Demography and spectrum of male and female hypospadias among the children: A single centre experience

Hypospadias is the most common urogenital anomalies in male with an incidence of 1 in 300 live births¹.

 

Female hypospadias is extremely rare in compare to male hypospadias and showed very minimal existing in previous literature^2,3.

 

Male hypospadias is a congenital defectwhere the urethral opening situated in the ventral surface of penis rather than the tip of glans. This anomaly alsoassociated with   arrested development of the spongiosum, ventral skin and ventral curvature⁴.

 

Most of the surgeons use the term ‘hypospadias' and urogenital sinus in case of female child interchangeably.

 

When the common channel is only (0.5-1.5) cm long, that is ‘very low anomaly' some consider this as ‘female hypospadias. But many of them consider when the urethral opening is locatedon the anterior vaginal wall just above the introitus up to the vaginal fornix₅.

 

Male hypospadias classified on several methods. Based on location of urethral opening, hypospadias is classified into anterior (glandular, coronal), mid-penile (distal, midshaft and proximal) and posterior (penoscrotal, scrotal, perineal)⁶.

 

Solov'ev⁷ classified the FH into three types, namely vestibular (partial), vestibulo- vaginal (subtotal) and vaginal (total).

 

On the basis of anatomy, Derevianko and his colleagues classified the FH into low -vaginal ectopic, high-vaginal ectopia andurogenital fusion of the bladder neck with vagina (vesicovaginal)³ and urovaginal (vesicovaginal).

 

Megameatus intact prepuce (MIP) is the rare variant of male hypospadias.  At birth, this type may be missed to diagnosed and may be noted during circumcision.

 

Common associated anomalies of hypospadias are undescended testis, inguinal hernia, hydrocele, persistant mullarien structures, pelviureteric junction obstruction, vesicoureteric reflux⁸.

 

No techniques for hypospadias repair still now not recommended as “gold standard” because no evidences have been found the superiority of one technique over another⁹.

 

In many studies, showed the repair of distal hypospadias   in single stage. Tubularized incised plate (TIP). Mathieu, Onlay island flap are the mostly used techniques for distal hypospadias⁸. Nevertheless, there is ‘two school thought' for surgical correction of proximal variety. One group favour one stage procedure such as inlays preputial flap, while other groups choose to perform two stage procedure^10,11.

 

So, this study aimed to show the demography, spectrum of male and female hypospadias, associated deformities, anomalies and surgical techniques.

This retrospective study was conducted in the paediatric surgery department of Bangladesh Medical University (BMU), Dhaka, Bangladesh. The study covered the 8-years period, from Jan'2016 to Dec'2023

 

Child with hypospadias was all admitted into paediatric surgery ward for repair through outdoor clinic. Majority of patients were not circumcised. After admission, routine investigations likeComplete blood count (CBC), Blood creatinine, Urine: R.M.E and C/ S and x-ray chest, USG of KUB region of all patients were performed. Only for posterior type of hypospadias MCU was done for excluding dilated prostatic utricle.

 

Patients who previously underwent hypospadias surgery in another hospital (Government and Private) and glanular hypospadias with no associated deformity (stenosis/ glans tilt/chordee) were excluded in this study.

 

Different surgeons repaired male hypospadias by their preferable different techniques. Vaginal flap urethroplasty was performed for reconstructed female hypospadias. Pyramid procedure was preferable techniques for MIP. Admission registry and Operation theatre registry from 2016 to 2023 were checked for spectrum of male and female hypospadias,  variant of hypospadias, age of repair, different techniques for repair, associated deformities andanomalies.

 

Follow-up of individual patients could not be included as these were not properly recorded.

 

For data entry and analysis, using Statistical Package for Social Sciences (SPSS) version 26. Datawas expressed by percentage, mean and range.

Out of 328, 322(98. 2%) patients were male and 6 (1.8%) patients were female.

 M:F= 53.7:1.

