hypospadias john w. duckett, jr pediatrics in review€1989 ... · the incidence of hypospadias is...

DOI: 10.1542/pir.11-2-371989;11;37Pediatrics in Review

John W. Duckett, JrHypospadias

http://pedsinreview.aappublications.org/content/11/2/37the World Wide Web at:

The online version of this article, along with updated information and services, is located on

Print ISSN: 0191-9601. Village, Illinois, 60007. Copyright © 1989 by the American Academy of Pediatrics. All rights reserved.trademarked by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove

andpublication, it has been published continuously since 1979. Pediatrics in Review is owned, published, Pediatrics in Review is the official journal of the American Academy of Pediatrics. A monthly

at UNIV OF CHICAGO on May 16, 2013http://pedsinreview.aappublications.org/Downloaded from

http://pedsinreview.aappublications.org/content/11/2/37

http://pedsinreview.aappublications.org/

The questions below should help

focus the reading of this article.

1. What genetic factors affect the oc-

currence of hypospadias?

2. What conditions are associatedwith hypospadias of varying de-grees?

3. What should the pediatrician ad-vise the parents concerning the opti-mal age for operative correction ofhypospadias?

EDUCATIONAL OBJECTIVE

6. The pediatrician should haveknowledge to make an appropriateevaluation of a newborn Infant withpenoscrotal hypospadlas, a bifidscrotum, one palpable gonad anda small phallus, with the ability todifferentiate among Idiopathic hy-pospadias, adrenogenftal syn-drome, mixed gonadal dysgene-sis, and androgen resistance syn-drome, with the ability to seekappropriate endocrinologic, ge-netic, or radiologic consultations(Topics, 89/90).

There is a familialoccurrence of 8% to 14% iffathers or siblings have the

condition.

* Professor of Urology, University of Pennsyl-

vania, Director of Pediatric Urology, Children’s

Hospital of Philadelphia.

Hypospad ias

pediatrics in review #{149}vol. 11 no. 2 august 1989 PIR 37

John W. Duckett, Jr, MD*

Hypospadias results in various de-grees of deficiency of the urethra,corpus spongiosum, and corporacavernosa. The fibrous tissue thatcauses ventral curvature replacesBuck fascia and dartos fascia. Theskin on the ventral surface may bethin; the prepuce is deficient ventrallyand forms a dorsal hood over theglans.

As a rule, the location of the mea-tus does not cause significant ob-structive urinary symptoms, althoughventral deflection of the stream ofurine commonly occurs. When themeatus is more proximal, the streamflows straight downward or back-ward, requiring the patients to urinatead modum feminarum. Uncorrected,the curvature of the penis is likely tocause painful erections and result insevere psychologic consequences.

INCIDENCE/GENETICS

The incidence of hypospadias isabout 1 in every 300 male children. Ifminor degrees of hypospadias are

Hypospadias is a commonanomaly (1 in 125 male

births).

included, this incidence may be ashigh as 1 in 125 male births. If 1 848500 male births occur in the United

States, we should see 6200 hypo-spadias cases each year.

The risk of occurrence in an infantis 8% if his father has hypospadiasand 14% if a sibling has the condition;if two members of the same familyhave hypospadias, the risk is about21%.

The cause of hypospadias is stillunknown. It is probably multifactonal(polygenic), given the familial mci-dence. The condition is more corn-mon in the white than the black pop-ulation and more common in Italiansand Jews. The incidence of hypo-spadias among monozygotic twins is8.5 times greater than in singletons.This might be explained by the de-mand of two male fetuses on theplacental production of human cho-rionic gonadotrophin in phase Ill or-

ganogenesis. It is possible that, withmonozygotic twins, one placentamay not be able to meet the humanchononic gonadotrophin require-ments for masculinization of two malefetuses. A similar explanation mayimplicate the condition of placentalinsufficiency. There is a greater inci-dence of hypospadias in winter con-ceptions, perhaps reflecting the ef-fect of daylight on maternal and,hence, fetal pituitary function.

CLASSIFICATION

Anatomic classification of hypo-spadias recognizes the level of themeatus without taking into accountcurvature. The first-, second-, andthird-degree classification representsthe location of the meatus along theshaft of the penis and down to thepenoscrotal junction. Other classifi-cations identify the location of themeatus, such as glanular, penile, orperineal. We prefer the classificationlocating the meatus after curvaturehas been released (Fig 1).

