Accepted Manuscript Title: Concealed Epispadias; Report of Two Cases and Review of Literature Author: Saurabh Garge PII: DOI: Reference:
S0090-4295(16)00076-5 http://dx.doi.org/doi: 10.1016/j.urology.2015.09.040 URL 19586
To appear in:
Urology
Received date: Accepted date:
16-7-2015 10-9-2015
Please cite this article as: Saurabh Garge, Concealed Epispadias; Report of Two Cases and Review of Literature, Urology (2016), http://dx.doi.org/doi: 10.1016/j.urology.2015.09.040. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Title of the article: CONCEALED EPISPADIAS; REPORT OF TWO CASES AND REVIEW OF LITERATURE Running title : CONCEALED EPISPADIAS Key words : Concealed ; Epispadias ; Intact prepuce ; Incontinence Contributors 1.DR.SAURABH GARGE, Consultant, M.Ch, Department of pediatric surgery, Choithram hospital & research centre, Indore
Department(s) and institution(s) ; Choithram hospital & research centre,Indore
Corresponding Author:DR.SAURABH GARGE, Choithram hospital & research centre,INDORE 09630956311 [email protected]
Total number of pages: 9 Total number of photographs: 2 Word counts for abstract: 128 for the text: 1800
ABSTRACT Objective: To present an overview of the clinical presentation and pathological anatomy of epispadias with intact prepuce; a rare condition that has only occasionally been reported in literature.
1 Page 1 of 16
Materials and methods: We present two cases of concealed Epispadias. We also review the available literature with regard to this rare condition.We found 14 cases in 7 studies,which were reviewed.Relevant variables were reviewed & are discussed. Results: 13 of 16 cases resented with complaints of buried or webbed penis.In 11 of 16 cases, epispadias was suspected or diagnosed at first presentation and could be surgically corrected in the first intervention. Epispadias repair was successful with regard to cosmesis and erectile function in all cases,including ours. Conclusion: A proper Pre operative diagnosis & Preoperative counseling of parents is essential for the successful treatment of this rare entity. Fewer complications, less additional interventions and better continence rates seem to be noted for the boys with epispadias and intact prepuce. INTRODUCTION Isolated Epispadias is a rare entity with incidence of approximately 1 in 120000 live births.(1-10) It is frequently associated with dorsal curvature, ventral hooded prepuce and defective skin dorsally. The position of meatus can vary from penopubic to shaft to coronal region. It is very uncommon for epispadias to present with an intact prepuce; to date only 18 cases have been reported previously in literature.(1-13) The entity does not appear to be that uncommon but remains under reported, as it can be easily overlooked . It usually presents with a phimotic prepucial orifice where glans is not visible and hence is also known as Concealed Epispadias(CE).(1-10) The article intends to report two cases and review the available literature.(TABLE 1,2) MATERIAL AND METHODS We present two cases of concealed Epispadias operated by us in one year period (Jun 2014-May 2015).We reviewed the literature for key words like concealed, Epispadias, Epispadias with intact prepuce. We found 11 articles contributing to 18 cases of concealed Epispadias. Out of these, one was in Korean.(12) For three articles full texts were not available, and hence were not reviewed.(1,2,4) The remaining 7 articles with 14 cases were reviewed.(TABLE 1,2) RESULTS A.Patient Characteristics 1.Presenting Complaints In most of the previous cases the presenting complaints of parents were related to the small size of the penis in the form of buried or webbed penis[11/14 cases;78%].(3,510)In three cases(22%), patients came with complaints of hernia, UTI & 2 Page 2 of 16
incontinence.(7,8,9)The disease was diagnosed pre operatively in 9 cases(63%) and these patients underwent primary surgery.(2,5,6,8-10)However, in 5 cases(37%) the disease was not diagnosed pre operatively.(4,10)Out of these three were not primarily operated because of consent issues,(10)and two were operated primarily after intra operative discussion with family.(7,10)In our cases, both our cases were diagnosed preoperatively and went across primary Epispadias surgery. 2. Clinical examination The clinical examination in various series showed Pre operative characteristics to diagnose this condition. These are tabulated in TABLE 2.The clinical pre and post operative figures of our patients is shown in Figures 1& 2. B.Pre operative work up(VCUG/VUDS/USG) In 3 of 14 patients, a voiding cystourethrogram(VCUG) was done. It was found to be normal in all cases. In 6 of 14 patients, a videourodynamic study (VUDS) was done which was normal in 2 cases. In 4 cases it showed presence of low grade vesicoureteral reflux(VUR). In one case it revealed open bladder neck while in 3 cases the bladder neck was normal.In our 2 cases we did VCUG and it was found to be normal. The pubic symphysis was found to be open in 3 patients ( 1 coronal and 2 shaft Epispadias).In 9 patients,symphysis were closed. In six patients the only preoperative study was an USG which was normal in all cases. In one patient of Bos et al USG showed normal bladder neck, hence VUDS was not done. In our patients, USG was also done and was found normal in both. C.Surgical Procedures Standard procedures were used to treat Epispadias in all cases.This involved degloving of penis,dissection of urethral plate,tubularistation of urethra,suturing of the corora over the urethra and preputiolasty.The Ipgam rocedure was used for glandular and coronal cases.Sarin et al,did not deglove or use the penile skin in one cases to give acceptable results.In our two cases we did a byars flap urethroplasty in our first case.While in the second case prepuce was left intact and the procedure was done without complete degloving of the penis.(FIGURE 1,2) Bladder neck procedures were not required in any of the patients reported.Maritima et al however had one patient incontinent after surgery where patient awaits bladder neck procedure. Penile lengthening surgeries were done by Bos etal in two patients of his.But they were taken u as secondary procedures. We also did penile lengthening surgery, in our first 3 Page 3 of 16
case, which involved removal of presymhysial fat,suturing of tunica albuginea to pubis and circumcision and dorsal Z plasty along with the primary procedure. D.Penile length (Pre & Post Operative) In all the studies the penis was referred to be short and broad.However exact pre operative and post operative sizes were not mentioned in any study.McCahill et al(5) used re operative testosterone in 2 patients before surgery. However,size of the penis is not mentioned. Merlob et al(3) reported normal penile size in a neonate with glandular Epispadias. Bos et al(10) mentioned post operative lengths in his patients. Bos et al did penile lengthening surgery as a secondary procedure in Two patients. Three patients of his required post operative testosterone.In both our cases, pre operative testosterone was not used.In one patient we did penile lengthening surgey, while the second case had satisfactory length. E.Continence Three patients were incontinent before surgery(One coronal, one shaft and one penopubic).all the other cases were continent before surgery. All the patient except one(shaft Epispadias) became continent after surgery. None required bladder neck procedure, except one. DISCUSSION