SERTOLI CELL ONLY SYNDROME AND GENETICS

Genetic work up of all males with azoopsermia and severe oligospermia is necessary. Severe male factor infertility may have a presently identifiable genetic basis. Y chromosomal microdeletions (e.g., an AZFc microdeletion), karyotypic anomalies (e.g., Klinefelter Syndrome), and mutations in both alleles of the cystic fibrosis transmembrane conductance regulator (CFTR) gene may be found, depending upon the etiology of the reproductive compromise.A genetic abnormality is identified in 1/4 th of the men with extreme oligozoospermia and azoospermia. Application of ICSI in these couples can result in offspring with an enhanced risk of unbalanced chromosome complement, male infertility due to the transmission of a Y-chromosomal microdeletion, and cystic fibrosis if both partners are CFTR gene mutation carriers. Genetic testing and counselling is clearly indicated for these couples before ICSI is considered.

We get many patients with non obstructive azoospermia. In all cases of non obstructive azoospermia , Sertoli Cell Only Syndrome is an important entity as the sperm harvesting is a real challenge during TESA/Microdissection TESE. The Sertoli cell only syndrome (SCOS) also known as germinal cell aplasia is a histological diagnosis which reveals seminiferous tubules with a complete absence of all germ cells and the presence of only, as the name suggests, sertoli cells .

The cause is likely multifactorial: congenital absence of germ cell, chemicals and gonadotoxins, klienfelters syndrome are some known causes of SCOS. Y microdeletions have also been identified as the causative factors for SCOS. In the West upto 34.5% of SCOS patients were found to have associated Y chromosome microdeletion .This association has been seen with deletions of DBY (dead box on Y) gene in AZFa (azzoospermia factor a) region, microdeletions in AZFb region and DAZ (deleted in azoospermia) microdeletion in AZFc regions of the Y chromosome.Patients normally present with small to normal sized testis and azoospermic semen specimens. Phenotypically these patients are normally virilized males without gynaecomastia. Plasma FSH and LH levels are often but not invariably elevated because of the absence of germ cells, whereas plasma testosterone are normal.

Despite an apparent absence of germ cells on histologic examination, spermatozoa may be recorded in upto 50% of patients undergoing attempts at testicular sperm extraction (TESE).

MICRODISSECTION TESE CAN IMPROVE THE YIELD OF SPERM RETRIVEAL.wE ROUTINELY DO THSI PROCEDURE IN OUR HOSPITAL

This statistic is important as these patients could be candidates for In Vitro Fertilization (IVF) with Intra Cytoplasmic Sperm Injection (ICSI). Study of Y chromosome microdeletions has gained importance in this group of patients since artificial reproduction techniques (ART) can be used for them. ART bypasses the critical natural selection barriers that normally prevent genetic disease transmission. One of the major causes of concern with the use of ICSI in male infertility and nonobstructive azoospermia is the vertical transmission of genetic abnormalities to the male offspring. In the genetic abnormalities the Y microdeletions have been found in 1 -55 % cases.

                

CYSTOSCOPY AND URETHRAL DILATATION FORTH URETHRAL STENOSIS: STEPS

WE USE 20 Fr TWO WAY FOLEYS FOR A PERIOD OF 24 HOURS POST DILATATION

UROFLOWMETRY IN FEMALES

Bladder outlet obstruction is not as commonly seen in females as in males. The usual causes are Postmenopausal urethral stenosis, severe cystocele leading to kinking of bladder neck, urethral mucosal prolapse, post hysterectomy status, urethral caruncle, urethral carcinoma and other neoplastic conditions in the adjacent organs.

Uroflowmetry is a good screening test for BOO in both males and females. A recent study conducted by Dr Barapatreetal on in healthy women suggested the normal Bladder Voided volumes to be around 290 ml +/- 160 ml and Qmax Around 23.06 ml/s +/- 9.4 ml/s. 

One of the uroflowmetry showing severe BOO in a female presenting with Recurrent UTI is displayed below.

 

TESA: STEP BY STEP DEMONSTRATION

THE INSTRUMENTS REQUIRED FOR THE TESA

TESA (testicular sperm aspiration), which is performed by sticking a needle in the testis and aspirating fluid and tissue with negative pressure.

We routinely perform TESA in our hospitals.Our approach has been to go for Microdissection TESE in cases of small atrophic testes with high FSH or failed TESA.

We use 18 G scalp vein and we pierce the testis and move the needle in to and fro fashion at the same time the assistant withdraws a negative suction with a plunger of 20 cc syringe.

The testicular tissue beutifully comes out and can be used for IVF purpose/ cryopreservation and also for the histopathological analysis.We stress upom all the patients for cryopreservation.In cases where the testis is smaller in volume with higher FSH ; we go for multiple TESA .The success of TESA has been excellent even in cases of Non Obstrictive azoopsemria with borderline high FSH.This is a good alterbative for Microdissection TESE where the microscopy or expertise for the same is not available.

LETTER OF PRAISE FROM THE PATIENT

The Ramayya Pramila Hospital was established in 1970 by Dr.G.P.Ramayya as a first Specialized Uro-Surgical Hospital in the State of AP. Dr GP Ramayya was the Professor of Surgery Osmania Hospital and Principal Gandhi Medical College. He was also the personal surgeon to the Nizam of Hyderabad. 