Demographic profile of the study patients (n=328)                              Female Hypospadias

Sex	No of patients	(%)
Male	322	98.2
Female	6	1.8
Total	328	100.0
Male: Female ratio	53.7: 1

 

 

 

 

 

 

 

73.2% patients belong to (>3-12) years at the time of surgery.

Age at Presentation (n=328)

In male hypospadias, 25% patients were distal penile variety.

In female hypospadias, most (1.5%) patients were total (vaginal) type.

Rare hypospadias variant (MIP) was 2.1% of patients.

 

Types and variant of Hypospadias (n=328)

             

Mild chordee was seen in 41.3%, moderate chordee was in 16.2% and severe chordee was in 35.9% of male hypospadias.

Meatal stenosis was seen in 1.9% of cases.

 

Associated deformities of male hypospadias (n=315)

Among the associated anomalies, in male hypospadias 13.7% patients had UDT

 

Associated anomalies of male hypospadias (n=315)

 

Hypospadias is a Greek word, 'Hypo' means 'under' 'spadon' means a 'rent' or 'fissure'12

Formation of hypospadias occur by incomplete virilization of the genital tubercle, so developing an ectopic urethral opening on the ventral surface of penis. This opening may be located from the glans penis to the perineum with or without ventral preputial defect and curvature6.

 

Surgery is the only treatment for hypospadias. In the first and second century AD, the two Alexandrian surgeons, Helidorus and Antyllus, first to describe, classify, and define the pathophysiology and the treatment of hypospadias13. Repair of hypospadias is important for cosmesis and good functional restoration of the penile structure14

 

In our study, most of the child seeks for medical advisedbeyond the recommended age. In low-income countries, age of presentation for hypospadias repair is higher than in developed countries due to ignorance, poverty and illiteracy15 In addition, in our institution, most of the surgeons preferred to operate the child at school going age due to inadequate nursing facilities.

 

In our study, the mean age was 6.33 years for hypospadias repair.  Hoag CC and his colleagues16 showed in their studies mean age for hypospadias repair was 1.97 year. The recommended age for surgery is (6-18) month17. At the 6 months of age, is the good tolerance of surgery and anaesthesia. At the age of 18-month, child is aware about his genitalia and achieve some toilet training.

 

In our eight- years retrospective study (Jan'2016 to Dec'2023), there were 6(1.8%) female hypospadias out of 328 patients with male: female- 53.7:1.

 

 On the other hand, Bhat A and his colleagues5 showed in their retrospective study since 1991, four female hypospadias out of 600 male hypospadias patients with male: female:150:1.

 

MadhusoodananS and his colleagues18, also reported five female hypospadias in their 10 years retrospective study.

 

In our study, distal penile hypospadias was more (25%) than the other types and next was penoscrotal variety, which was 13.0%. Different study showed different results regarding types of hypospadias.  Gabra A and his colleagues19 showed in their study 23.5% was coronal and 12. 6% was scrotal variety, which was more common than the other variety, in their study. Other studies 20,21 also showed mostly coronal hypospadias.

 

Megameatus Intact Prepuce (MIP) is a clinically rare and unique variant of hypospadias, that accounts for 1% to 3% of hypospadias cases13,14 MIP detection is rising due to increase of physical examination of the patient22. In our study 7 (2.1%) patients had MIP out of 328 hypospadias repair cases from 2016 to 2023. But Cendron M23 reported in their study period from 2007 to 2017, MIP patients were 21(5%) out of 481 hypospadias cases

 

Inguinal hernia, hydrocele and cryptorchidism are more common associated anomalies of hypospadias patients 24

 

In our study, undescended testes (cryptorchidism) were found in 13.7% of patients. On the other hand, Kolon TF and his colleagues25 showed 7% and Ceccarelli and his colleagues26 showed 5.4% cryptorchridism of hypospadias patients in their studies.