The embryology of the penile prepuce remains controversial, particularly in epispadias or hypospadias with intact prepuce. McCahill et al. (5) explain the phenomenon of Epispadias with an intact prepuce with the active growth of mesenchyme between the preputial fold and the glandular lamella, which transports the fold distally until it covers the glans completely. If these folds appear proximal to the urethral defect, they cover the defective urethra as well as the glans. This explains cases of distal epispadias, however proximal Epispadias still remain unexplained. Although various hypotheses have been formulated, it remains difficult to explain the development of epispadias in combination with an intact prepuce. (1-13) Experimental studies and animal models with similar abnormalities need to be studied to elucidate the embryological mystery. The diagnosis of CE is usually overlooked because the patients present in the form of buried penis, with non retractile prepuce and normal urinary stream. (1-12)Some clinical features which can raise a suspicion of an underlying Epispadias in such cases are(112); 1. Dorsally directed urinary stream 2. Dorsally placed preputial opening 4 Page 4 of 16
3. Dorsal chordee 4. Gap felt between corpora cavernosa 5. Bifid, spade-like enlarged glans 6. Absence of penile raphe on glans 7. Absence of frenulum A proper diagnosis and recognition of the pathology avoids intra operative surprises and unnecessary invasive surgical interventions and anesthesia. While operating it is important to know the expectations of parents and surgeons .The parents are unaware of the fact that the meatus is located at the corona because they always have witnessed a normal urinary stream from the phimotic orifice. The primary cause for referral in these cases is short phallic length, which many a times persists despite best of efforts on the part of the surgeon. Hence this should be explained to the parents, and they should be advised to have realistic expectations. In previous studies, Bos et al(10)mentions none of the parents were satisfied with the final penile cosmesis mainly due to the short length. McCahill et al (5) patient had satisfactory penile length, while five other studies do not mention anything about final parental satisfaction.(3,4,69)In our study ,parental satisfaction was achieved. This was probably because of two reasons, First because of proper counseling and realistic expectations of parents & second, because penile lengthening procedure were performed during the primary surgery only. Our study also had two cases of coronal Epispadias which usually has acceptable penile lengths. In previous studies, Bos et al (10) performed penile lengthening procedures in three patients while three required testosterone application for the same. The regime used for testosterone usage is not mentioned. McCahill et al (5) used testosterone injection at a dose of 3mg/kg in two of his patients preoperatively. In our case, both our patients had cosmetically acceptable penile lengths post operatively and did not need testosterone stimulation. Another important aspect is increased incidence of either unmasking of new vesicoureteral reflux (VUR) or up gradation of pre existing VUR. This happens because of increase resistance after urethroplasty in a previous low pressure bladder dynamics. Thus re operative & post operative need for a VCUG and long term antibiotic prophylaxis (If VUR is unmasked) should be explained to the parents. We recommend that the detailed analysis of bladder dynamics and VUR should be based on the norms used for isolated Epispadias. In distal varieties where patients are continent, only an USG may suffice. Any abnormal finding in USG should prompt further 5 Page 5 of 16
studies in the form of Videourodynamic Study(VUDS), VCUG or cystoscopy. In proximal varieties and those who are incontinent a proper work up is imperative. Another aspect is that of incontinence which may become apparent after surgery, may it be because of increased post void residue due to VUR or it may be inherent bladder neck in sufficiency. However, in most of the studies the continence improved after surgery and most patients attained continence after surgery. (3, 5-10)Only two patients, one a case of penile Epispadias (8) and other in a penopubic Epispadias (10) were incontinent. Only one case was a candidate for bladder neck procedures in previous studies. (8) The surgical steps used during surgery are standard steps done for Epispadias repair. (1-10)An isolated IPGAM can be used for glandular and coronal Epispadias, and Cantwell -Ransley repair for other more severe varieties of epispadias. Penile length is the basic grievance of a parent. (10)We advise that all interventions described to gain length in cases of buried penis should be done for these children.(11)These are fixing the tunica albuginea of the corpora to pubis at 3,6,9 o' clock positions, cutting the suspensory ligament if it is defective and pulling the shaft inside the pelvis, removing the presymphysial fat and lengthening the dorsal skin.(11)Sarin et al(6) have reported to save the prepuce and create a neo meatus by an isolated IPGAM procedure, but we advice a circumcision in the surgical approach as it gives an extra pacification about the length, in cases with small penile length(our first case).It also avoids another procedure in future. Bos et al performed additional procedures in the form of ventral skin plasty , dorsal Z plasty and circumcision.(10)These could have been incorporated in the primary procedure in order to avoid extra surgeries and anesthesia. In cases of penis with good length an isolated IPGAM procedure with preputioplasty is recommended, as was done in our second case. With regard to the outcome of epispadias repair, the combination of epispadias with an intact prepuce seems to be favorable compared to cases with classical isolated epispadias. Fewer complications, less additional interventions and better continence rates seem to be noted for the boys with epispadias and intact prepuce. CONCLUSION A proper Pre operative diagnosis & Preoperative counseling of parents is essential for the successful treatment of this rare entity. Primary surgery should involve urethral reconstruction and penile lengthening procedures and preputioplasties in order to achieve good cosmetic and functional outcome after first surgery. The aim should be to give best possible cosmetic outcome in as few procedures as possible. 6 Page 6 of 16
REFERENCES 1. Raghavaiah NV. Epispadias associated with phimosis. J Urol 1976;116:671-2. 2.Bhattacharya V, Sinha JK, Tripathi FM. A rare case of Epispadias with normal prepuce. Plast Reconstr Surg 1982;70:372-4. 3. Merlob P, Mor N, Reisner SH. Epispadias with complete prepuce and phimosis in a neonate. Clin Pediatr 1987;26:43-5. 4.Krishna A, Iyer PU. Epispadias with complete non-retractile prepuce. Indian Pediatr 1989;26:1055e6. 5. McCahill PD, Leonard MP, Jeffs RD. Epispadias with phimosis, an unusual variant of the concealed penis. Urology 1995;45:158-60. 6.Sarin YK, Sinha A. Concealed epispadias. Indian J Urol 2001;17:183-4. 7.Sina A, Alizadeh F. Concealed male epispadias, a rare form of penile epispadias presenting as phimosis. Urol J 2011;8:328-9. 8. Maitama HY,Ahmed M,Bello A ,et al. Epispadias with complete prepuce: A rare anomaly . African Journal of Urology 2012;18:90–92. 9. Narayan SK,Akbar SVS,Babu prepuce.Research 2014;1:708.
