| Dialogue Archives: Management of CAH: Vaginoplasty
(Volume 5, Number 7, July 1982)
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Guest Editor: Stuart B. Bauer, M.D.
W. Hardy Hendren, M.D. Howard McM. Snyder, M.D.
John Money, Ph.D.
Richard M. Ehrlich, M.D. Professor of Surgery/Urology Co-Chief of Renal Transplantation University of California, Los Angeles School of Medicine, Los Angeles
A. Barry Belman, M.D. Chairman, Department of Pediatric Urology Children's Hospital National Medical Center, Washington, D.C., Professor of Urology George Washington Medical School
Donald B. Halverstadt, M.D. Clinical Professor of Urology & Pediatrics University of Oklahoma College of Medicine Chief, Pediatric Urology Service, Children's Memorial Hospital, Executive Chief of Staff, State of Oklahoma Teaching Hospitals, Oklahoma City
W. Hardy Hendren, III, M.D. Professor of Surgery (Pediatrics) Harvard Medical School, Boston Chief, Pediatric Surgery Massachusetts General Hospital
Panayotis Kelalis, M.D.
Professor and Head, Section of Pediatric
Urology Mayo Clinic, Rochester, Minn.
Selwyn Levitt, M.D. Director, Division of Pediatric Urology Co-Director,.Children's Kidney Center Albert Einstein College of Medicine and Montefiore Hospital, New York
William J. Miller
Associate Editor Jacqueline Nardi Egan
Lester Persky, M.D. Professor of Urology Case Western Reserve University School of Medicine, Cleveland
Victor A. Politano, M.D. Professor and Chairman, Dept. of Urology University of Miami School of Medicine Miami, Florida
Edward Tank, M.D.
Associate Professor of Surgery & Pediatrics University of Oregon School of Medicine Portland, Oregon
Robert M. Weiss, M.D. Professor, Section of Urology Yale University School of Medicine New Haven, Conn.
Robert H. Whitaker, F.R.C.S. Consultant Urologist Addenbrooke's Hospital Cambridge, England Associate Lecturer University of Cambridge
SUBJECT OF CONTROVERSY: MANAGEMENT OF CAH
This second issue on the management of CAH under Dr. Bauer's superb stewardship is informative and fascinating. Dr. Money's multivariate concept of sexual definition is extremely important and deserves emphasis. His admoni-tions regarding careful, knowledgeable evaluation in these newborns cannot be overemphasized.
These two issues on CAH have placed a rather esoteric subject in clear focus. Our compliments and thanks to Dr. Bauer and to the participants in these issues for an outstanding job.
Richard M. Ehrlich, M.D.
vaginal reconstruction early, within the first year of life. Dr. Money's investigations support this concept of early surgical management. Correction of the genital deformity, as with hypospadias, is best completed before the child develops a strong self-image of herself. In addition, it makes it easier for the parents to interact with the child and provide appropriate clues relating to her femininity. This is especially true for the more masculinized female with the U-G sinus extending out onto the shaft of the phallus. Only now are we recognizing the long-term psychological effects of late reconstructive surgery on the genitalia in both males and females.
Stuart B. Bauer, M.D.
Assistant Professor of (Urology) Surgery, Harvard Medical School; Children's Hospital Medical Center, Boston
GUEST EDITOR'S NOTES:
This second issue on CAH deals with the surgical aspects of vaginal reconstruction. The stenotic introitus associated with a thin membrane can be managed by a simple midline cutback incision. When a single perineal opening exists, it is important to delineate the extent of the urogenital sinus (U-G sinus) and the position of the communication between the urethra and the vagina. Before the U-G sinus is opened, the surgeon must be sure that the vagina enters distal to the external urethral sphincter. Otherwise, injury of the sphincter mechanism will occur when the tract is opened.
Although many people have preferred the midline incision in the perineal body, with closure of the skin and vaginal mucosa transversally, Dr. Snyder's description of a broad-ly based perineal flap advanced into the vaginal vault has more appeal. There is less chance for stricturing of the introitus and the opening is oriented somewhat posteriorly, as the perineal body below the midline flap is partially excised. This incision also allows excessive preputial skin form the hypertrophied clitoris to be advanced posteriorly into the lateral corners of the midline V-flap, creating labia minora which are absent in these girls.