 

Chordee is the most common associated deformity in hypospadias. It usually noticeable during an erection. In our study, mild chordee with hypospadias patients 41.3%, which was higher than moderate (16.2%) and severe (35.9%) variety of chordee with hypospadias.  Aslam OM27 and Duarsa GWK and his colleagues28 showed the moderate chordee with hypospadias 40.6% and 69.4% respectively, which was higher than the mild and severe variety of chordee in their studies. Variation of this result in different literature may be type of hypospadias patients in different country.

 

Principles of hypospadias repair included penile degloving, orthoplasty, urethroplasty, glanuloplasty and cosmosis. Correction of Hypospadias are performed either by tubularization, augmentation or replacement method.

 

 

Duplay and TIP procedure based on tubularization technique. Snodgrass, Mathieu and Duckett repair based on augmentation technique. Replacement technique used in proximal hypospadias where urethral plate is poorly developed and require two stage urethroplasty (Koyanagi, Bracka, STAG)29.

 

For achieving the sufficientwidening of the urethral plate, a midline incision was given in the distal urethral plate during reconstructing theneourethra. This tubularization method of urethral plate described in the year 1987. But, Snodgrass in the year 1994, tubularized the neourethra with well vascularized strip by incised the entire length of urethral plate at its midline. Snodgrass (TIP) procedure became very popular among the surgeons due to low complication rate as well as its simplicity and cosmosis30.

 

In our study, 38.8% cases TIP (Snodgrass) procedure was performed in hypospadias repair, which was comparable to other studies 31-33

 

In case of proximal hypospadias, where mild chordee present and requiring minimal dissection, TIP procedure was preferred by surgeons due to unsatisfying cosmotic results34.

 

Conclusion: Over the last 50 years there had been many studies with contradictory results.  Age of repair in our centre was much higher than the recommended age. Like the previous literatures, our study also showed the very rarity of female hypospadias. Procedure choice was different according to the type of hypospadias and preference of surgeons.

 

Limitations: First, our study was mainly retrospective as well as single centre study.

 

Second, In this centre no universal standardprotocolfor follow up.  So, sufficient outcome data after hypospadias repair not properly recorded.

 

Finally, in this centre no comparative studied mentioned regarding the proceduresperformed by the different consultants.

1.     Baskin LS. Hypospadias.  In: Baskin LS, (ed). Hypospadias and genital development. Advances in experimental medicine and biology. 2004. Springer. Boston MA.

2.     Doi: https://doi.org/10.1007/978-1-4419-89995-6_1.

3.     Sarin YK, Kumar P. Female hypospadias -need for clarity of definition and management. J.Ind. Assoc. Pediatr. Surg.2019; 24 (2):141-3.Doi: 10.4103/jiaps.  JIAPS_69_18. PMID:31105404; PMCID:PMC 6417043.

4.     Derevianko IM, Derivianko TI, Ryzhkov VV. Hypospadia in females. Urologii.2007; 3: 26-8.

5.     https://pubmed.ncbi.nim.nih.gov.

6.     Iqbal T, Uddin N, Khan M, Naz S. Frequency of complications in the Snodgrass repair and risk factors.Pak. J. Surg. 2011; 27(3): 188-93.

7.     https://www.mathewsopenaccess.com

8.     Bhat A, Saxena R, Bhat MP, Dawan M, Saxenal G. Female hypospadias with vaginal stones: a rare congenital anomaly. J. Pediatr. Urol. 2009; 6(1): 70-4. Doi: 10.1016/jpurol.2009.03.014.Epub 2009 April 26.PMID: 19394897

9.     Bhat A.General considerations in hypospadias surgery. Indian J. Urol. 2008; 24(2):188-94. Doi: 10.4103/0970-1591.40614.PMID: 19468396,PMID: PMC2684287.

10.   Solov'ev AE. The diagnosis and treatment of hypospadias in girls.  Urol. Nefrol (Mosk).1993; 6:  11-3.https://pubmed.ncbi.nim.nih.gov.