PR
.Concealed
epispadias
with
normal
10.Bos EM, Kuijper CF, Chrzan RJ,et al. Epispadias in boys with an intact prepuce. J Pediatr Urol. 2014 Feb;10(1):67-73. 11. Liu, X., He, D.-w., Hua, Y., Zhang, D.-y,et al . (2013), Congenital completely buried penis in boys: anatomical basis and surgical technique. BJU International, 112: 271– 275 12. Kang JG, Yoon JH, Yoon JB. Penile Epispadias: A Case Report. Korean J Urol. 1985;26:387-91.
LEGEND FOR FIGURES: Figure 1 Intra operative and Post operative figures of case 1 Figure 2 Intra operative and Post operative figures of case 2 7 Page 7 of 16
TABLE 1 : REVIEW OF LITERATURE Author & year Merlob 1987 McCahill 1995 Mc Cahill 1995 Sarin 2001 Sina 2011 Maitama 2012 Narayan 2014 Bos 2014 Bos 2014 Bos 2014 Bos 2014 Bos 2014 Bos 2014
Age
No.of Pre Primary Bladder surgeries Operative surgery neck diagnosis Procedures neonate Yes -
Penile Testosterone for lengthening Penile length surgery -
1 yr
Parental satisfaction -
One
Yes
Yes
Not reqd
Not reqd
PRe Yes operatively(3mg/kg) Preoperatively ? nm dose
21 mths one
Yes
Yes
Not reqd
Not reqd
3 yrs
one
Yes
Yes
Not reqd
Not reqd
Not reqd
nm
2 mths
One
No
Yes
Not reqd
Not reqd
Not reqd
nm
7 yrs
One
Yes
Yes
Awaited
Not reqd
Not reqd
nm
8 yrs
One
Yes
Yes
Not reqd
Not reqd
Not reqd
nm
5.5 yrs
three
No
No
Not reqd
No
Yes
No
4 yrs
three
No
No
Not reqd
Yes
No
No
4.2 yrs
two
No
No
Not reqd
No
Yes
No
14.3 yrs three
Yes
Yes
Not reqd
Yes
No
No
6.2 yrs
one
Yes
Yes
Not reqd
Not reqd
Not reqd
No
1 yr
one
Yes
Yes
Not reqd
no
Yes
No 8 Page 8 of 16
Author and year Merlob et al (1987) Mchill 1995 Mc hill 1995 Sarin 2001 Sina 2011 Maitama 2012 Narayan 2014 Bos 2014 Bos 2014 Bos 2014
Bos 2014 Garge Garge
3.3 yr
two
No
Yes
Not reqd
Yes
No
No
8 mths 1 yr
One One
Yes Yes
Yes Yes
Not reqd Not reqd
Yes No
No No
Yes Yes
Age
Type of Complaints Epispadias
Urinary control
VCUG
USG
Penile size
Glans width
NM
Not done
normal
Normal for age
Larger
Small,Pre broad Op.testosterone Small, Pre broad Op.testosterone Short broad
Neonate Glandular
Observation by clinician
1 year
Coronal
Not done
normal
21 months 3 years
Penile
Concealed continent Penis Concealed continent Penis Buried Penis continent
Not done
2 months 7 years
glandular
Buried Penis continent & UTI Incontinence incontient
Normal
Not done Not done Normal Short
broad
Normal
Normal Short
Broad
8 years
Penile
normal
Short
Broad
5.5 years 4 years
glandular
Not done normal
5cm
4.2 years
Peno pubic
Not done
5cm
no mention no mention no mention
Glandular
Penile
Coronal
Not done
Inguinal continent Normal hernia Buried Penis incontinent VUR R0L1,oen bladder neck Webbed continent not done Penis Buried and continent Normal webbed penis
4.5cm
9 Page 9 of 16
Bos 2014 Bos 2014
14.3 years 6.2 years
Coronal shaft
Buried penis phimosis epispadias
Bos 2014 Bos 2014 Garge Garge
1 year
Coronal
phimosis
3.3 years 8 mths 1 yr.