When the communication between the vagina and the urethra is high and close to or above the level of the external urethral sphincter, the vagina must be separated from the urethra without injuring the sphincter mechanism. An inflated Fogarty balloon passed through the U-G sinus and into the vagina can easily identify this junction, as Dr. Hendren describes. After separating the vagina, the urethral opening is closed and the vagina brought to the perineum.
The contributors to this issue prefer to complete the
W. HARDY HENDREN, M.D.
Professor of Surgery (Pediatrics), Harvard Medical School, Boston; Chief, Pediatric Surgery, Massachusetts General Hospital, Boston
Adrenogenital Syndrome and Its Repair
In females with the adrenogenital syndrome there is wide variation in the degree of masculinization of the external genitalia, presumably related to the intensity of androgenic stimulation during early fetal development. At the mild end of the spectrum is a girl whose clitoris is slightly enlarged and there is minimal labial fusion. At the ' extreme end of the spectrum is a female with a normal-looking penis with a urethra which empties at its tip, and an "empty scrotum." We have seen some severe cases who were thought to be normal males with undescended testes and actually had been circumcised!
It is important to appreciate that the anatomy of the lower urinary tract can vary widely in anatomic configu-ration in these patients. They cannot all be repaired by a single technique. In a minimal case, a Heineke-Mikulicz posterior vaginoplasty may suffice. In the majority of cases, we use a flap vaginoplasty and also do clitoro-plasty in the same operation. The clitoris is recessed after reducing it by subtotal resection of the shaft. Shaft skin is used to make labia minora. The vagina is exteri-orized with a large perineal flap, also advancing posteri-orly the labioscrotal tissue to form labia majora.
In very masculinized cases, however, a different type of vaginal reconstruction is required. As shown in Figure 1 a, the vagina can have a high confluence with the urogenital sinus, actually above the external urethral sphincter. It is, therefore, important to panendoscope these children to discover this. At the point where the vagina enters there is a mound which looks like the veru montanum in males. If a cut-back procedure or a flap vaginoplasty should be performed on these high-placed vaginas, not only will the vagina be inadequately exteri-orized, but stress incontinence can result because there will remain only the bladder neck for continence. This can also predispose to urinary infection.
We'd like to hear from you and welcome your comments, experiences, criticisms, etc. on the subject of this issue and other issues of this publication. Please send them to Richard M. Ehrlich, M.D., Division of Urology, UCLA Medical Center, Los Angeles, CA. 90024.
The technique, a perineal pull-through vaginoplasty, (Figures 1a-e), is a much more difficult procedure than a flap vaginoplasty. We defer doing it until the child is about 2 years old. Flap vaginoplasties and clitoroplasties are usually performed when the child is 3 to 6 months of age.
After a pull-through procedure to disconnect the vagina from the urogenital sinus, a subsequent revision will be necessary when the child is older (Figure 2). The open-ing is enlarged with secondary flaps in front and back, and the labioscrotal tissue is advanced posteriorly. This
results in a very normal-looking female appearance. Other technical details of these operations are described in the bibliography cited below. □
Hendren WH, Crawford JD: Adrenogenital Syndrome: The anatomy of
the anomaly and its repair. Some new concepts. J Ped Surg
Hendren WH: Reconstructive problems of the vagina and the female
urethra. Plastic Cl of N Amer 7:207-34,1980.
Hendren WH, Donahoe PK: Correction of congenital abnormalities of
the vagina and perineum. J Ped Surg 15:751,1980.
SUBJECT OF CONTROVERSY: MANAGEMENT Of CAH
HOWARD McM. SNYDER, III, M.D.
Associate Director, Division of Urology, Children's Hospital of Philadelphia
Introduction. Vaginoplasty is generally elective in female infants with virilized external genitalia. Urine does not usually pool sufficiently in the vagina to produce infection or bladder emptying problems. When the vagina enters the urogenital sinus at a low level, we generally have carried out surgery after the endocrinologic stabilization of the child, which is usually 2 or 3 months after the diagnosis was made. There may be a good rationale for postponing this surgery until a child is 6 months old. General anesthetics administered prior to that age are associated with a slightly increased risk of sudden infant death syndrome after surgery.