EMBRYOLOGY

There are three separate portionsof the male urethra. The portionabove the wolffian duct openingforms the urethra down to and includ-ing the verumontanum, the utncle,and urogenital sinus. The second an-lage forms the segment extendingfrom the verumontanum to the baseof the glans. The glans segment isformed separately. If the genital foldsfail to fuse in the midline, the preputialtissues do not develop ventrally. Con-sequently, they form in an excessivemanner dorsally. Because the distalpenile urethra ends normally at thesubcoronal area and the glans chan-nel from the tip fuses downwardly,this is the most common anomaly,making up 65% to 70% of cases.

The abnormal ventral curvature islabeled chordee, in accordance withthe concept of bowstnnging. Thechordee tissue actually fans out onthe ventrum of the penis and probablyrepresents the rudimentary vestige ofthe urethral plate. In some cases,there may be differential growth dif-ferences that create abnormal ventralurethral plate deficiencies.

ASSOCIATED ANOMALIES

Undescended testis and inguinalhernia are the most common anom-alies associated with hypospadias,with undescended testis and inguinalhernia each present in 9% of cases.



Fig 1. Classification of hypospadias.

Hypospadias

PIR 38 pediatrics in review #{149}vol. 11 no. 2 august 1989

In posterior hypospadias, this mci-dence for undescended testis ishigher (32%), and with middle andanterior hypospadias, it is 6% and5%, respectively. Inguinal hernia oc-curs with 1 7% of posterior hypo-spadias, 8% middle and 7% anterior.

A utricle (utriculus masculinus) isfound in the posterior urethra as adiverticulum of persistent urogenitalsinus origin. The total incidence ofutricles is about 11% to 14%,whereas a higher percentage is re-flected in the more severe cases. Inmost patients, this anomaly poses noassociated complications except forinfection. Utricles usually do not needto be removed.

It is not surprising that urinary tractanomalies are infrequently associ-ated because the external genitaliaare formed much later in gestationthan the upper urinary tract. Variousseries report genitourinary anomaliesof 1% to 3% in association with hy-pospadias. When other systems areinvolved, the incidence increases to7%. We do not recommend routineevaluation of the upper urinary tractfor patients with hypospadias, exceptin cases in which other systems areinvolved, such as imperforate anus,myelomeningocele, or sacral dysge-nesis. The most common anomaliesfound are unilateral renal agenesis,ureteropelvic junction obstruction, ormild vesicoureteral reflux.

INTERSEX

Hypospadias is considered bysome to be a form of intersex. Cer-tainly, in severe hypospadias, inter-

� sex should be considered. However,in the majority of patients with hypo-spadias with normally descendedtestes, the presence of an intersexcondition is unlikely.

If unilateral nondescent of the tes-� tis is present, particularly in an im-

palpable state, this may reflect a pa-tient with mixed gonadal dysgenesisand warrants chromosomal assess-ment. These pateints with hype-spadias may have a testis on oneside and a streak gonad on the other.They usually have a fallopian tube, auterus, and a vagina on one side anda negative chromatin pattern with amosaic karyotype of 45X0, 46XY.Most of these patients (60%) are un-dervirilized, have short stature, andare reared as girls because of theinadequacy of phallic growth (penilelength less than 1 .8 cm stretched).However, diagnosis may not be madein those assigned a male gender untiltheir hypospadias is repaired be-cause of significant virilization. Theyusually have a testis in the inguinal orscrotal position and no testis on theopposite side.

If hypospadias occurs with bilateraltesticular nondescent, then the infantshould be regarded as a girl withadrenogenital syndrome until provenotherwise. No matter how virilized thegenitalia may appear, the finding ofnondescent of the testicles needs animmediate adrenogenital assess-ment.

Partial androgen insensitivity is arare condition, with a severely under-virilized genitalia and hypospadiaseven with descended testes. Truehermaphroditism is rare but may in-clude severe hypospadias.

Some have suggested that hype-spadias is a local manifestation of asystemic endocrinopathy. Allen andGriffin found endocrine abnormalitiesin 11 of 15 patients. Nearly half oftheir patients had substandard re-sponse to human chorionic gonado-trophin when initially studied, al-though four of these patients even-tually had an improved response.Others have found a low response ofluteinizing hormone to gonadotrophin

stimulation and a depressed testos-terone response to human chorionicgonadotrophin stimulation in childrenwith hypospadias.

ELEMENTS OF THEHYPOSPADIAS ANOMALY

Meatus

The urethral meatus may be onlyslightly ventrally placed, just belowthe blind dimple in the normal meatalopening on the glans. On the otherhand, the urethra may be so far backin the perineum that it appears as a“vaginal hypospadias.” Most patientshave a meatus in one of the manytransitional forms. There is a blind-ending periurethral duct located justdistal to the meatus that courses dor-sal to the urethral channel for a shortdistance. It ends blindly and is oftenmisconstrued as the urinary meatus.As the meatus is displaced moreproximally, it is associated with a se-ries of changes determining the se-verity of each anomaly. Penile curva-ture becomes progressively moreprominent. The base of the penisshows caudal displacement; there-fore, there may be penoscrotal trans-position or a bifid scrotum. This con-figuration is analagous to the normalfemale anatomy, where one finds cur-vature of the clitoris that is caudallylocated between the labia.