Peno pubic Coronal Coronal
webbed and buried penis Buried penis Abnormal prepuce and short penis
continent
Normal
Not done continent VUR Not R1L1,normalbladder done neck continent VUR R2L3,normal Not bladder neck done incontinent VUR R2L2,normal Not bladder neck done continent Not done Normal continent Normal Normal
6cm 6cm 3.5cm 5.5cm 3.5cm 4.5cm
no mention no mention no mention no mention 3.8cm 3.0cm
10 Page 10 of 16
TABLE 2 : Table showing features of clinical examination
Merlob 1987 Mc Cahill 1995 Mc Cahill 1995 Sarin 2001 Sina 2011 Maitama 2012 Narayan 2014 Bos 2014 Bos 2014 Bos 2014 Bos 2014 Bos
Dorsally directed URINARY STREAM Y
Dorsally Dorsal placed CHORDEE PREPUTIAL OPENING Y N
Gap felt between CORPORA CAVERNOSA Y
Bifid,spadelike ENLARGED GLANS Y
Absence of PENILE RAPHE on glans Y
Absence of Pubic FRENULUM Symhysis N
Closed
N
N
Y
Y
Y
N
N
Closed
N
N
Y
Y
Y
nm
nm
nm
Y
N
Y
Y
Y
Y
Y
Closed
Nm
Nm
Y
Nm
Nm
Nm
Nm
nm
Y
Y
Y
Y
Y
Y
Nm
open
Y
Y
Y
Y
Y
nm
nm
Closed
Nm
Nm
Nm
Nm
Nm
Nm
Nm
Closed
Nm
Nm
Nm
Nm
Nm
Nm
Nm
closed
Nm
Nm
Nm
Nm
Nm
Nm
Nm
closed
Nm
Nm
Nm
Nm
Nm
Nm
Nm
open(0.5)
Nm
Nm
Nm
Nm
Nm
Nm
Nm
Closed 11 Page 11 of 16
2014 Bos Nm 2014 Bos Nm 2014 Garge Yes Garge Yes Nm= Not Mentioned
Nm
Nm
Nm
Nm
Nm
Nm
open(0.7)
Nm
Nm
Nm
Nm
Nm
Nm
Closed
No Yes
Yes Yes
Yes Yes
Yes Yes
Yes Yes
Yes Yes
Closed Closed
12 Page 12 of 16
Manuscript #: (Editorial Comment to URL-D-15-01394) Title: “Associated anomalies and manifestations of epispadias are a spectrum regardless of the level of the defect” Corresponding Author:
Joseph G. Borer, MD
Only Author: Joseph G. Borer, M.D. Associate Professor of Surgery (Urology) Reconstructive Urologic Surgery Chair Director, Neurourology and Urodynamics Director, Bladder Exstrophy Program Department of Urology Boston Children’s Hospital & Harvard Medical School
Email: [email protected] Phone: 617-355-7796
13 Page 13 of 16
Male epispadias is a rare and challenging diagnosis within the exstrophy-epispadias complex (EEC). The authors present management of a case of distal epispadias. Management is as per contemporary understanding and technique for distal epispadias with intact prepuce. The status of the prepuce is not of supreme importance here or in any boy with epispadias. What is most important is characterizing the scope and severity of other manifestations, if present, of the field defect in this epispadias, and for epispadias of any level. The authors also provide a thorough review of the literature regarding this entity. One difference of opinion from the authors is that it is my strong belief based on references reported in the manuscript, and personal experience (unpublished data) that a boy with epispadias at any level deserves evaluation with cystourethroscopy and voiding cystourethrogram, and potentially urodynamics as well, in addition to renal and bladder ultrasound (RUS). Epispadias is a spectrum (in and of itself) within the EEC – in other words, epispadias is a spectrum within a spectrum and associated anomalies and manifestations of epispadias are a spectrum regardless of the level of the defect. Epispadias is typically classified by level; peno-pubic, penile, or glanular. This classification fails to include some of the associated malformations that may be present such as pubic diastasis, bladder neck abnormalities, and vesicoureteral reflux (VUR). There is a significant incidence of VUR in these boys. Some may have abnormality in pubic diastasis, with potential repercussions regarding urinary continence. Careful preoperative evaluation should allow the identification of these associated abnormalities, some that will be observed, and some that will help lead to appropriate successful surgical management based on the clinical history, imaging, urodynamic and physical findings. Clinical follow up with progress regards toilet training; successful, delayed or not attained at all, along with non-invasive or invasive assessment of voiding parameters/ function as needed, based on clinical impression. Follow up with physical exam is also important in order to assess for potential development of significant dorsal penile curvature. All of the above; VUR, impaired urinary continence, and development of dorsal penile curvature are possible and these possibilities should be discussed with the parent/ caregiver(s) of a boy with epispadias, regardless of level, peno-pubic, penile, or glanular. This commitment to the care of these boys and their future, should not be overly taxing given the rarity of epispadias of any severity. These rare and, at times, challenging patients deserve are utmost attention regarding evaluation and care.
14 Page 14 of 16
Title of the article: CONCEALED EPISPADIAS; REPORT OF TWO CASES AND REVIEW OF LITERATURE AUTHOR RESPONSE TO EDITORIAL COMMENTS Running title : CONCEALED EPISPADIAS Key words : Concealed ; Epispadias ; Intact prepuce ; Incontinence Contributors 1.DR.SAURABH GARGE, Consultant, M.Ch, Department of pediatric surgery, Choithram hospital & research centre, Indore
Department(s) and institution(s) ; Department of pediatric surgery, Choithram hospital & research centre,Indore
Corresponding Author: DR.SAURABH GARGE, Room No.29, Choithram hospital & research centre,INDORE Mob.No.+919630956311 Fax No. 07314206707 [email protected]
Total number of pages: 1 Word counts : 365 Author Response I agree to the editors comments regarding the need to diagnose associated abnormalities and the need for long term post operative follow up. We strongly believe that the cases of isolated epispadias, especially the distal ones, are associated with far better prognosis than the varieties associated with exstrophy. Therefore we think that all invasive procedures have to be strongly backed with evidence, and should only be done when indicated. We are concerned about three variables in epispadias : 1. Incontinence 15 Page 15 of 16
This is due to an associated weak bladder outlet and should be manifest pre operatively in the form of incontinence. This is rare in cases of distal epispadias. If a patient is incontinent pre operatively, many of them will improve after surgery and toilet training. If patients remain incontinent even after toilet training, Parents need to be explained that another surgery may be required. Usually a weak bladder outlet is also associated with pubic diastasis. An ultrasound and x ray pelvis(both non invasive) can give us enough details and, can lead us to,whether cysto urethroscopy and Urodynamic study (invasive) will be required or not. Moreover, it is easy to convince parents regarding the need for cysto urethroscopy and UDS in patients who are already incontinent. But doing these procedures in continent patients without sufficient evidence is difficult. 2.Penile length and appearance of dorsal curvature Pre operative counseling and need for long term follow up will allay this issue. Accompanying Penile lengthening procedures in the same sitting can decrease the number of surgeries and help in better results pertaining to both the above concerns. 3.Unmasking of vesicoureteral reflux Parents should be counseled for this. A Pre operative Ultrasonography can tell us regarding the presence of vesicoureteral reflux(VUR). But if a patient has never had any urinary tract infections and USG is normal, I will still not recommend a preoperative VCUG(Voiding Cysto urethrogram).Post operatively urine routine microscopy can be ordered. If these are positive or if patient is symptomatic and has recurrent UTIs, further investigations can be done and the patient can be followed on basis of the VUR Protocols. There is no place according to us for pre operative prophylactic invasive procedures in continent isolated distal epispadias patients.