An evaluation of the vagina's entry level into the U-G sinus is critical for planning the timing and type of vagino-plasty. As part of the child's initial intersex evaluation, we carry out a genitogram, which is a combination of a retrograde injection of the U-G sinus and a voiding cys-tourethrogram. This permits accurate definition of the in-volved anatomy. At the time of vaginoplasty, we routinely carry out endoscopy of the U-G sinus to confirm our im-pressions. If the vagina has a high entry on the U-G sinus (there's a considerable gap between the vagina and perineum), then a pull-through vaginoplasty should be delayed until the child is older. We do not use any topical agents in preparation for surgery. Even in the in-fant, the vaginal and perineal tissue are adequate for surgical repair.
Cut-back Vaginoplasty This type of vaginoplasty is suit-able only in the rare instance when the vagina reaches very low on the perineum and the anatomical defect is primarily that of fusion of the labia. A vertical incision is made over the U-G sinus and the edges are then sutured with fine chromic catgut. If the incision is carried a short distance into the vagina, a minor Heineke-Mikulicz re-arrangement results which may draw a small amount of additional tissue into the introitus. This procedure, how-ever, interposes little tissue into the vaginal introitus and -accordingly, the introitus must be of adequate calibre when it is unroofed. More commonly, the introitus does require widening and a more extensive procedure is needed.
Flap Vaginoplasty. This is the most commonly performed vaginoplasty to exteriorize a low-lying vagina. It consti-tutes a form of the standard plastic surgical Y-to-V-plasty. A broadly based inverted U incision is made (Figure 1). As the flap is developed, a holding stitch in the tip prevents excessive tissue handling and damage. The flap should be kept thick. The underlying rectum can be easily identified by intermittently placing a Kelly clamp in the rectum. Carefully dissecting the tissue layer by layer avoids injury to the rectum. A Foley catheter is kept in the urethra to mark and protect that structure. Traction made on a small Foley catheter blown up in the vagina draws the vagina down. This facilitates dissection by permitting better exposure of the vagina's posterior wall. The vagina's posterior wall must be incised deeply enough above its junction with the U-G sinus to permit
the interposition of a good portion of the U flap (Figure 2). Otherwise, the introitus is not significantly widened. This follows the basic principles of a Y-to-V-plasty.
Placing holding stitches in the wall of the vagina as it is divided will help expose the area and place the flap to the apex of the vaginal incision. Absorbable material, usually fine chromic catgut, is used to suture the perin-eal flap to the vagina (Figure 3).
A drain is not employed. The Foley catheter is optionally maintained for 24 to 48 hours after surgery. Ten to 14 days after surgery, gentle vaginal dilatation is begun. While we have used polyethylene plastic anal dilators, which the general surgeons in our hospital use for im-perforate anus repairs, a centrifuge tube, or in the small infant an eye-dropper, would be as satisfactory. We do this twice a day for a few weeks, then once a day there-after. The vaginal introitus should be calibrated inter-
mittently for the 6 months after surgery and if stenosis appears to be occurring, vaginal dilatation should be resumed.
The procedure described above usually suffices to provide good vaginal drainage early in life. A minor re-vision is commonly needed when the child sexually matures because some introital stenosis may recur de-spite these efforts. It is usually possible to carry out a small repeat Y-to-V-plasty.
Creation of Labia Minora (Marburger Technique). In girls with masculinization of the external genitalia, the labia minora are usually absent. To provide as completely normal a female-appearing perineum as possible, we have constructed labia minora from the skin of the shaft of the phallus using the technique of Hans Marburger of Innsbruck. We do this at the time of clitoroplasty; it does not have to be done concomitantly with a vaginoplasty.
Incisions are made on the ventrolateral surface of the phallus and continued down inside the labia majora to open a bed for the new labia minora. Dorsally, a trans-verse incision just proximal to the glans joins the two vertical incisions (Figure 3). The dorsal shaft skin with all of its subcutaneous tissue is mobilized, carrying the dis-section in the avascular plane just outside Buck's fascia. The inner surface of the foreskin is unfolded and the foreskin is split in the midline down to the mons level (Figure 4). The linear strips of skin are laid into the in-cisions which have been previously made inside the labia majora. The newly-created labia may look more normal if an effort is made not to flatten out these strips of skin excessively in the process of suturing their medial and lateral borders down (Figure 5). Since the cosmetic effect is generally quite good, we feel that this minor amount of additional surgery (Figure 6) is well justified. □
SUBJECT OF CONTROVERSY: MANAGEMENT OF CAH
JOHN MONEY, Ph.D.
