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Possible Genetic Links in Development of Syringocoele

We report two sets of brothers with the diagnosis of syringocoele, raising the possibility of genetic implications in its development.

Authors: Dr Handoo Rhee, Urology Registrar, Mater Children’s Hospital, Raymond Terrace,South Brisbane,  QLD  4101

Dr David Winkle, Consultant Urologist, Mater Children’s Hospital, Raymond Terrace, South Brisbane, QLD  4101

Corresponding Author: Dr Handoo Rhee, Urology Registrar, Mater Children’s Hospital, Raymond Terrace,South Brisbane,  QLD  4101Corresponding author. +61 407766382E-mail address: [email protected]

 

Abstract
Syringocoele of Cowper’s gland is a rare congenital abnormality that can cause obstructive or irritative symptoms.  There have been many hypotheses in the past regarding the embryogenesis and aetiology of the condition.  However, due to its rarity, there have been few clues to proceed with further investigation.  We report two sets of brothers with the diagnosis of syringocoele, raising the possibility of genetic implications in its development.  A literature search has been performed to provide evidence for this theory’s plausibility.

 

Case reports
Syringocoele of Cowper’s gland is a rare congenital abnormality that can present as an obstructive or an irritative condition in the paediatric population.[1]  Cowper described bulbourethral glands in 1705 as “two small glands, placed on each side of the urethra, a little above the bulb of its cavernous body”. [2]The ducts cohere in the mid bulbar urethra, and their dilatation was first recognized as a syringocoele by Fenwick in 1896, and classified by Maizel in 1983.[3-4]  Typically diagnosed in male infants and children, it is a relatively uncommon condition.
We present two sets of brothers with voiding difficulties and later diagnosed as having syringocoele, which raises the possibility of hereditary components in its embryogenesis and aetiology.

 

Brothers 1
The older brother presented with post micturition dribbleing at age of 12.  The symptom was initially described at the age of 6.  At the time of cystourethroscopy, Type III Cowper’s duct syringocoele was identified and deroofed (Figure 1).

 

Figure 1. Cystourethroscopy of older brother 1. (a) Urethral sphincter. (b) Type III syringocoele. (c,d) Endoscopic deroofing of the lesion.

 

Eight years later, his younger brother aged 14 at the time was diagnosed with the same condition.  He was also increasingly suffering from post void dribbling.  Ascending urethrogram demonstrated a filling defect in the bulbar urethra on the inferior aspect, representing a syringocoele (Figure 2).
Figure 2. Retrograde urethrogram of untreated Cowper’s syringocoele

 

 

This was again marsupialized endoscopically with a good result.

 

Brothers 2
These brothers were both diagnosed with Type III syringocoele during cystoscopies to treat cystine stones.  Both had been diagnosed with cystinuria requiring approximately 200 procedures altogether including open pyelolithotomy, percutaneous nephrolithotomy and lasertripsy. Both patients eventually required treatment for their syringocoeles to alleviate developing symptoms.

 

Discussion
Cowper’s gland cyst or syringocoele is a rare condition that is well documented and classified.  Unfortunately, its embryogenesis and aetiology are not well understood.  We performed extensive literature search via PubMed, Ovid, Sciencedirect, Google Scholar and Elsevier databases to find 43 case reports since 1983 when Maizel described and classified the lesion.  The collected case reports described 149 patients in total.

 

History of Understanding Syringocoele
Many investigators have attempted to delineate the aetiology of syringocoele.  Lebowitz (1978), together with Fenwick’s description in 1896, stated that the cystic development in the bulbourethral duct was due to stenoses of the ductal orifice(s) from urethritis or other inflammatory disease. [5] However, due to the lack of convincing evidence, the focus turned to a congenital aetiology as more patients presented from a very early age without the history of infections or instrumentation.  Cook (1961) hypothesised that the cysts in the glands were more common when there is anomalous development of the ducts, such as when the paired Cowper’s ducts have joined to form a single distal duct. [6]  So far, there have been no reports to develop the hypothesis.