11.   Mansoor K, Abdul M, Waqas H, Hidayat U, Shazia N, Syed AS, etal  Hypospadias Repaie: A single Centre Experience. Plast. Surg. Int. 2014; 1: 1155-60. https://doi.org/10.1155/2014/453039.

12.   Subramaniam R, Spinoit AF, Hoebeke P. Hypospadias repair:an overview of the actual techniques. SeminPlast. Surg.2011; 25: 206-12.

13.   Doi: 10.1055/s-0031-1281490.PMID:22851912;PMCID:PMC 3312185

14.   Cook A, khoury AE, Neville C, Bagli DJ, Farhat WA, Pippi Salle JL. A multicenter evaluation of technical preferences for primary hypospadias repair. J. Urol. 2005; 174 (6): 2354-7.

15.   Doi: 10.1097/01.ju.0000180643.01803.43.PMID:16280842.

16.   Springer A, Krois W, Horcher E. Trends in hypospadias surgery: results of a worldwide survey. Eur. Urol. 2011; 60(6): 1184-9.

17.   Doi: 10.1016/j.eururo.2011.08.031.Epub2011Aug22.PMID:218771708

18.   Duckett JW, Baskin LS. Hypospadias. In: Gillenwater JY, Grayback JT, Hawards SS, Duckett JW, editors. Adult and Pediatric Urology. 4th ed. St. Louis Mosby- year book. 2001; 2549-89.

19.   Hadidi AT. History of Hypospsdias. In: Hadidi AT editor. Hypospadias Surgery. 2nd. ed. Springer, Switzerland. 2012; 3-24.

20.   Von Der Horst HJR, De Wall LL. Hypospadias, all there is to know. 2017; 176(4): 435-41

21.   https://doi.org/10.1007/s00431-017-2864-5.

22.   Bhat A.Extended urethral mobilization in incised plate urethroplasty for severe hypospadias: variation in technique to improve chordee correction. J. Urol. 2007; 178(3 Pt 1):1031-5.

23.   Doi:10.1016/j.juro.2007.05.074.Epub 2007 Jul 16. PMID:17632146.

24.   Hoag CC, Gotto GT, Morrison KB, Coleman GU, Macneily AE. Long- term functional outcome and satisfaction  of patients with Hypospadias repaired in childhood. Can. Urol. Assoc. J. 2008, 2: 23-31. Doi:https://doi.org/10.5489/cuaj.521.

25.   Dale J, Woodward B, Elagami H. Age- dependent early complications of hypospadias repair: a single institutional experience.  Pediatr. Surg. Int. 2023.39(1):115-23. Doi: 10.1007/s00383-023-05388-2.

26.   Madhusoodanan S, Soman AC, Menon H. Spectrum of Female Hypospadias: A case series. Cureus.2024; 16(10): e 71703. Doi: 10.7759/ cureus.71703. PMID:39553133; PMCID: PMC11568794.

27.   Gabra A, Beyari BM, Al Nuwaiser SJ, Allaf SM, Alghanmi R, Alrayiqi R, et al. Outcomes of hypospadias repair based on surgical techniques: A 4- year retrospective study. Research and Reports in Urol. 2024; 16: 79-87. Doi: 10.2147/RRU.S451552.PMD:38558857; PMCID:PMC 10981871.

28.   Chukwubuika KE, Obianyo NEN, Ezomike UO. Assessment of the effect urethral plate width on outcome of hypospadias repair. J. Pediatr. Urol. 2019; 15(6): 627-31. Doi:10.1016/j.jpurol.2019.09.019.Epub 2019 Oct 1. PMID:31672475.

29.   Wenli L, Yuehong T, Dominic F, Brad F. Different outcomes of hypospadias surgery between North America, Europe and China. Nephrourol. Mon. 2012; 4(4): 609-12.