Professor of Medical Psychology, Department of Psychiatry and Behavioral Sciences, and Associate Professor of Pediatrics, The Johns Hopkins University School of Medicine and Hospital, Baltimore
Psychologic Aspects of Hermaphroditic Masculiniza-tion (Defeminization) in 46.XX Babies Assigned as Girls The definition of sex as a multivariate concept'2 was formally recognized in 1965 in the 24th edition of Dor-land's Medical Dictionary. The new definition is essen-tial for science and medicine. It is also, because of the authority of the dictionary, of great value in counseling the parents of a newborn baby with a birth defect of the genitals concerning the reannouncement of its sex and future program of surgical, hormonal, and psychologic management.
The new definition (from the 26th edition, 1981) includes the following: chromosomal sex: sex as determined by the presence of the XX (female) or the XY (male) genotype in somatic cells, and without regard to pheno-typic manifestations (also called genetic sex); endo-crinologic sex: the phenotypic manifestations of sex determined by endocrine influences, such as breast development, etc.; genetic sex: chromosomal sex; gonadal sex: the sex as determined on the basis of the gonadal tissue present, whether ovarian or testic-ular; morphologic sex: sex determined on the basis of the morphology of the external genitals; nuclear sex: the sex as determined on the basis of the presence or absence of sex chromatin in somatic cells, its pres-ence normally indicating the XX (female) genotype, and its absence the XY (male) genotype; psychological sex: the self-image of the gender role of an individual; social sex: the complex of attitudes, expectations etc., that a society attaches to the male and female roles.
In the overwhelming majority of cases, congruency be-tween all of the variables is complete, and each variable can, therefore, be predicted perfectly on the basis of knowing one of them. In cases of genital birth defect, however, predictions made on the basis of standard expectations may be in error.
Know Your Syndromes. It is absolutely necessary, therefore, to know the etiology, diagnosis and especially the prognosis of each syndrome. Otherwise, psycho-logic counseling of either the parents or the older patient will be haphazard and erroneous. To illustrate: in some cases of the adrenogenital syndrome, instead of a clitoris, a fully-formed penis differentiates, despite XX chromosomal sex and ovarian gonadal sex. In the absence of corticosteroid therapy, it is then the sex of the external genitals, not of the chromosomes or ovar-ies, that correctly predicts masculinizing hormonal sex at puberty and, since the assignment and rearing often is as a boy, a boy's status in G-l/R (gender identity and role).
Conversely, in cases of the androgen-insensitivity syn-drome, it is the external sex of the vulva, not the XY chromosomal sex nor the testicular gonadal sex, that correctly predicts feminizing hormonal sex at puberty and, since the rearing is typically as a girl, a girl's status in G-l/R.
Surgical Prognosis. In all cases of a clitoropenis (or penoclitoris), a satisfactory feminizing surgical result, cosmetically, should be predictable, but the prediction, at least in some cases, has fallen short. The vaginal orifice may stenose, the clitoral hood and labia minora may be virtually nonexistent, the clitoris totally absent, and the urethral orifice so positioned that the urinary stream is directed too much forward. The likelihood of such unsightly and dysfunctional a result of attempted surgical feminization increases proportionately to the extent of the presurgical external genitalia masculini-zation.
Defective feminization has a severely adverse psycho-logic effect on the parents. They may become phobic about allowing their baby to be seen even by her own siblings. It also has an effect on the child herself, at all stages of her life, especially when she tries to enter a romantic and erotic bonding with a partner.
The surgical technique that has the best cosmetic and functional prognosis is the one in which the hooded glans and its labial extensions, as well as the rami of the phallic organ, are preserved, though reduced to clitoral size. The same applies in those syndromes of 46.XY hermaphroditism and of extreme micropenis, in which the plan of case management is decided in favor of habilitating the baby as a girl.