16 Page 16 of 16
S0090-4295(16)00076-5 http://dx.doi.org/doi: 10.1016/j.urology.2015.09.040 URL 19586
To appear in:
Urology
Received date: Accepted date:
16-7-2015 10-9-2015
Please cite this article as: Saurabh Garge, Concealed Epispadias; Report of Two Cases and Review of Literature, Urology (2016), http://dx.doi.org/doi: 10.1016/j.urology.2015.09.040. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Title of the article: CONCEALED EPISPADIAS; REPORT OF TWO CASES AND REVIEW OF LITERATURE Running title : CONCEALED EPISPADIAS Key words : Concealed ; Epispadias ; Intact prepuce ; Incontinence Contributors 1.DR.SAURABH GARGE, Consultant, M.Ch, Department of pediatric surgery, Choithram hospital & research centre, Indore
Department(s) and institution(s) ; Choithram hospital & research centre,Indore
Corresponding Author:DR.SAURABH GARGE, Choithram hospital & research centre,INDORE 09630956311 [email protected]
Total number of pages: 9 Total number of photographs: 2 Word counts for abstract: 128 for the text: 1800
ABSTRACT Objective: To present an overview of the clinical presentation and pathological anatomy of epispadias with intact prepuce; a rare condition that has only occasionally been reported in literature.
1 Page 1 of 16
Materials and methods: We present two cases of concealed Epispadias. We also review the available literature with regard to this rare condition.We found 14 cases in 7 studies,which were reviewed.Relevant variables were reviewed & are discussed. Results: 13 of 16 cases resented with complaints of buried or webbed penis.In 11 of 16 cases, epispadias was suspected or diagnosed at first presentation and could be surgically corrected in the first intervention. Epispadias repair was successful with regard to cosmesis and erectile function in all cases,including ours. Conclusion: A proper Pre operative diagnosis & Preoperative counseling of parents is essential for the successful treatment of this rare entity. Fewer complications, less additional interventions and better continence rates seem to be noted for the boys with epispadias and intact prepuce. INTRODUCTION Isolated Epispadias is a rare entity with incidence of approximately 1 in 120000 live births.(1-10) It is frequently associated with dorsal curvature, ventral hooded prepuce and defective skin dorsally. The position of meatus can vary from penopubic to shaft to coronal region. It is very uncommon for epispadias to present with an intact prepuce; to date only 18 cases have been reported previously in literature.(1-13) The entity does not appear to be that uncommon but remains under reported, as it can be easily overlooked . It usually presents with a phimotic prepucial orifice where glans is not visible and hence is also known as Concealed Epispadias(CE).(1-10) The article intends to report two cases and review the available literature.(TABLE 1,2) MATERIAL AND METHODS We present two cases of concealed Epispadias operated by us in one year period (Jun 2014-May 2015).We reviewed the literature for key words like concealed, Epispadias, Epispadias with intact prepuce. We found 11 articles contributing to 18 cases of concealed Epispadias. Out of these, one was in Korean.(12) For three articles full texts were not available, and hence were not reviewed.(1,2,4) The remaining 7 articles with 14 cases were reviewed.(TABLE 1,2) RESULTS A.Patient Characteristics 1.Presenting Complaints In most of the previous cases the presenting complaints of parents were related to the small size of the penis in the form of buried or webbed penis[11/14 cases;78%].(3,510)In three cases(22%), patients came with complaints of hernia, UTI & 2 Page 2 of 16
incontinence.(7,8,9)The disease was diagnosed pre operatively in 9 cases(63%) and these patients underwent primary surgery.(2,5,6,8-10)However, in 5 cases(37%) the disease was not diagnosed pre operatively.(4,10)Out of these three were not primarily operated because of consent issues,(10)and two were operated primarily after intra operative discussion with family.(7,10)In our cases, both our cases were diagnosed preoperatively and went across primary Epispadias surgery. 2. Clinical examination The clinical examination in various series showed Pre operative characteristics to diagnose this condition. These are tabulated in TABLE 2.The clinical pre and post operative figures of our patients is shown in Figures 1& 2. B.Pre operative work up(VCUG/VUDS/USG) In 3 of 14 patients, a voiding cystourethrogram(VCUG) was done. It was found to be normal in all cases. In 6 of 14 patients, a videourodynamic study (VUDS) was done which was normal in 2 cases. In 4 cases it showed presence of low grade vesicoureteral reflux(VUR). In one case it revealed open bladder neck while in 3 cases the bladder neck was normal.In our 2 cases we did VCUG and it was found to be normal. The pubic symphysis was found to be open in 3 patients ( 1 coronal and 2 shaft Epispadias).In 9 patients,symphysis were closed. In six patients the only preoperative study was an USG which was normal in all cases. In one patient of Bos et al USG showed normal bladder neck, hence VUDS was not done. In our patients, USG was also done and was found normal in both. C.Surgical Procedures Standard procedures were used to treat Epispadias in all cases.This involved degloving of penis,dissection of urethral plate,tubularistation of urethra,suturing of the corora over the urethra and preputiolasty.The Ipgam rocedure was used for glandular and coronal cases.Sarin et al,did not deglove or use the penile skin in one cases to give acceptable results.In our two cases we did a byars flap urethroplasty in our first case.While in the second case prepuce was left intact and the procedure was done without complete degloving of the penis.(FIGURE 1,2) Bladder neck procedures were not required in any of the patients reported.Maritima et al however had one patient incontinent after surgery where patient awaits bladder neck procedure. Penile lengthening surgeries were done by Bos etal in two patients of his.But they were taken u as secondary procedures. We also did penile lengthening surgery, in our first 3 Page 3 of 16
case, which involved removal of presymhysial fat,suturing of tunica albuginea to pubis and circumcision and dorsal Z plasty along with the primary procedure. D.Penile length (Pre & Post Operative) In all the studies the penis was referred to be short and broad.However exact pre operative and post operative sizes were not mentioned in any study.McCahill et al(5) used re operative testosterone in 2 patients before surgery. However,size of the penis is not mentioned. Merlob et al(3) reported normal penile size in a neonate with glandular Epispadias. Bos et al(10) mentioned post operative lengths in his patients. Bos et al did penile lengthening surgery as a secondary procedure in Two patients. Three patients of his required post operative testosterone.In both our cases, pre operative testosterone was not used.In one patient we did penile lengthening surgey, while the second case had satisfactory length. E.Continence Three patients were incontinent before surgery(One coronal, one shaft and one penopubic).all the other cases were continent before surgery. All the patient except one(shaft Epispadias) became continent after surgery. None required bladder neck procedure, except one. DISCUSSION The embryology of the penile prepuce remains