30.   Doi: 10.5812/numonthly.1853.

31.   Musa MU, Yunusa B, Mashi SA, Babagana A, Sadiq MA, Suleiman AK. Tips and Tricks in the management of Megameatus Intact Prepuce, Variant of Hypospadias.: A review article. Med. Research Archives. 2023; 11(7): 1-9. Doi: https://doi.org/10.18103/mra.V117.2.4165.

32.   Cendron M. The Megameatus Intact Prepuce Variant of Hypospadias: Use of Inframeatal Vascularized Flap for Surgical Correction. Front Pediatr.2018; 6: 55-62.Doi: 10.3389/fped.2018.00055.

33.   WU HY, Rusnack SL, Bellah RD, Plachter N, McDonald- Mcginn DM, Zackai EH, et al. Genitourinary malformations in chromosome 22q11.2. Deletion. J. Urol. 2002; 168: 2564-5.

34.   Kolon TF, Herndon CDA, Baker LA, Baskin LS, Baxter CG, Cheng EY, etal. Evaluation and Treatment of Cryptorchidism: AUA Guideline. J. Urol. 2014; 192(2): 337-45. Doi: 10.2016/j. juro. 2014.05.005.Epub May 20.

35.   Ceccarelli PL, Lucaccioni L, Poluzzi F, Biancini A, Biondini D, Lughetti L, et al. Hypospadias: clinical approach, surgical technique and long- term outcome. BMC Pediatr. 2011; 21: 523-8.

36.   Doi: https://doi.org/ 10.1186/s12887-021-02941-4.

37.   Aslam OM. Ten-years review of hypospadias surgery from a single centre. JPRAS. 2005; 58(6): 780-9. Doi: 10.1016/j.bjps.2005.01.015.

38.   Duarsa GWK, Tirtayasa PMW, Nurhadi P, Rolando J, Tarmono T, Utomo T, et al. Common Practice of Hypospadias Management by Pediatric Urologists in Indonesia: A Multi- center Descriptive Study from Referral Hospitals. Mecad.  J. Med. Sci.2019; 7(14): 2242-5.

39.   https://doi.org/ 10.3889/oamjms.2019.825.

40.   Keays MA, Dave S. Current hypospadias management: diagnosis, surgical management, and long-term patient- centred outcome. Can. Urol. Assoc. J. 2017; 11(1-2):48-53. https://doi.org/10.5489/cuaj.4386.

41.   Braga LHP, Lorenzo AJ, Pippi Salle JL. Tubularized incised plate urethroplasty for distal hypospadias: a literature review. Indian J. Urol.2008; 24(2):219-25. Doi: 10.4103/0970-1591.40619.

42.   Cook A, Khoury AE, Neville C, Bagli DJ, Farhat WA, Pippi Salle JL. A multicenter evaluation of technical preferences for primary hypospadias repair. J Urol. 2005; 174(6): 2354-7.

43.   Doi: 10.1097/01.ju.0000180643.01803.43.PMID:16280842.

44.   Mammo TN, Negash SA, Negussie T, Getachew H, Dejene B, Tadesse A, et al. Hypospadias repair in Ethiopis: A five year review. J.Sci. 2018 28(6): 735-40. Doi: 10.4314/ejhs.V2816.8.PMID: 30607090; PMCID: PMC 6308759.

45.   Khan M, Majeed A, Hayet W, Ullah H, Naz S, Shah SA,etal. Hypospadias repair: a single center experiance. Plast. Surg.Int. 2014; 1: 1-7. Doi:10.1155/2014/453039 Epub 2014 Jan 20.PMID:24579043; PMCIF: PMC3918360.

46.   Duarsa GWK, Nugroho TD, Mahadewa TGB, Yasa KP, Suryawisesa IBM, Oka AAG. Cosmetic outcome of tubularized incised plate depends on the type of hypospadias: A case control study. Bali Med J. 2016; 5(2): 355-7. Doi: 10.15562/bmj.V5i238.