Regardless of the neonatal sex announcement, there exists in the newborn period a degree of freedom in deciding on surgical feminization of the genitalia which is lost completely some time during the second year of life, usually by the age of 18 months. This is the age when a child's self-concept and body image as male or female begins to enter into awareness, and can sub'-sequently be retrieved from memory. Thenceforth, gen-ital surgery by edict is experienced as genital mutilation and has adverse long-term psychopathological reper-cussions. The parents also may be affected adversely. In addition, juvenile siblings may be covertly threatened with a fear of genital mutilization of themselves. To il-lustrate, a 46.XX adrenogenital 3-year-old child with a fully-formed penis and scrotum would, if surgically feminized by edict, experience the surgery as mutilation in the same way as would any boy or man. The same would apply if the phallic organ were a greatly hyper-trophied, hooded clitorine structure with a urethral groove instead of a tubular urethra.
In the adrenogenital syndrome, early reconstructive genital surgery has a still unexplained side effect, namely, enuresis that persists too long into the pre-adolescent and, in some cases, even into the adoles-cent years. A purely conjectural explanation, based on animal experimental evidence, is that the brain had been fetally masculinized for urination in the standing position, whereas surgery to the external anatomy had a contrary effect.
Hormonal Prognosis. In the prenatal nonadrenogenital forms of female hermaphroditic masculinization, the counseling regarding the hormonal prognosis at puberty is straightforward since puberty is with virtual certainty
feminizing. No hormonal treatment is necessary. By contrast, in the adrenogenital syndrome, the counseling may be made complicated because feminizing puberty of normal chronological onset is utterly dependent on the regularity of corticosteroid therapy. Not all parents are consistently compliant. Noncompliance becomes an even greater problem when the child herself becomes old enough to repudiate illness symbolically by repudi-ating treatment—a very common phenomenon in pre-puberty and adolescence. Consequently, she becomes somatically virilized and in response she may be baffled, indifferent, ambivalent or, in a few instances, self-vin-dicated insofar as she experiences herself as being erotically attracted toward only a female as a lover.
"Falling in Love" Prognosis. In my own published34 and unpublished follow-up studies of female adrenogential hermaphroditism, it became evident that those few with a complete penis raised as males had less difficulty in em-barking on a teenage romantic life than did those raised as girls with an early history of surgery for clitoromegaly and corticosteroid therapy since infancy. During their teenage years, these girls were reticent loners. In their twenties, they became more talkative. Ot a group of 30, one-third said they were heterosexual, both in imagery and practice; one-third said they were bisexual or homosexual (3 among 10 found themselves to be exclu-sively homosexual), and one-third were noncommital.
In the follow-up of girls prenatally masculinized by progrestinic compounds administered to the mother, erotic imagery and practice was heterosexual only.5 Since this is a new finding that has not been given notice by either the media or the scientific press, its full impact on psychologic counseling of parents of newborn adre-nogenital hermaphroditic girls has not yet been made manifest.
Erotic Prognosis. Erotic counseling in the adrenogenital syndrome may, on the basis of unsystematized clinical observations, proceed in anticipation of no known ill ef-fects from corticosteroid therapy.
There are no extensive before-and-after data on the ef-fects of genital surgery on erotic sensation and orgasm. The data that do exist, however, indicate that ablative surgery in either women or men may change the intensity or quality of the feeling, but not destroy it totally.6 Con-firmatory information has been obtainedjrom African women subjected as girls to the traditional genital muti-lation known as pharaonic circumcision or infibulation.7
Postsurgical counseling in syndromes of hermaphroditic masculinization may, therefore, assumed positive erotic prognosis, though absolute prophecies are best avoided. Explicit sex and coital education, preferably using explicit pictures and movies, is essential in order to promote erotic sex confidence and to eliminate handicap. Edu-cation, however, does not compensate for loss of erotic tissue. The less surgical mutilation or ablation, the better.
Genetic Prognosis. In all hermaphroditic syndromes, the parents need genetic counseling, family-planning and next-birth counseling. In the adrenogenital and other hermaphroditic syndromes in which fertility is preserved, the patients themselves, as they become older, require
the same. They are best advised to keep the information private and within the family. They should not reveal it too quickly with a prospective partner who eventually may share parenthood with them. Health insurance ahead of time is imperative. Pregnancy need not be con-traindicated.