controversial, particularly in epispadias or hypospadias with intact prepuce. McCahill et al. (5) explain the phenomenon of Epispadias with an intact prepuce with the active growth of mesenchyme between the preputial fold and the glandular lamella, which transports the fold distally until it covers the glans completely. If these folds appear proximal to the urethral defect, they cover the defective urethra as well as the glans. This explains cases of distal epispadias, however proximal Epispadias still remain unexplained. Although various hypotheses have been formulated, it remains difficult to explain the development of epispadias in combination with an intact prepuce. (1-13) Experimental studies and animal models with similar abnormalities need to be studied to elucidate the embryological mystery. The diagnosis of CE is usually overlooked because the patients present in the form of buried penis, with non retractile prepuce and normal urinary stream. (1-12)Some clinical features which can raise a suspicion of an underlying Epispadias in such cases are(112); 1. Dorsally directed urinary stream 2. Dorsally placed preputial opening 4 Page 4 of 16
3. Dorsal chordee 4. Gap felt between corpora cavernosa 5. Bifid, spade-like enlarged glans 6. Absence of penile raphe on glans 7. Absence of frenulum A proper diagnosis and recognition of the pathology avoids intra operative surprises and unnecessary invasive surgical interventions and anesthesia. While operating it is important to know the expectations of parents and surgeons .The parents are unaware of the fact that the meatus is located at the corona because they always have witnessed a normal urinary stream from the phimotic orifice. The primary cause for referral in these cases is short phallic length, which many a times persists despite best of efforts on the part of the surgeon. Hence this should be explained to the parents, and they should be advised to have realistic expectations. In previous studies, Bos et al(10)mentions none of the parents were satisfied with the final penile cosmesis mainly due to the short length. McCahill et al (5) patient had satisfactory penile length, while five other studies do not mention anything about final parental satisfaction.(3,4,69)In our study ,parental satisfaction was achieved. This was probably because of two reasons, First because of proper counseling and realistic expectations of parents & second, because penile lengthening procedure were performed during the primary surgery only. Our study also had two cases of coronal Epispadias which usually has acceptable penile lengths. In previous studies, Bos et al (10) performed penile lengthening procedures in three patients while three required testosterone application for the same. The regime used for testosterone usage is not mentioned. McCahill et al (5) used testosterone injection at a dose of 3mg/kg in two of his patients preoperatively. In our case, both our patients had cosmetically acceptable penile lengths post operatively and did not need testosterone stimulation. Another important aspect is increased incidence of either unmasking of new vesicoureteral reflux (VUR) or up gradation of pre existing VUR. This happens because of increase resistance after urethroplasty in a previous low pressure bladder dynamics. Thus re operative & post operative need for a VCUG and long term antibiotic prophylaxis (If VUR is unmasked) should be explained to the parents. We recommend that the detailed analysis of bladder dynamics and VUR should be based on the norms used for isolated Epispadias. In distal varieties where patients are continent, only an USG may suffice. Any abnormal finding in USG should prompt further 5 Page 5 of 16
studies in the form of Videourodynamic Study(VUDS), VCUG or cystoscopy. In proximal varieties and those who are incontinent a proper work up is imperative. Another aspect is that of incontinence which may become apparent after surgery, may it be because of increased post void residue due to VUR or it may be inherent bladder neck in sufficiency. However, in most of the studies the continence improved after surgery and most patients attained continence after surgery. (3, 5-10)Only two patients, one a case of penile Epispadias (8) and other in a penopubic Epispadias (10) were incontinent. Only one case was a candidate for bladder neck procedures in previous studies. (8) The surgical steps used during surgery are standard steps done for Epispadias repair. (1-10)An isolated IPGAM can be used for glandular and coronal Epispadias, and Cantwell -Ransley repair for other more severe varieties of epispadias. Penile length is the basic grievance of a parent. (10)We advise that all interventions described to gain length in cases of buried penis should be done for these children.(11)These are fixing the tunica albuginea of the corpora to pubis at 3,6,9 o' clock positions, cutting the suspensory ligament if it is defective and pulling the shaft inside the pelvis, removing the presymphysial fat and lengthening the dorsal skin.(11)Sarin et al(6) have reported to save the prepuce and create a neo meatus by an isolated IPGAM procedure, but we advice a circumcision in the surgical approach as it gives an extra pacification about the length, in cases with small penile length(our first case).It also avoids another procedure in future. Bos et al performed additional procedures in the form of ventral skin plasty , dorsal Z plasty and circumcision.(10)These could have been incorporated in the primary procedure in order to avoid extra surgeries and anesthesia. In cases of penis with good length an isolated IPGAM procedure with preputioplasty is recommended, as was done in our second case. With regard to the outcome of epispadias repair, the combination of epispadias with an intact prepuce seems to be favorable compared to cases with classical isolated epispadias. Fewer complications, less additional interventions and better continence rates seem to be noted for the boys with epispadias and intact prepuce. CONCLUSION A proper Pre operative diagnosis & Preoperative counseling of parents is essential for the successful treatment of this rare entity. Primary surgery should involve urethral reconstruction and penile lengthening procedures and preputioplasties in order to achieve good cosmetic and functional outcome after first surgery. The aim should be to give best possible cosmetic outcome in as few procedures as possible. 6 Page 6 of 16
REFERENCES 1. Raghavaiah NV. Epispadias associated with phimosis. J Urol 1976;116:671-2. 2.Bhattacharya V, Sinha JK, Tripathi FM. A rare case of Epispadias with normal prepuce. Plast Reconstr Surg 1982;70:372-4. 3. Merlob P, Mor N, Reisner SH. Epispadias with complete prepuce and phimosis in a neonate. Clin Pediatr 1987;26:43-5. 4.Krishna A, Iyer PU. Epispadias with complete non-retractile prepuce. Indian Pediatr 1989;26:1055e6. 5. McCahill PD, Leonard MP, Jeffs RD. Epispadias with phimosis, an unusual variant of the concealed penis. Urology 1995;45:158-60. 6.Sarin YK, Sinha A. Concealed epispadias. Indian J Urol 2001;17:183-4. 7.Sina A, Alizadeh F. Concealed male epispadias, a rare form of penile epispadias presenting as phimosis. Urol J 2011;8:328-9. 8. Maitama HY,Ahmed M,Bello A ,et al. Epispadias with complete prepuce: A rare anomaly . African Journal of Urology 2012;18:90–92. 9. Narayan SK,Akbar SVS,Babu prepuce.Research 2014;1:708.