Skin

The skin of the penis radicallychanges as a result of the disturb-ance in the formation of the urethra.Distal to the meatus, the normal skinshows a V-shaped defect, the ure-thral delta. The edges of this defectgradually merge into the reverted foldof the divided prepuce. The frenulumis absent, and vestiges of the frenu-lum are found inserting on either sideof the open navicular fossa.

The urethra/plate extends from themeatus to the glanular groove andmay be well developed. Even with themeatus proximal on the shaft, thisnormal urethral plate is often elasticand nontethering. It can be used inthe repair without dividing it.

If there is underdevelopment of theurethral plate, fibrous tethering is theusual situation and release of thiscurvature is required surgically.



Fig 2. Transverse preputal island flap with glans channel hypospadias repair.

GENITOURINARY DISEASE

pediatrics in review #{149}vol. 11 no. 2 august 1989 PIR 39

Curvature

The skin deficiency may some-times play a significant part in thecurvature of the penis. Dartos fasciaalso contributes in varying degrees tothe fibrous development. True fibrouschordee is considered a deficiency ofthe normal elastic urethral plate andthe fibrous tissue must be excised torelease the curvature completely. Re-section of this aspect of the anomalyis one of the most difficult surgicalexercises. Sometimes dorsal p//ca-tions of the corpora cavernosa arenecessary to get the penis completelystraight. This is done with a parallelincision and tucks are taken in thetunica albuginea with permanent su-tures.

Artificial erection is used through-out the operative procedure to re-lease chordee and curvature. This isaccomplished with a tourniquet at thebase of the penis and injection ofphysiologic saline solution into theerectile tissue.

TREATMENT OF HYPOSPADIAS

The object of the surgical recon-struction is to straighten the penisand form a urethral extension so thatthe new meatus is as close as pos-sible to the normal site, thus permit-ting a forward-directed stream andnormal coitus. Because the study ofhypospadias has become a subspe-cialty interest of the pediatric urolo-gist, techniques have evolved duringthe last 25 years that have drasticallyimproved the results. In the past,staged procedures were the stand-ard approach. Since the innovation ofvascularized urethroplasties, thehealing process is much more as-sured and total repair with straight-ening and urethral reconstruction cannow be done in one stage.

Element of Surgical Repair

There are five basic phases for asuccessful outcome: (1) meatoplastyand glanuloplasty; (2) orthoplasty, re-moval of chordee, and straighteningof the penis; (3) urethroplasty (con-structing a new urethra from adjacentskin); (4) skin cover; and (5) scroto-plasty.

Meatoplasty and Glanuloplasty.Every effort today is made to have

the meatus at the top of the glans.This is done either with the glanschannel technique (Figs 2 and 3) orby splitting the glans with a urethralstrip down the middle and wrappingthe glanular wings around beneaththis to reform the conical shape (Fig4). The most common proceduredone today, the meatal advancement

and glanuloplasty (MAGPI) (Fig 5), al-lows advancement of the meatus anda glanuloplasty to wrap the glans be-neath this advanced meatus. Thispermits a normally directed streamand a glans with a conical appear-ance.

Orthoplasty or Straightening of theChordee. This is often the most diffi-



In centers that specialize inhypospadias surgery,

excellent results can beachieved with one-stage

repairs frequently done asoutpatient surgeries.

Fig 3. Island flap urethroplasty. A, Generous glans channel of 20-French caliber and long 5-cmisland flap from inner prepuce; B, neourethra in place.

Fig 4. Mathieu procedure.

PIR 40 pediatrics in review #{149} vol. 11 no. 2 august 1989

cult phase of hypospadias repair.About 25% of cases of hypospadiasrequire considerable resection of truefibrous bands. Sometimes dorsaltuck procedures are required to makeup for ventral corporal deficiency andachieve straightening in this manner.

Urethroplasty. The most commonmethods for urethral reconstructionused today require isolating a vas-cularized strip of preputial skin, gen-erally in a transverse fashion, rotatingthis around to the ventrum and fash-

ioning a new urethra from this deli-cate skin. When the blood supply isleft attached, healing is much moreassured (Figs 3 and 6). A free graftrequires revascularization in its newbed and is less likely to achieve theresults desired. Sometimes, it is nec-essary to open the bladder and takea strip of urothelium, fashioning thisinto a graft that can be placed in thebed of the penis for a new urethra.This is a fall-back stage for compli-cations of previous repairs and notused as a primary approach.