 

Associations of Syringocoele
Syringocoele has been described with other conditions, although without any consistency.  Some of the conditions associated with syringocoele include urethral diverticulum, anterior urethral valves, posterior urethral valves, ureterocoele, acontractile bladder and Cobb’s collar. [7-11]  Recently Lo described syringocoele in a 5 week old boy with posterior urethral valves, and bilateral small simple single system ureterocoeles. In this report, we described a pair of brothers both with cystinuria and syringocoele.  There are no other reports of such association.

 

Syringocoele in Animals
In an attempt to explain the aetiology of our serendipitous findings, the literature search was extended to include experimental animals.  Kiupel et al (2000) described an extremely high incidence of syringocoele in certain inbred mice colonies.  The article described the necropsy report of particular mice strains (SJL/J and RBF/DnJ), where cystic bulbourethral glands were found in 83.8% of mice, without other significant abnormalities. [12] A high incidence of bulbourethral gland cysts and ductal dilatations were also found in certain strains of bulls and sheep (up to 26%). [13-14] Some have implicated the oestrogenic effects of particular clover pastures in development of low fertility and high incidence of syringocoeles in wethers.  In Western Australia, the subterranean clover content of pastures is strictly monitored to maintain fertility and well being of animals. [15]

 

Transforming growth factor and the bulbourethral gland
The bulbourethral gland is often studied in isolation to demonstrate the effects of various agents on androgen dependent development.  Observing the ultrastructural features of the human bulbourethral glands demonstrate complex parenchyma made up of secretory tubules and alveoli lined by mucoid cells and excretory duct systems, supported by stroma and smooth muscle cells. [16] These are the result of complex interactions between dihydrotestosterone, mesonephric duct and urogenital sinus, which triggers epithelial-mesenchymal reactions.
Transforming growth factor (TGF) beta is an agent that appears to aid epithelial-mesenchymal interactions in many organs including the prostate and Cowper’s glands. TGF-Beta related mutations have been associated with disturbances in the regulations of immunity, cancer, heart disease, Marfan’s syndrome, and Loeys-Dietz syndrome. [17]
Dunker and Aumuller in 2002 [18] described development of cystic dilatation of Cowper’s gland in mutant mice with a deletion of transforming growth factor beta subtype 2 gene.  They postulated that the hyperplasia of Cowper’s gland epithelium and cystic dilatation is the result of disturbance in epithelial-stromal interactions secondary to reduced TGF-Beta subtype 2 level in heterozygous mutants and eventual decrease in apoptosis of these cells. However, given the in-utero mortality associated with homogenous TGF-Beta subtype 2 mutation, and its roles in a variety of cells, it is unlikely to be the entire cause for the development of isolated syringocoele without other medical conditions. [19-21]

 

Cystinuria
Cystinuria is an autosomal recessive disorder of cystine and dibasic amino acid transporters found in the kidneys and small intestine.  SLC3A1 and SLC7A9 are gene mutations that cause different types of cystinuria.  Cystinuria is not known to cause hormonal or stromal-epithelial interaction abnormalities.  During the literature search, no articles that links mutations in TGF and cystinuria could be found.

 

Future Directions
Syringocoele appears to develop in a variety of environments across different species.  The literature search has provided us with some clues to the future directions in understanding the aetiology of syringocoele.  They include genetic mutations that affect stromal-epithelial interactions with or without the effects of disturbances in hormone balance and amino acid transporting pathways.

 

Conclusion
Historically, there has been significant interest in understanding the aetiology of syringocoele.  Due to the rarity of the reports, there have been little clues to direct research.  Although these cases are not proof enough to firmly associate genetic implications on the development of syringocoele, the report does provide some clues for future research.

