet, and scalp should be evaluated carefully for presence of other lesions. The draining lymph node basin is palpated for the presence of adenopathy. A suspicious lesion should undergo complete excisional biopsy with a 1 to 2 mm margin and some subcutaneous fat. If the lesion is large (greater than 5 cm) or is located in a cosmetically sensitive area, then an incisional biopsy from the most abnormal appealing area should be done. If the specimen is positive for melanoma, tumor-free margins are obtained based on the vertical thickness of the tumor: less than 1 mm deep lesion to 1 cm margin; 1 to 4 mm deep lesion to 2 cm margin; at least 4 mm in thickness to 2 cm margin. Patients with 1.1 to 2 mm thick lesions and those who are younger than 60 years seem to benefit from lymph node dissection. Sentinel node biopsy is standard and can give prognostic, diagnostic, and therapeutic information. Patients with greater than 4 mm deep lesions or regional lymph node involvement should be considered for adjuvant therapy. There is improvement in survival seen with high-dose IFN-α2b. Dacarbazine has a 10% to 15% response rate in advanced-stage melanoma. Immunotherapy with high- dose IL-2 also has improved survival rates in 10% of these patients.

Summary

There are several conditions for which adolescents seek surgical opinion. Many of these necessitate the performance of an office procedure. This article gave perspective on those relatively common and superficial lesions in an office setting.

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Eustace Stevers Golladay, MD

Department of Surgery, Division of Pediatric Surgery, The University of Michigan, 1500 East Medical Center Drive, F3970 Mott Children’s Hospital, Ann Arbor, MI 48109, USA

E-mail address: egoll@umich.edu

Copyright Hanley & Belfus, Inc. Oct 2004