ASSOCIATED CONDITIONS OF THE PREPUCE AND CIRCUMCISION
Gina M. Badalato, Marc Holden, Michael Whalen, and Grace Hyun
Phimosis describes a condition whereby the prepuce cannot be retracted over the glans due to a tight distal, preputial ring. Approximately 96% of newborn boys have physiologic phimosis, although by 3 years of age 90% of foreskins can be retracted over the glans without difficulty; by the age of 17, less than 1% of men have phimosis.1,2 Young boys often present with preputial adhesions in which the foreskin is adherent to the glans; however they do not have the distal constricting cicatrix of fibrotic tissue that defines true phimosis. In this setting, early forceful retraction of the foreskin is not recommended, because recurrent adhesions may form between the foreskin and denuded glandular tissue and propagate the formation of a cicatrix at the tip of the foreskin, thereby causing secondary phimosis.
When physiologic phimosis is present, it may hinder adequate penile hygiene, which may propagate balanitis or balanoposthitis, as well as repeated urinary tract infections; among adults, this condition may predispose patients to the transmission of sexually transmitted diseases and carcinoma of the penis.3-8 In terms of nonsurgical treatment options, the application of corticosteroid cream to the phimotic band 3 to 4 times per day has been shown to increase foreskin retractibility.9 Specifically, in a study of 70 boys aged 1 to 12 years, the application of betamethasone valerate 0.06% on this schedule resulted in a 81.3% response rate, as measured by an improvement in foreskin retraction.9 If phimosis recurs, a second course of topical steroids may be beneficial.10 However, in cases where steroid treatment has not been effective or in the setting of recurrent urinary or skin infections, circumcision is the next preferred option. _ENREF_3_ENREF_3_ENREF_3_ENREF_1
Inconspicuous Penile Disorders
The term inconspicuous penis refers to a group of conditions where the phallus appears to be diminutive in size.11 These conditions include webbed penis, concealed penis, and trapped penis, in which the penis is normal in size but partially or fully obscured by the overlay of adjacent adipose or skin tissue. In contrast, micropenis is the only condition within the rubric of inconspicuous penile disorders in which the penis is abnormally small.
While the ensuing section will focus on the phenomenology of concealed penis, it remains important to define the other entities in this family of disorders as a point of contradistinction. Webbed penis describes a condition, much as the name implies, in which the scrotal skin extends to form a web or flap of tissue on the ventrum of the penile shaft. On the contrary, trapped penis is an iatrogenic form of inconspicuous penis in which the phallus has become embedded in the suprapubic fat pad following circumcision. This presentation can occur following neonatal circumcision in the setting of significant scrotal enlargement (such as that from a hydrocele or hernia), after the circumcision of a child with a webbed penis, or in any other case where the phallus naturally retracts into the scrotum. Among these boys, if a circumcision is performed, the skin at the base of the shaft can form a cicatrix over the retracted phallus postoperatively. This group of patients may in turn be managed with a combination of topical steroid therapy, dilation of the cicatrix with a hemostat, or, in infants over 6 months of age, surgery similar to that which will be described for concealed penis cases.12 Lastly, micropenis refers to cases in which the penis size is 2.5 standard deviations below the mean size for a given age group.13
Concealed (Buried/Trapped) Penis
Concealed penis encompasses both congenital and acquired conditions whereby the phallus is retracted inward beneath the suprapubic fat pad and partially obscured by preputial skin.14 The congenital etiology of concealed penis is thought to result from (1) poor elasticity of dartos fascia, restricting its ability to move freely through deep layers of tissue, and (2) the lack of anchoring of the penile skin to deep fascia, which in turn limits extension.15 In older or obese children, anatomical factors such as a pronounced suprapubic fat pad or a large pannus may contribute to the aforementioned congenital anomalies to effectively obscure the phallus. Moreover, an unsuccessful circumcision may also serve to exacerbate this condition by causing penile entrapment or by allowing the concealed penis to recede further beneath surrounding tissue.