 

References
 
1. Campobasso P, Schieven E, Fernandes EC. Cowper’s syringocoele: an analysis of 15 consecutive cases. Arch Disease Child. 1996; 75: 71-73.
2. Cowper W: Two new glands near the prostate glands, with their excretory ducts, lately discover’d.  The Philosophical Transactions and Collections, London 3: 194, 1705.
3. Fenwick EH. Retention cysts of Cowper’s glands as a cause of chronic gleet, spasmodic stricture, organic stricture, extravasation of urine and of so-called “false passages” in the bulbous urethra.  Br Med J.  1896; 1:4.
4. Maizels M, Stephens FD, King LR, et al.  Cowper’s syringocoele: a classification of dilatations of Cowper’s gland duct based upon clinical characteristics of 8 boys. J Urol.  1983; 19: 129: 111-114.
5. Colodny AH, Lebowitz RL. Lesions of Cowper’s ducts and glands in infants and children. J Urol. 1978; 11:4:321-325.
6. Cook FE, Shaw JL. Cystic anomalies of the ducts of Cowper’s glands. J Urol. 1961; 85:659.
7. Salas RJ, Corominas CI. Diverticulum of the anterior urethra or syringocoele of the Cowper glands. Anal Esp Ped. 1989; 31:6:605-6.
8. Lo A, Upadhyay V, Teele R. Syringocoele of the bulbourethral duct with additional lower genitor-urinary anomalies. Pediatr Radiol. 2011; 41: 1201-1204.
9. Turker Koksal I, Erdogru T, Usta M, Ates M, Baykara M. Unexpected presentation of syringocele: Acontractile bladder. Urol Intern. 2003; 71:2:222-3.
10. Mutlu N, Culha M, Mutlu B, Acar O, Turkan S, Gokalp A. Cobb’s collar and syringocoele with stone. Intern J Clin Prac. 1998; 52:5:352-3.
11. McLellan DL, Gaston MV, Diamond DA, Lebowitz RL, Mandell J, Atala A et al. Anterior urethral valves and diverticula in children: a result of ruptured Cowpers duct cyst? Brit J Urol Intern. 2004; 94: 375-378.
12. Kiupel M, Brown K, Sundberg J. Bulbourethral gland abnormalities in inbred laboratory mice. J Exp Anim Sci. 2000; 40: 178-188.
13. Campero C, Ladds P, Thomas A.  Pathological findings in the bulbourethral glands of bulls.  1988. Austr. Vet. J. 65: 241-244.
14. McEntee K, 1990. Reproductive pathology of Domestic Mammals. Academic Press, Inc., San Diego.
15. Department of Agriculture.  Farmnote No 41/2005 [newsletter]. Keith Croker et al; Government of Western Australia; 2007.
16. Riva A, Usai E, Cossu M, et al. Ultrastructure of human bulbourethral glands and of their main excretory ducts. Arch Androl. 1990; 24: 177-184.
17. Carlson B. Human Embryology and Developmental Biology, 4th Edition. Philadelphia, Pennsylvania: Mosby/Elsevier, 2009.
18. Dunker N, Aumuller G. Transforming growth factor-beta 2 heterozygous mutant mice exhibit Cowper’s gland hyperplasia and cystic dilations of the gland ducts (Cowper’s syringocoeles). J Anat. 2003. 201: 173-183.
19. Sims Lucas S, Caroona G, Dowling J, et al. Augmented and accelerated nephrogenesis in TGF-beta2 heterozygous mutant mice. Pediatr Res. 2008; 63:6:607-12.
20. Dunker N, Krieglstein K. Targeted mutations of transforming growth factor-beta genes reveal important roles in mouse development and adult homeostasis. Eur J Biochem. 2000; 267: 6982-6988.
21. Sanford LP, Ormsby I, Gittenberger-de Groot A, et al. TGF Beta 2 knockout mice have multiple developmental defects that are non-overlapping with other TGF beta knockout phenotypes. Dev. 1997; 127; 2659-2670.

Date added to bjui.org: 23/01/2012


DOI: 10.1002/BJUIw-2011-117-web

Fournier’s Gangrene Complicating Diverticular Colovesical Fistula

We present an 82 year old gentleman who developed Fournier’s gangrene as a result of a colovesical fistula secondary to diverticular disease of sigmoid colon. 

 

Authors: Mr. Yao Pey YONG, SHO General Surgery, Doncaster Royal Infirmary.