Skin Cover. After the penis isstraight and the urethra is rebuilt, var-ious flaps of dorsal preputial skin aretransferred to the ventrum of theshaft of the penis for skin cover.

Scrotop/asty. Transposition of thescrotum requires rotating the superi-orly placed scrotal tissue to the yen-trum or to fuse bifid scrota.

Specific Techniques

Examples of the techniques usedat the Children’s Hospital of Philadel-phia are diagramed in Figs 2 to 6.The MAGPI and Mathieu techniquesare used for anterior hypospadiasthat are the majority of cases at 65%.For middle hypospadias (15%), anonlay island flap technique is fre-

quently appropriate, and for moreposterior or scrotal hypospadiases(20%), a transverse preputial islandflap technique is applied.

Results

The MAGPI procedure has resultedin less than 1 % reoperative rate, theMathieu has a 7% reoperative rate,the onlay has a 7% reoperative rate,

and the transverse preputial andmore proximal complex proceduresrequire about a 1 5% reoperative rate.The total for all hypospadias is 5%.

Complications

Because the tissues are so delicateand exact, and per primum healingmust occur, it is not surprising thatproblems in healing may result. Ureth-rocutaneous fistula formation is themost common complication. Strictureor narrowing with a pocket (divertic-ulum) may require reoperation. Oc-casionally, meatal stenosis is a prob-lem. These all will need a secondexposure to an anesthetic agent butrequire a much less extensive repairthan the initial one-stage repair.

Home Care

At The Children’s Hospital of Phil-adelphia, all hypospadias proceduresare carried out as outpatient surgery.The patients go home with a smallwhite Silastic tube that drips into thediaper and is easily managed. Thetube is thin enough to allow voidingaround it should it become pluggedand soft enough to cause little imta-tion. For bladder spasms, we supplythe parents with banthine and opium(B & 0) suppositories cut into thirds.Acetaminophen usually suffices forpain. We give the babies tnmetho-prim/sulfamethoxazole to suppressany infection. The tube is left in for 1to 2 weeks, depending on the com-



A

D �

�L. 4,/.

1’ �;i� \.�.

The earlier the age forsurgery, the better it istolerated by parent and

patient. Our recommendedage is 6 to 18 months.

Fig 5. Meatal advancement and glanuloplasty (MAGPI) procedure.

Fig 6. Island flap urethroplasty. A, Mobilization of generous pedicle supplying inner preputialneourethra; dorsal penile skin and outer prepuce have been incised in the midline; B, lateralrotation of island flap.

GENITOURINARY DISEASE

pediatrics in review #{149} vol. 11 no. 2 august 1989 PIR 41

plexity of the surgery, and is removedin the office by snipping a holdingstitch that goes through the glans andthrough the tubing. No Foley ballooncatheters are used. Dressings are leftfor 2 to 3 days and either removed athome or the patients return to theoffice for a dressing change. Post-operative visits are made 2 weeks, 6weeks, 1 year, and 5 years after sur-gery.

Age for Surgery

Age for surgery is usually 6 to 18months. Earlier than 6 months forelective surgery is considered to

carry an increased anesthesia risk.However, this outpatient surgery iswell tolerated at any age.

PEDIATRICIAN’S ROLE ANDRESPONSIBILITY

Genital anomalies are generallyperceived as devastating catastro-phes to new parents. Anything wrongwith this particularly delicate area in-flicts immediate guilt and irrationalprejudices. Unless there is clearly anintersex problem (specifically, adren-ogenital syndrome with a virilized fe-male and nonpalpable gonads), greatcare should be taken to not implicatea question of gender. In most cases,hypospadias can be easily diagnosedby inspection alone. We have seen asevere hypospadias with bilateral pal-pable gonads and a generously sizedphallus treated with great alarm andconcern in the newborn nursery. Ob-viously, one must avoid an inappro-priate male gender assignment in apatient with severe androgen insen-sitivity or undervirilized mixed go-nadal dysgenesis, but these situa-tions are generally obvious.

Except for the inguinal anomaliesof undescended testis and inguinalhernia, hypospadias, in general, is anisolated anomaly that does not re-quire elaborate evaluation. Upper



as the patient’s advocate to seek outa specialized center for this delicateand precise surgical reconstruction.

tract studies are not necessary un-less the condition is associated withmalformations in other systems.Chromosomal studies are not nec-essary unless this pattern of testicu-lar descent signals possible mixedgonadal dysgenesis. A voiding cys- SUGGESTED READING

tourethrogram, to evaluate the pres-ence of a utricle, is unnecessary inthe first several months. This may berequired for the surgical correctivestage. Patients should not be circum-cised.