Patients with concealed penis usually present to a surgeon due to poor cosmesis or difficulty maintaining proper hygiene. In some cases, patients are prompted to seek further evaluation as a result of symptoms, including recurrent balanitis or spraying of the urinary stream, caused by ballooning of the foreskin with voiding. Last, patients may be referred with the erroneous request for a redo circumcision. On physical exam, a normal penile shaft should be palpable although the glans and shaft are not visualized above the skin level. If the phallus can be exposed simply by retracting the overlying skin, a repair is indicated at the surgeon’s discretion.
Historically, several techniques have been described to exhume a concealed penis.14,16-21 The fundamental aspects of repair involve removal of fibrotic bands on the dorsum of the penis, and unraveling the prepuce tissue to provide ventral shaft coverage. In order to help fixate the skin layer proximal to the phallus, the subcutaneous tissue on the dorsum of the penis should be tacked to the pubic fascia, and ventrally the scrotal subcutaneous tissue fixed to the base of the shaft. One technique describes a vertical incision of the median raphe, followed by complete penile degloving and fixation of the shaft base to prepubic fascia.20,22 The shaft skin is then affixed to the base of the penis with vertical mattress sutures. This technique avoids circumferential incision of the base of the penis and thereby reduces the incidence of postoperative lymphedema. In some cases, however, Z-plasty at the level of the penoscrotal junction or lateral penile shaft may be required to reconstitute an adequate skin flap.
Congenital penile torsion is a rotational defect of the penile shaft; it is a common anomaly, for which the exact incidence remains unknown. In children, the shaft is almost always rotated in the counterclockwise direction, and this condition may be associated with other congenital abnormalities such as hypospadias or dorsal hooding with no urethral defect. Although the developmental etiology for torsion is indeterminate, skin tethering and corporal disproportion, along with abnormal dartos fascial development have been named as possible contributing factors. The parents of affected children seek medical attention, usually after a circumcision is performed or the foreskin is retracted, because of concern about the cosmetic appearance and/ or future dysfunction. In fact, however, the defect has no physiologic of functional significance if the rotation is less than 60 to 90 degrees from the midline.
In most cases the median raphe spirals around the shaft, and, although the glans may be directed away from midline, the orientation of the erectile tissue is normal at the base of the shaft. In cases where there is no evidence of torsion or chordee related to corporal disproportion, the repair involves degloving the penis to realign the overlying skin and restore the raphe to the midline position; several techniques have been described in this regard. One approach, deemed the “dorsal dartos flap” repair, involves creating a forked or V-shaped flap of tissue with the dorsal aspect of the degloved shaft skin. One flap can then be brought around to the ventrum of the penile shaft at the appropriate length as a fulcrum to oppose the degree of torsion.23,24 Following degloving, another technique involves circumferentially realigning the tissue starting at the 12 o’clock position and moving laterally in a manner that counterbalances the torsion. The ventral flap of tissue that remains after the tissue has been realigned superolaterally, may then be excised.25 In cases where skin realignment is not sufficient, however, such as in the setting of torsion exceeding 90 degrees, the base of the penis must also be mobilized to remove dysgenic bands of tissue; maneuvers involving corporal plication and/ or suturing the tunica albuginea to the periosteum of the pubis may also be required.19,26,27
Circumcision—the surgical removal of preputial tissue—has been performed for thousands of years for a wide array of cultural, religious, and medical indications.28 Presently, common indications for circumcision include recurrent urinary tract infections and phimosis refractory to the conservative options discussed above. In addition, there are ancillary benefits to circumcision in the reduction of the risk of sexually transmitted infections and a reduced incidence of penile cancer (detailed below). However, the great majority of circumcisions are performed electively, for cosmesis or cultural reasons, and without urgent medical indication.