Mr. Vivek Kumar, Consultant Urology, Doncaster Royal Infirmary

 
Corresponding Author: Mr. Yao Pey YONG, E:mail: [email protected]

 

Abstract
Colovesical fistula has been described as a cause of Fournier’s gangrene but it is a rare occurence. We present an 82 year old gentleman who developed Fournier’s gangrene as a result of a colovesical fistula secondary to diverticular disease of sigmoid colon. The unusual findings during debridement were gangrene of the urethra and testicles. The patient subsequently had a Hartmann’s procedure and closure of the bladder defect. Fournier’s gangrene is a urological emergency that requires a multidisciplinary team approach to optimise patient outcome.

 

Introduction
 
Fournier’s gangrene is necrotising fasciitis of the male genitalia. It was first described by Avicenna in 1025 but was named after a French professor in syphilology in 1883 after he described a series of five previously healthy young men suffering from a rapidly progressive gangrene of the penis and scrotum without apparent cause. We present an unusual case of Fournier’s gangrene complicating colovesical fistula secondary to sigmoid diverticulosis, with urethra and bilateral testicular involvement. Although diverticular disease is known to be one of the causes of Fournier’s gangrene, the authors have yet to come across any similar cases in the literature during the writing of this manuscript.

 

Case Report
 
An 82 year old gentleman was admitted through the Accident and Emergency Department with a one day history of lower abdominal pain and rigors. His past medical history included osteoarthritis, benign prostatic hypertrophy and colovesical fistula secondary to sigmoid diverticulosis, which was diagnosed one month previously on computerised tomography (CT) scan.

 

Figure 1. CT scan

 

 

He was awaiting elective surgery due a week from the date of admission. His vital signs were as follows: temperature 38°C, blood pressure 120/65mm Hg, heart rate 108/minute, respiratory rate 24/minute and oxygen saturation 98% on room air. Physical examination revealed tenderness across the lower abdomen with minimal voluntary guarding. Initial laboratory studies revealed the following values: haemoglobin 11.1 g/dl, white cell count 7.4 x109/L, C-reactive protein 65.5 mg/L, amylase 54 U/l, creatinine 143 umol/L, urea 12.8 mmol/L, sodium 133 mmol/L and potassium 4.6 mmol/L. Urinalysis revealed protein, leucocytes and nitrites. A diagnosis of acute renal failure and urinary sepsis secondary to colovesical fistula was made. He was fluid resuscitated and commenced on intravenous antibiotics.
On day five of admission, he complained of pain in the perineal region. Physical examination revealed swelling of his penis, scrotum and perineum.

 

Figure 2. Physical examination 

 

 

Blood cultures taken on admission revealed an Enterococcus and antibiotics were changed after discussion with the Department of Microbiology. Repeated physical examination the following day revealed patchy necrotic penile skin. A diagnosis of Fournier’s gangrene was made and an immediate urological referral made. Repeated laboratory studies at this point revealed the following values: haemoglobin 8.7 g/dl, white cell count 27.8 x109/L, creatinine 140 umol/L, urea 11.0 mmol/L, sodium 134 mmol/L and potassium 4.1 mmol/L.
The patient had a  metabolic acidosis and was fully resuscitated prior to the operation. He underwent four separate operations. The initial procedure was an extensive debridement of perineal, scrotal and penile skin. Surprisingly, deeper structures were involved as well, including the full length of the urethra, and perineal fat. The proximal urethra was ligated. Bowel diversion was deferred due to poor general condition of the patient.

 

Figure 3. The initial procedure was an extensive debridement of perineal, scrotal and penile skin.

 

 

He was brought back to the operating room the next day for further debridement, Hartmann’s procedure, closure of bladder defect and suprapubic cystostomy. He had two further debridements subsequently. Negative pressure wound therapy was applied to the perineal wound (VAC therapy).