Medical Self-Knowledge. Since this is the era of "the freedom of information act" and extensive media medical reporting, all counseling should be designed with the expectation that eventually nothing will be withheld. Counselors require special training and skill, syndrome by syndrome, so that they understand what information can be given and when in order for it to be therapeutic and not traumatizing. To illustrate, one uses such con-cepts as a birth defect of the sex organs; being born sexually unfinished; born with the vulva and vagina sealed over; being an XX man or an XY woman (or an XXY man, X/XX woman, or one of may other chromo-somal configurations); having sex glands or gonads with tissue that under the microscope appears either testicular or ovarian, or mixed in structure, though unable to do testicular or ovarian work, as the case may be; and so forth.
Parents and older patients need to be counseled on what they can expect to find exploited in the media about the feuding that exists among experts with respect to both the politics and the science of sex differences. They will be party to both theoretical and applied dissension, some of it applicable to themselves, revolving around the reactivated nature/nurture polarization of sex differences. The voice of reason points to neither extreme, but rather to the interaction of both. When professional hubris out-weighs professional reason, it is, as always when doctors disagree, the patients who get caught in the crossfire. They become our victims. □
1. Money J: Hermaphroditism, gender and precocity in hyperadreno-corticism; Psychologic findings. Bulletin of The Johns Hopkins Hos-pital, 96:253-264, 1955.
2. Money J, Hampson JG, Hampson JL: An examination of some basic sexual concepts: The evidence of human hermaphroditism. Bulletin of The Johns Hopkins Hospital, 97:301-319,1955.
3. Money J, Delery J: latrogenic homosexuality: Gender identity in seven 46.XX chromosomal females with hyperadrenocortical hermaphroditism born with a penis, three reared as boys, four reared as girls. Journal of Homosexuality, 1:357-371,1976.
4. Money J, Schwartz M: Dating, romantic and nonromantic friendships, and sexuality in 17 early-treated adrenogenital females, aged 16-25, in Lee PA, Plotnick LP, Lowarski AA, Migeon CJ (eds): Congenital Adrenal Hyperplasia. Baltimore, University Park Press, 1977.
5. Money J, Mathews D: Prenatal exposure to virilizing progestins: An adult follow-up study of twelve women. Archives of Sexual Behavior, in press.
6. Money J: Components of eroticism in man. II: The orgasm and genital somesthesia. Journal of.Nervous and Mental Disease, 132:289-297, 1961.
7. Lightfoot-Klein H: Female circumcision in the Sudan updated 1981. (Manuscript for Der Spiegel, personal communication, 1981.) Bibliography
Money J: Hermaphroditism: An Inquiry into the Nature of a Human Paradox. Doctoral Dissertation, Harvard University Library, 1952. University Microfilms Library Services, Xerox Corporation. Ann Arbor, Michigan 48106, 1967.
SUBJECT OF CONTROVERSY MANAGEMENT OF CAH
Due to space limitations in the Ureterosigmoidostomy issue (Vol. 5, No. 3, March 1982), it was not possible to run a bibliography supplied by Harry Spence^M.D., one of the contributors to the issue. Below is that bibliography.
(35 case reports prior to 1978 are listed in our paper "Tumor of the colon as a late complication of ureterosigmoidostomy for exstrophy of the bladder" (BrJUrol 51(6):466-470, Dec 1979). Articles subsequent to this follow and include 17 patients for a total of 52 where the operation was done for exstrophy with the number for all indications at 85.)
New References thru Nov 1981
Dube S et al: Can J Surg 22(5):484-486, Sept 1979.
Dunn M, Roberts JB, Smith PJ: BrJUrol 51 (4):260-263,~Aug 1979.
Erakl.is AJ, Folkman AJ: J Ped Surg 13(6D):730-734, Dec 1978.
Leadbetter GW Jr, Zickerman P, Pierce E: J Urol 121(6):732-735,
O'Higgins N, Disney J, Duff FA, Kelly DO: Br J Urol 53(3)278-279
Recht KA, Belis JA, Kandzari SJ, Milam DF: Cancer 44(4):1538-1542,
Rubay J, Lefebre P, de Pierpont B, Filvez M: Ada gastroenterol (Belg)
42(7r8):275-284, Jul/Aug 1979.