Male infants suffer from urinary tract infections more than females, and in a study by Ginsberg and McKracken, 95% of male patients with urinary tract infections in the first 3 months of life were uncircumcised.29 Meta-analysis data investigating the impact of circumcision on the incidence of primary and recurrent urinary tract infection (UTI) indicated circumcision reduced the risk of the development of UTI as compared to uncircumcised counterparts (OR = 0.13; 95% CI, 0.08 to 0.20; p<0.001). Nevertheless, among asymptomatic patients with no prior history of UTI, infection was rare enough that the complication rate of circumcision (conservatively estimated at 2.0%) outweighed the risk reduction seen in treated patients; routine circumcision for prophylaxis against a first UTI is hence not substantiated by the data. The protective effect of circumcision among patients with a history of symptomatic UTI, however, supports prophylactic circumcision among patients with symptomatic UTI in order to reduce the incidence of recurrent infection. In fact, the recurrence rate of UTI among all patients, estimated at 10%, reduced to 1.3% with circumcision, and an even greater benefit was seen among patients with high-grade vesicoureteral reflux who developed UTI.8 The mechanism of the protective effect of circumcision on is thought to be related to a change in bacterial colonization and the behavior of pathogens. Circumcision has been shown to greatly reduce colonization with uropathogenic organisms,30 as Fussell and colleagues reported that uropathogenic strains of bacteria adhered to the inner mucosal tissue of the prepuce but not the keratinized, outer epithelial surface.31
Lichen sclerosus et atrophicus refers to a condition traditionally known as balanitis xerotica obliterans, a chronic inflammatory process yielding edema and hyperkeratosis of affected tissue. Lichen sclerosus can lead to meatal stenosis in circumcised and uncircumcised patients. In uncircumcised patients, the lesion can be initially confined to the prepuce but may ultimately progress to involve adjacent tissues and cause stenosis or stricture. In such cases of chronic inflammatory lichen sclerosus confined to the prepuce of an uncircumcised patient, circumcision can be performed therapeutically.
While incidence remains low in the United States, penile cancer represents nearly 20% of male cancers in Africa and Southeast Asia. The foreskin is a major site of penile intraepithelial neoplasia and penile cancer; its removal therefore removes a locus for malignant transformation. Furthermore, phimosis is a risk factor for the development of penile cancer, and therefore circumcision is of great protective benefit to patients with respect to this predisposing factor. Schoen and colleagues studied a population-based sample and found that, of 89 patients with invasive penile cancer, 87 were uncircumcised, further supporting the protective nature of circumcision.32 In addition to the prophylactic benefit of circumcision, the procedure can be performed with curative intent in cases of penile squamous cell carcinoma in which the lesion is confined to the prepuce.
Especially among men with multiple sexual partners, circumcision reduces the risk of both oncogenic and non-oncogenic strains of human papillomavirus (HPV) in the patient and a reduction in the incidence of cervical cancer in their sexual partners.33 As HPV infection is thought to be responsible for a great proportion of cervical, vaginal, penile and anal cancers, the impact of circumcision on this area of public health is significant.
Circumcision as a means of reducing the risk of HIV infection was first suggested by Alcena and Fink in 1986.34 Since that time, many observational studies have reported that circumcision reduced the risk of infection across all populations, especially populations exhibiting high-risk behavior. Since 2005, three large randomized trials have been published that support these observations. A randomized, controlled trial by Auvert and colleagues in South Africa enrolled 3,274 men to either circumcision or observation. Over the course of a mean follow-up of 18.1 months, the authors observed an HIV incidence rate of 0.85 cases per 100 person-years among the intervention group and 2.1 cases per 100 person-years among controls, corresponding to a risk reduction of 60%.35 Bailey and colleagues enrolled 2,784 men in Kenya and, after an average follow-up of 24 months, found an incidence of 2.1% among the intervention group and 4.2% among controls; once corrected for participants seropositive at enrollment and non-adherence to intervention, the authors found a 60% reduction in HIV incidence with circumcision.36 Both trials were halted based on the findings at interim analysis. In 2007 Gray and colleagues reported the results of a trial in which 4,996 men in Uganda were randomized to circumcision or control.37 Following 24 months of follow-up, modified intention-to-treat analysis revealed an incidence of 0.66 cases per 100 person-years among the intervention group and 1.33 cases per 100 person years among controls, corresponding to a 51% rate reduction. Several mechanisms have been proposed to explain the reduction in disease transmission among circumcised patients. One explanation is that the less keratinized tissue of the uncircumcised penis is vulnerable to microabrasions that disrupt the epithelium and enable the entry or adherence of pathogens. Ulcerative disease seen secondary to collections within the preputial sac would also predispose to further disease. In addition, cellular factors have been proposed. The inner mucosal layer of the prepuce is rich in Langerhans cells, which densely express CCR5 and CXCR4 receptors that facilitate HIV entry, and therefore reduction in the area of tissue expressing these receptors is thought to reduce viral entry and subsequent infection.