 

Discussion
 
Fournier’s gangrene is an acute and potentially fatal, deep-seated bacterial infection with secondary necrosis of the skin and soft tissue of the scrotum, perineum and abdominal wall. The infection spreads very rapidly and progressively along the deep fascia.[1] The risk factors include old age, diabetes, alcoholism, malignancy, immunosuppression and any conditions that involve the genitourinary tract or gastrointestinal tract that present potential portals of entry for opportunistic bacteria. A primary source can be identified in 95% of cases.[2] However, Efem[3] argues that Fournier’s gangrene is never idiopathic, and when the cause is not found, it implies that the clinician is unable to determine the cause because the portal of entry may have been so trivial that it was overlooked. In our case, the patient’s risk factors were old age and colovesical fistula secondary to sigmoid diverticulosis.
The symptoms of rigor and discomfort in the external genitalia were initially overshadowed by lower abdominal pain and a positive urinalysis. As the patient had a prior diagnosis of colovesical fistula secondary to diverticulosis, a preliminary diagnosis of lower urinary infection was made on admission. Garcea [4] in his study, showed the most common presenting symptoms of colovesical fistula were pneumaturia (90.1%), faecaluria (76.2%), abdominal pain (70.1%) and recurrent urinary tract infection (66.7%). Diverticulosis is known to be the most common cause of colovesical fistula and occurs mainly in the sixth and seventh decade of life. It is reported that male to female ratio is between 5 to 1 and 1.5 to 1.[5] This is thought to be due to the protective effect of the female reproductive organs as a physical barrier to fistulisation from diseased bowel.[5,6,7] Studies have shown that 50% of women have had a hysterectomy before the development of colovesical fistula. Other causes include Crohn’s disease, malignancy, radiation therapy, trauma and rarely, appendicitis.
The identification and diagnosis of Fournier’s gangrene is challenging in the early stages. Hefny[1] reported only 4 out of 11 patients in his study had a correct preliminary diagnosis. Patients usually present with an insidious onset of pruritis and discomfort in the external genitalia. This is followed by pain, swelling and systemic symptoms including rigors. When gangrene develops, pain may actually subside due to destruction of nerve tissue. The bacteria involved are usually both aerobes and anerobes, with E. coli the predominant aerobe and Bacteroides the predominant anaerobe.[8] They act synergistically with enzymes to invade and destroy fascial planes. As obliterative endarteritis develops, cutaneous and subcutaneous vascular necrosis leads to localised ischemia and further bacterial proliferation.[8] The infection in Colles fascia may spread to the penis and scrotum via Buck’s and Dartos fascia or to the anterior abdominal wall via Scarpa’s fascia. Colles fascia is attached to the perineal body and urogenital diaphragm posteriorly and to the pubic rami laterally, thus limiting progression in these directions.[8] As the infection spreads, induration worsens and bullae are formed. The overlying skin becomes anaesthetised and necrotic at a very late stage.
Fournier’s gangrene is primarily a clinical diagnosis. However, radiological evaluation may aid to reach the diagnosis if in doubt. Plain films are commonly performed and may reveal air in the soft tissue. This is not pathognomonic but nevertheless should alert the clinician to the possibility of necrotising fasciitis.[9] Ultrasonography may be a more useful diagnostic tool as it may show a thickened scrotal wall and peritesticular fluid.[10] Echogenic shadows with ring down artifact represent air, which is the ultrasonographic hallmark of Fournier’s gangrene.[10,11] An infected hydrocoele may mimic Fournier’s gangrene as the testes may be surrounded by fluid. However, no air should be seen in the subcutaneous tissues.[10] CT findings of Fournier’s gangrene include asymmetric fascial thickening, subcutaneous emphysema, fluid collections and abscess formation.[10] Although CT provides higher specificity, ultrasonography is inexpensive and may provide similar findings to those of CT. Magnetic resonance imaging may be used to aid the diagnosis of Fournier’s gangrene, however the evidence is limited.
Textbooks often describe sparing of the testes as their blood supply is directly from the aorta. However, some authors[9,12,13,14] have argued that the incidence of patients requiring orchidectomy for non-viable testes is up to 21%, but in most cases the indications for orchidectomy were pre-existing epididymo-orchitis, scrotal abscess and extensive tissue damage in the surrounding scrotum, groin, and perineal area. Interestingly, Gupta et al[15] did report a case of bilateral testicular gangrene in Fournier’s gangrene. Erke16 stated that the testicular involvement was rare and that when it occurs it indicates a retroperitoneal or intra abdominal source of infection. In our case, the patient had bilateral orchidectomy as the gangrenous tissue was extensive and the testes appeared non-viable. In addition it is unusual to find gangrene of the urethra. In this particular case, the whole of the urethra was gangrenous.
A review done by Elliott et al[17] stated that colostomy was not routinely performed even when the infection involved the perianal area as Fournier’s gangrene can ascend the anterior abdominal wall. Withholding colostomy until the infection has been halted, rather than performing it at initial presentation, may be prudent.[17] Hartmann’s procedure and bladder repair was performed to disconnect the fistula as it was the source of infection in our patient. Although there is limited randomised trials to clarify the role and efficacy of VAC therapy, it appears to be an effective tool for post-operative wound management in Fournier’s gangrene.[18]