Schipper H, Decter A: Cancer 47(8):2062-2065, Apr 15,1981.
Shapiro A et al (including Caine M): Urology 13(6):617-620, Jun 1979.
Warren RB, Warner TF, Hafez GR: J Urol 124(4):550-551, Oct 1980.
Weshler A, Sulkes A, Rizel S: Dis Colon Rectum 22(6):434-436,
Crissey MM, Steele CD, Gittes RF: Rat model for carcinogenesis in ureterosigmoidostomy. Science 207:1079-1080, Mar 7 1980. Bristol JB, Williamson RCN: Ureterosigmoidostomy and colon carcinogenesis. Science 214(4518):851, Oct 16 1981. Rabinovitch HH: Ureterosigmoidostomy in children—Revival or demise? J Urol 124(4):552, Oct 1980.
Stewart M, Hill JM, Pugh RCB, Williams JP: The role of N-nitrosamine in carcinogenesis of the ureterocolic anastomosis. Br J Urol 53(2):115-118, Apr 1981.
Shouska H et al: Meal loop carcinoma after cystectomy for bladder exstrophy. Br Med J (2):(6134)397-398, Aug 5 1978. (Note: only case we found of Ca in ileal loop.)
Chiang MS et al (including Wise HA II): Carcinoma in a colon conduit urinary diversion. In press. Read at May 1981 Meeting of AUA. (Note: only case we found after colon loop.)
standing the physical and psychological aspects of this condition and in time, progress toward advance research and treatment."
Please address all inquiries to: Mr. and Mrs. Boross, 5075 Medhurst Ave., Solon, OH 44139
Along the same lines, Mrs. Elayne Shapiro, also an exstrophy parent, has done an outstanding job at writing and publishing a publication titled "Parents for Exstrophy Letter." She can be contacted at 1450 North 12th Street, Fargo, North Dakota, 58102
Mr. and Mrs. Boross and Mrs. Shapiro deserve our support and assistance. Please communicate with them and send them the names and addresses of exstrophy families you know.
I would like to call your attention to a recently published book Genitourinary Problems in Pediatrics coauthored by Drs. A. Barry Belman and George W. Kaplan. It was published by W. B. Saunders Co., Philadelphia, as Volume XXIII in the series on Major Problems in Clinical Pediatrics.
The book was written with the pediatrician in mind rather than the urologist, but it is a superb compilation of the personal views of the authors and on the state of the art in pediatric urology today. In my judgment, the book would make an excellent gift for the referring pediatrician. Belman and Kaplan are to be complimented on their perserverance and obvious expertise in producing this useful volume.
Here are some of the topics which will be discussed in the upcoming issues of Dialogues in Pediatric Urology and the Guest Editors of these issues:
Pediatric Urology Office Practice
(Guest Editor: R. Lawrence Kroovand, M.D.)
Yolk Sac Tumors
(Guest Editor: George W. Kaplan, M.D.)
(Guest Editor: Stephen A. Kramer, M.D.)
Posterior Urethral Valves
(Guest Editor: Bernard M. Churchill, M.D.)
Sports Medicine and Pediatric Urology (Guest Editor: William J. Cromie, M.D.)
I have been in contact recently with a Mr. and Mrs. Boross. They are the concerned parents of a child born with exstrophy of the bladder which has prompted them to form a support group. Below is a portion of a letter that I received from them which I think will be of extreme interest to the readers of this publication:
"As parents of a child with exstrophy of the bladder, my husband and I are forming a support group comprised of other parents and guardians of children who are afflicted with this condition. We are also encouraging adults with exstrophy to become part of what we hope will become a nationwide organization for the benefit of all people so afflicted. Our objectives in forming this group are to ex-change experiences and information with others who are so affected and with those who care for them. My husband and I feel that with the sincere efforts of all those involved, great strides can be made in under-
Opinions expressed in this publication are the sole responsibility of the individuals named and do not necessarily reflect the opinions of the editorial board or the publisher and members of this organization.
Copyright © 1982 by William J. Miller Associates, Inc.
All rights reserved. No part of this publication may be reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying recording or by any information retrieval system, without written permission from the publisher.