The outer portion of the prepuce, acting as a sheath around the glans, consists generally of a keratinized epithelium. The inner mucosal tissue that lines the sheath and lies in contact with the glans contains a large population of Langerhans cells.38 Variations in the histology of different portions of the prepuce has been posited as the source of reduced UTI and sexually transmitted infections seen among circumcised patients.
In neonatal and younger patients, several devices are often used to facilitate the procedure. Among these, three are common: the Gomco clamp, the Mogen shield, and the Plastibell. The Gomco and Mogen devices facilitate sustained crush injury of the prepuce, followed by excision of the prepuce. Use of the Plastibell device involves placing a ligature between the device and the tissue, inducing tissue necrosis and eventual sloughing of the skin and device. The Mogen shield is often preferred for the relative simplicity of the device, shorter procedure length, and less operative pain.
In adults and older children, a sleeve circumcision is often preferred. With the foreskin retracted, an initial circumferential incision is marked and made just proximal to the coronal margin, leaving a small cuff of tissue proximal to the corona. The incision is made through the dartos fascia to the superficial Buck’s fascia. The foreskin is allowed to return to its anatomical position, and a second circumferential incision is marked at the coronal margin. The resulting sleeve of tissue between these incisions is removed, and following hemostasis the skin edges are reapproximated. Given the large size of vessels in patients of this age, bipolar cautery is often used for hemostasis rather than the compression of circumcision clamps used in neonates.
Complications and ContraindicationsIn the hospital setting, the perioperative complication rate of circumcision is reportedly as low as 0.2%, with the most common complications being minor bleeding and local infection.39 Serious complications are exceedingly rare. However, removal of an insufficient portion of foreskin or improper reapproximation can result in phimosis and associated problems. Contraindications to circumcision include prematurity, a bleeding disorder, ambiguous genitalia, hypospadias or other conditions whose correction might require foreskin tissue as part of a repair of reconstruction.
Anesthesia in Infant Patients
Attitudes towards analgesia and anesthesia during circumcision have changed dramatically in the last several decades. Prior to this change several reasons existed for the avoidance of this intervention – from poor familiarity with anesthetic techniques in this age group to the idea that intervention would itself be more painful than the result of such a minor procedure. Further understanding of the physiological responses attributable to pain have shown that the procedure is in fact painful and has also enabled the measurement of various interventions to determine relative efficacy at minimizing pain. In neonatal patients, a multimodal approach might include a sucrose pacifier, low room lighting, and pharmacologic anesthetic intervention to minimize patient discomfort. Pharmacologic methods include topical lidocaine/prilocaine preparations, dorsal penile nerve block (DPNB), and ring block, as well as acetaminophen for postoperative pain control. DPNB is administered via two injections at the 10 and 2 o’clock positions on the shaft of the penis to anesthetize the dorsal nerve. Ring block involves circumferential subcutaneous injections around the mid-shaft. Regardless of method, appropriate attention should be given to anesthesia and analgesia as standard of care for all patients undergoing circumcision.
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Image 0808: Penile torsion, approximately 30 degrees counter-clockwise.
Image 0815: Buried penis in an uncircumcised boy.
Image 0819: Pushing downward on the shaft exposes the buried phallus.
Image 0821: Following penile degloving, the base of the shaft is fixed to the prepubic fascia.
Image 0822: Lateral view of the aforementioned process.
Image 0824: Cosmetic appearance following surgery (lateral view).
Image 0825: Anterior view.
Image 0826: Inferior view.
Image 0827: Circumcision, sleeve technique. The foreskin has been removed, leaving a mucosal collar proximal to the glans.
Image 0830: Bovie electrocautery is used to attain adequate hemostasis at the skin edges and on the shaft.
Image 0832: Good hemostasis has been achieved. Now the skin edges are to be reapproximated using interrupted, chromic simple sutures.
Image 0838: Note the dressing: Bacitracin at the tip of the penis. Hemostatic gauze and coban are applied to the shaft and left in place for 24 hours if possible. It is important to ensure the dressing is not so tight as to obstruct glans circulation.