A pediatrician is obligated asthe patient’s advocate to

seek a specialized center forthis delicate and precisesurgical reconstruction.

Hypospadias

PIR 42 pediatrics in review #{149}vol. 11 no. 2 august 1989

The parents should be encouragedto understand that this is a correcta-ble anomaly, with surgery generallydone from 6 months to 18 months ofage as a one-stage reconstruction. Inmost specialized centers, this can bedone as an outpatient surgery or witha short duration inpatient hospitali-zation if long distance travel is re-quired. The complication rate is about20% for severe hypospadias and lessthan 5% for the more distally placedmeatus. These results are obtainedin specialized centers where manyhypospadias repairs are done andsurgeons are familiar with the modernapproach. A pediatrician is obligated

Allen TD, Griffin JE. Endocrine studies in pa-tients with advanced hypospadias. J Urol.1984;131 :310-314

Bauer SB, Retik AB, Colodny AH. Geneticaspects of hypospadias. Urol Clin North Am.19818:559-564

Devine CH Jr, Horton CE: Hypospadias repair.J Urol. 1977;118:188-192

Duckett JW, ed. Symposium on hypospadias.Urol Clin North Am. 1981 ;8:371

Duckett JW. Hypospadias. In: Gillenwater JY,Grayhack JT, Howards 55, Duckett JW,eds. Adult and Pediatric Urology. Chicago,IL: Year Book Medical Publishers, Inc;1987:1880-1915

Ducket JW. MAGPI (meatal advancement andglanuloplasty): a procedure for subcoronal

hypospadias. Urol Clin North Am.1981 ;8:513-520

Duckett JW. The island flap technique for hy-pospadias repair. Urol Clin North Am.19818:503-511

Gibbons AD, Gonzales ET Jr. The subcoronalmeatus. J Urol. 1983;130:739-742

Horton CE. Plastic and Reconstructive Surgeryof the Genital Area. Boston, MA: Little,Brown & Co; 1973:237-248

Khuri FJ, Hardy BE, Churchill BM. Urologicanomalies associated with hypospadias.Urol Clin North Am. 1981 ;8:565-571

McMillan RDH, Churchill BM, Gilmore RE. As-sessment of urinary stream after repair ofanterior hypospadias by meatoplasty andglanuloplasty. J Urol. 1 985;1 34:100-102

Shelton H, Noe HN. The role of excretoryurography in patients with hypospadias. JUrol. 1985;1 35:97-100

Shima H, Ikoma F, Yahumoto H, et al. Gonad-otropin and testosterone response in pre-

pubertal boys with hypospadias. J Urol.1 986;1 35:539-542

Self-Evaluation Quiz

1. Hypospadias occurs most commonly as

A. Autosomal dominant condition of low pe-netrance.

B. Autosomal recessive condition.

C. X-linked dominant condition of low pe-netrance.

D. X-Iinked recessive condition.E. Multifactorial (polygenic) condition.

2. Among the following, the condition leastlikely to be associated with hypospadias inan infant who, when examined, has no cvi-dent abnormality of heart, gut, skeleton, orcentral nervous system is:

A. An upper urinary tract anomaly.B. An inguinal hernia.

C. An undescended testis.0. A utricular divertriculum of the urethra.E. Superficial chordee (curvature) of the

penis and deficiency of the ventral pre-

puce.

3. You are asked to see a 10-day-old infantin whom the diagnosis of hypospadias wasmade at birth and who has been vomitingand losing weight for 2 days. Your findingsare consistent with mild dehydration in aninfant with penoscrotal hypospadias. Testescannot be felt. Among the following, themost urgent study to be made is:

A. Intravenous pyelography.B. Karyotype.C. Determination of serum electrolyte levels.D. Ultrasonography of the pelvic area.E. An upper gastrointestinal radiologic

study.



DOI: 10.1542/pir.11-2-371989;11;37Pediatrics in Review

John W. Duckett, JrHypospadias

ServicesUpdated Information &

http://pedsinreview.aappublications.org/content/11/2/37including high resolution figures, can be found at:

Permissions & Licensing

/site/misc/Permissions.xhtmlits entirety can be found online at: Information about reproducing this article in parts (figures, tables) or in

Reprints/site/misc/reprints.xhtmlInformation about ordering reprints can be found online:



hypospadias john w. duckett, jr pediatrics in review€1989 ... · the incidence of hypospadias is...

Documents