PR
.Concealed
epispadias
with
normal
10.Bos EM, Kuijper CF, Chrzan RJ,et al. Epispadias in boys with an intact prepuce. J Pediatr Urol. 2014 Feb;10(1):67-73. 11. Liu, X., He, D.-w., Hua, Y., Zhang, D.-y,et al . (2013), Congenital completely buried penis in boys: anatomical basis and surgical technique. BJU International, 112: 271– 275 12. Kang JG, Yoon JH, Yoon JB. Penile Epispadias: A Case Report. Korean J Urol. 1985;26:387-91.
LEGEND FOR FIGURES: Figure 1 Intra operative and Post operative figures of case 1 Figure 2 Intra operative and Post operative figures of case 2 7 Page 7 of 16
TABLE 1 : REVIEW OF LITERATURE Author & year Merlob 1987 McCahill 1995 Mc Cahill 1995 Sarin 2001 Sina 2011 Maitama 2012 Narayan 2014 Bos 2014 Bos 2014 Bos 2014 Bos 2014 Bos 2014 Bos 2014
Age
No.of Pre Primary Bladder surgeries Operative surgery neck diagnosis Procedures neonate Yes -
Penile Testosterone for lengthening Penile length surgery -
1 yr
Parental satisfaction -
One
Yes
Yes
Not reqd
Not reqd
PRe Yes operatively(3mg/kg) Preoperatively ? nm dose
21 mths one
Yes
Yes
Not reqd
Not reqd
3 yrs
one
Yes
Yes
Not reqd
Not reqd
Not reqd
nm
2 mths
One
No
Yes
Not reqd
Not reqd
Not reqd
nm
7 yrs
One
Yes
Yes
Awaited
Not reqd
Not reqd
nm
8 yrs
One
Yes
Yes
Not reqd
Not reqd
Not reqd
nm
5.5 yrs
three
No
No
Not reqd
No
Yes
No
4 yrs
three
No
No
Not reqd
Yes
No
No
4.2 yrs
two
No
No
Not reqd
No
Yes
No
14.3 yrs three
Yes
Yes
Not reqd
Yes
No
No
6.2 yrs
one
Yes
Yes
Not reqd
Not reqd
Not reqd
No
1 yr
one
Yes
Yes
Not reqd
no
Yes
No 8 Page 8 of 16
Author and year Merlob et al (1987) Mchill 1995 Mc hill 1995 Sarin 2001 Sina 2011 Maitama 2012 Narayan 2014 Bos 2014 Bos 2014 Bos 2014
Bos 2014 Garge Garge
3.3 yr
two
No
Yes
Not reqd
Yes
No
No
8 mths 1 yr
One One
Yes Yes
Yes Yes
Not reqd Not reqd
Yes No
No No
Yes Yes
Age
Type of Complaints Epispadias
Urinary control
VCUG
USG
Penile size
Glans width
NM
Not done
normal
Normal for age
Larger
Small,Pre broad Op.testosterone Small, Pre broad Op.testosterone Short broad
Neonate Glandular
Observation by clinician
1 year
Coronal
Not done
normal
21 months 3 years
Penile
Concealed continent Penis Concealed continent Penis Buried Penis continent
Not done
2 months 7 years
glandular
Buried Penis continent & UTI Incontinence incontient
Normal
Not done Not done Normal Short
broad
Normal
Normal Short
Broad
8 years
Penile
normal
Short
Broad
5.5 years 4 years
glandular
Not done normal
5cm
4.2 years
Peno pubic
Not done
5cm
no mention no mention no mention
Glandular
Penile
Coronal
Not done
Inguinal continent Normal hernia Buried Penis incontinent VUR R0L1,oen bladder neck Webbed continent not done Penis Buried and continent Normal webbed penis
4.5cm
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Bos 2014 Bos 2014
14.3 years 6.2 years
Coronal shaft
Buried penis phimosis epispadias
Bos 2014 Bos 2014 Garge Garge
1 year
Coronal
phimosis
3.3 years 8 mths 1 yr.