 

Conclusion
 
Fournier’s gangrene is a potentially fatal, rapidly spreading necrotising soft tissue infection. Clinicians should have a high index of suspicion in patients who carry risk factor(s). Early diagnosis, resuscitation and aggressive debridement are paramount to a favourable outcome.

 

Conflict of Interest
The authors have no competing interests.

 

References
 
1. Hefny AF, Eid HO, Al-Hussona M, et al. Necrotising fasciitis: A challenging diagnosis. European Journal of Emergency Medicine 2007;14:50–52.
2. Champion SE. A case of Fournier’s gangrene. Urol Nurse 2007;27:296-299.
3. Efem SE. The features and aetiology of Fournier’s gangrene. Postgrad Med J 1994;70:568-571.
4. Garcea G, Majid I, Sutton CD, et al. Diagnosis and management of colovesical fistulae: Six-year experience of 90 consecutive cases. Colorectal Disease 2006;8:347-352.
5. Hsieh JH, Chen WS, Jiang JK, et al. Enterovesical fistula: 10 year experience. Chin Med J (Taipei) 1997;59:283-8.
6. Abeshouse BS, Robbins MA, Gann M, et al. Intestinovesical fistulas: Report of seven cases and review of the literature. JAMA 1957;164:251-7.
7. Carpenter WS, Allaben RD, Kambouris AA. One-stage resections for colovesical fistulas. J Urol 1972;108:265-7.
8. Marynowski MT, Aronson AA. Fournier Gangrene in Emergency Medicine. https://emedicine.medscape.com/article/778866-overview#a0104 (access 27 May 2011)
9. Smith GL, Bunker CB, Dinneen MD. Review: Fournier’s gangrene. British Journal of Urology 1998;81:347-355.
10. Safriel Y, Cohen HL, Torrisi J. Ultrasound Imaging of Scrotal Wall Thickening and Its Significance in the Diagnosis of Fournier’s Gangrene in Older Men. Journal of Diagnostic Medical Sonography 2000;16:29-33.
11. Tsai MJ, Lien CT, Chang WA, et al. Transperineal ultrasonography in the diagnosis of Fournier’s gangrene. Ultrasound Obstet Gynecol 2010;36:387-391.
12. Hejase MJ, Simonin JE, Bihrle R, et al. Genital Fournier’s gangrene: Experience with 38 patients. Urology 1996;47:734-9.
13. Hohenfellner M, Santucci RA. Fournier’s gangrene. In: Heyns CF, Theron PD (Eds.) Emergencies in Urology. Germany: Springer; 2007. p50 – 59.
14. Ayan F, Sunamak O, Paksoy SM, et al. Fournier’s gangrene: A retrospective clinical study of forty one patients. ANZ J Surg 75:1055-1058.
15. Gupta A, Dalela D, Sankhwar SN, et al. Bilateral testicular gangrene: Does it occur in Fournier’s gangrene? Int Urol Nephrol 2007;39:913-915.
16. Eke N. Fournier’s gangrene: A review of 1726 cases. Br J Surg 2000;87(6):718-728.
17. Elliott DC, Kufera JA, Myers RAM. Necrotizing soft tissue infections: Risk factors for mortality and strategies for management. Annals of Surgery 1996;224(5):672-683.
18. Tucci G, Amabile D, Cadeddu F, el al. Fournier’s gangrene wound therapy: Our experience using VAC device. Langenbecks Arch Surg 2009;394:759-760.

 
Date added to bjui.org: 27/09/2011


DOI: 10.1002/BJUIw-2011-071-web

 

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