Peno pubic Coronal Coronal
webbed and buried penis Buried penis Abnormal prepuce and short penis
continent
Normal
Not done continent VUR Not R1L1,normalbladder done neck continent VUR R2L3,normal Not bladder neck done incontinent VUR R2L2,normal Not bladder neck done continent Not done Normal continent Normal Normal
6cm 6cm 3.5cm 5.5cm 3.5cm 4.5cm
no mention no mention no mention no mention 3.8cm 3.0cm
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TABLE 2 : Table showing features of clinical examination
Merlob 1987 Mc Cahill 1995 Mc Cahill 1995 Sarin 2001 Sina 2011 Maitama 2012 Narayan 2014 Bos 2014 Bos 2014 Bos 2014 Bos 2014 Bos
Dorsally directed URINARY STREAM Y
Dorsally Dorsal placed CHORDEE PREPUTIAL OPENING Y N
Gap felt between CORPORA CAVERNOSA Y
Bifid,spadelike ENLARGED GLANS Y
Absence of PENILE RAPHE on glans Y
Absence of Pubic FRENULUM Symhysis N
Closed
N
N
Y
Y
Y
N
N
Closed
N
N
Y
Y
Y
nm
nm
nm
Y
N
Y
Y
Y
Y
Y
Closed
Nm
Nm
Y
Nm
Nm
Nm
Nm
nm
Y
Y
Y
Y
Y
Y
Nm
open
Y
Y
Y
Y
Y
nm
nm
Closed
Nm
Nm
Nm
Nm
Nm
Nm
Nm
Closed
Nm
Nm
Nm
Nm
Nm
Nm
Nm
closed
Nm
Nm
Nm
Nm
Nm
Nm
Nm
closed
Nm
Nm
Nm
Nm
Nm
Nm
Nm
open(0.5)
Nm
Nm
Nm
Nm
Nm
Nm
Nm
Closed 11 Page 11 of 16
2014 Bos Nm 2014 Bos Nm 2014 Garge Yes Garge Yes Nm= Not Mentioned
Nm
Nm
Nm
Nm
Nm
Nm
open(0.7)
Nm
Nm
Nm
Nm
Nm
Nm
Closed
No Yes
Yes Yes
Yes Yes
Yes Yes
Yes Yes
Yes Yes
Closed Closed
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Manuscript #: (Editorial Comment to URL-D-15-01394) Title: “Associated anomalies and manifestations of epispadias are a spectrum regardless of the level of the defect” Corresponding Author:
Joseph G. Borer, MD
Only Author: Joseph G. Borer, M.D. Associate Professor of Surgery (Urology) Reconstructive Urologic Surgery Chair Director, Neurourology and Urodynamics Director, Bladder Exstrophy Program Department of Urology Boston Children’s Hospital & Harvard Medical School
Email: [email protected] Phone: 617-355-7796
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Male epispadias is a rare and challenging diagnosis within the exstrophy-epispadias complex (EEC). The authors present management of a case of distal epispadias. Management is as per contemporary understanding and technique for distal epispadias with intact prepuce. The status of the prepuce is not of supreme importance here or in any boy with epispadias. What is most important is characterizing the scope and severity of other manifestations, if present, of the field defect in this epispadias, and for epispadias of any level. The authors also provide a thorough review of the literature regarding this entity. One difference of opinion from the authors is that it is my strong belief based on references reported in the manuscript, and personal experience (unpublished data) that a boy with epispadias at any level deserves evaluation with cystourethroscopy and voiding cystourethrogram, and potentially urodynamics as well, in addition to renal and bladder ultrasound (RUS). Epispadias is a spectrum (in and of itself) within the EEC – in other words, epispadias is a spectrum within a spectrum and associated anomalies and manifestations of epispadias are a spectrum regardless of the level of the defect. Epispadias is typically classified by level; peno-pubic, penile, or glanular. This classification fails to include some of the associated malformations that may be present such as pubic diastasis, bladder neck abnormalities, and vesicoureteral reflux (VUR). There is a significant incidence of VUR in these boys. Some may have abnormality in pubic diastasis, with potential repercussions regarding urinary continence. Careful preoperative evaluation should allow the identification of these associated abnormalities, some that will be observed, and some that will help lead to appropriate successful surgical management based on the clinical history, imaging, urodynamic and physical findings. Clinical follow up with progress regards toilet training; successful, delayed or not attained at all, along with non-invasive or invasive assessment of voiding parameters/ function as needed, based on clinical impression. Follow up with physical exam is also important in order to assess for potential development of significant dorsal penile curvature. All of the above; VUR, impaired urinary continence, and development of dorsal penile curvature are possible and these possibilities should be discussed with the parent/ caregiver(s) of a boy with epispadias, regardless of level, peno-pubic, penile, or glanular. This commitment to the care of these boys and their future, should not be overly taxing given the rarity of epispadias of any severity. These rare and, at times, challenging patients deserve are utmost attention regarding evaluation and care.
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Title of the article: CONCEALED EPISPADIAS; REPORT OF TWO CASES AND REVIEW OF LITERATURE AUTHOR RESPONSE TO EDITORIAL COMMENTS Running title : CONCEALED EPISPADIAS Key words : Concealed ; Epispadias ; Intact prepuce ; Incontinence Contributors 1.DR.SAURABH GARGE, Consultant, M.Ch, Department of pediatric surgery, Choithram hospital & research centre, Indore
Department(s) and institution(s) ; Department of pediatric surgery, Choithram hospital & research centre,Indore
Corresponding Author: DR.SAURABH GARGE, Room No.29, Choithram hospital & research centre,INDORE Mob.No.+919630956311 Fax No. 07314206707 [email protected]
Total number of pages: 1 Word counts : 365 Author Response I agree to the editors comments regarding the need to diagnose associated abnormalities and the need for long term post operative follow up. We strongly believe that the cases of isolated epispadias, especially the distal ones, are associated with far better prognosis than the varieties associated with exstrophy. Therefore we think that all invasive procedures have to be strongly backed with evidence, and should only be done when indicated. We are concerned about three variables in epispadias : 1. Incontinence 15 Page 15 of 16
This is due to an associated weak bladder outlet and should be manifest pre operatively in the form of incontinence. This is rare in cases of distal epispadias. If a patient is incontinent pre operatively, many of them will improve after surgery and toilet training. If patients remain incontinent even after toilet training, Parents need to be explained that another surgery may be required. Usually a weak bladder outlet is also associated with pubic diastasis. An ultrasound and x ray pelvis(both non invasive) can give us enough details and, can lead us to,whether cysto urethroscopy and Urodynamic study (invasive) will be required or not. Moreover, it is easy to convince parents regarding the need for cysto urethroscopy and UDS in patients who are already incontinent. But doing these procedures in continent patients without sufficient evidence is difficult. 2.Penile length and appearance of dorsal curvature Pre operative counseling and need for long term follow up will allay this issue. Accompanying Penile lengthening procedures in the same sitting can decrease the number of surgeries and help in better results pertaining to both the above concerns. 3.Unmasking of vesicoureteral reflux Parents should be counseled for this. A Pre operative Ultrasonography can tell us regarding the presence of vesicoureteral reflux(VUR). But if a patient has never had any urinary tract infections and USG is normal, I will still not recommend a preoperative VCUG(Voiding Cysto urethrogram).Post operatively urine routine microscopy can be ordered. If these are positive or if patient is symptomatic and has recurrent UTIs, further investigations can be done and the patient can be followed on basis of the VUR Protocols. There is no place according to us for pre operative prophylactic invasive procedures in continent isolated distal epispadias patients.
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