Dr Ramesh Ramayya , the present Chairman and Managing Director of the Ramayya Pramila Urology Hospital has pioneered and was instrumental in introducing many Modern State of the Art Minimal Invasive, Non Invasive and Natural Orifice Urological procedures like PCNL, HIFU, LASER, RIRS, URS since his return from the UK in in 1986.

RamayyaPramila Urology Hospital has a strong four member highly qualified and experienced team of Urologists. Apart from Dr Ramesh Ramayya, Dr Naveen Acharya, Dr Vamsi Krishna, Dr Abdul Fathah offer a 24/7 Urology Service. 

In addition Dr Siva Prasad an experienced Anaesthetist trained at the prestigious Post Graduate Institute in Chandigarh is the full time Clinical Director to ensure standardized preoperative Care. The hospital audits patient care through an electronic platform Excelicare to ensure process driven, protocol based Urology HealthCare Delivery to ensure excellent outcome.

Our approach is patient centric.The patient once walks in the hospital immediately gets attention from the consultant urologist 24X7 ; we can proudly claim that the patient is seen within 15 minutes of the arrival at the hospital by the consultant urologist and advised due treatment.

We regularly get patients from outside state and India.The fame of the hospital is mainly due to word of mouth by previously treated patients rather than marketing. 

one such patient gave a plenty of accolades to our chief urologist and rendered a letter of acknowledgement for the excellent treatment that he got here.

FRENULOPLASTY: Z PLASTY TECHNIQUE

A frenuloplasty is the release of an abnormally short frenulum of the penis that restricts normal retraction of the erection or causes pain while retraction of the prepuce.

TIGHT FRENULUM

This may be a a congenital(since birth) or complication of circumcision because of the scarring effect . The goal of treatment is to allow normal retraction of the foreskin. Circumcision may relieve this condition but is not indicated solely for treating tight frenulum. Many people have fear loosing the foreskin thinking that it will reduce the sexual pleasure.We have seen people opting for frenuloplasty over circumcision for fear of loosing foreskin. 

Z-plasty is a versatile reconstrcutuve technique that is used to improve the functional and cosmetic appearance of scars. It can elongate a contracted scar or rotate the scar tension line. The middle line of the Z-shaped incision (the central element) is made along the line of greatest tension or contraction, and triangular flaps are raised on opposite sides of the two ends and then transposed. The length and angle of each flap are usually the same to avoid mismatched flaps that may be difficult to close.

RAISING Z INCISION WITH VERTICAL ARM MATCHING THE FRENULUM BREVE

INCISING ALONG THE HORIZONTAL ARMS.WE MAKE THE ARMS AT AN ANGLE OF 45%

WHOLE PROCEDURE IN A DIAGRAMMATIC FASHION 

We conduct the surgery under sedation and penile block and it is conducted as an OPD procedure and the patient is allowed to go home the same day with anti-inflammatory medications to take care of the pain or swelling.

HOW WE DO PCNL: A STEP BY STEP DEMONSTRATION

The first step we do is RGP followed by injection of air to demonstrate the posterior group of calyces.

THE POSITION WE PREFER IS SIMPLE PRONE WITHOUT ANY KIDNEY BRIDGE 

The desired calyx is marked with tip of the puncture needle and the axis of the puncture is decided.
Then the needle is progressed along the axis of the needle.We use only 0 degree view of the C-Arm and puncture the desired calyx.

We usually put the guide wire in the ureter by manipulating the needle and then use the screw dilator for dilatation.Once the screw dilator is the system ; then we put the guide rod and over that we do single step dilatation with 24 Fr Amplatz dilator followed by 24 Fr Amplatz sheath and use mini nephroscope 15 Fr ( we use either pneumatic or Holmium LASER for stone fragmentation )

IMPORTANCE OF CYSTOSCOPY IN RECURRENT UTI IN POSTMENOPAUSAL WOMEN

A 70-year-old postmenopausal woman presented with a history of high grade fever and dysuria. She was a diabetic with fairly good control of sugars.We evaluated the patient - her USG KUB was normal and urine culture was E.Coli sensitive to imipenem group of drugs. 

The patient was started on meropenem and catheterised.Her sugar were brought to the normal with sliding scale of insulin regimen.

She was treated for recurrent UTI s with multiple antibiotics that's the reason probably she was having multi-drug resistant bacteria. 

The woman underwent cystoscopy under sedation. There was urethral stenosis which were dilated with Hegar’s dilators till Hegar 11/14. After dilatation we usually keep the Foley's catheter (20 Fr) for 24 hours.

CYSTOSCOPY AND URETHRAL DILATATION 

The patient was given a 5 days course of meropenem and was instructed to apply estrogen cream locally twice a week for 6 weeks. She was given nitrofurantoin as long term suppressive antibiotic for long term duration.She was also started on cranberry extract and D-Mannose tablets to prevent recurrence of UTI.

Etiological factors for recurrent UTI in adult women are senile vaginitis, hypoestrogenism, lack of sexual activity, and these factors leading to urethral stenosis.

CRANBERRY JUICE OR EXTRACT IS BENEFICIAL IN PREVENTING RECURRENCE TO SOME EXTENT

Treatment is most often conservative in the form of antibiotics and estrogen cream for hypoestrogenism and atrophic mucosa. It is beneficial to start the patient on long term suppressive antibiotics if the lady suffers from recurrent Febrile UTI.

WE USUALLY PREFER THE OESTROGEN CREAM FOR ALL POST MENOPAUSAL PATIENTS 

Cystoscopy is essential thing in recurrent febrile UTI as it can detect atrophic hypo-estrogenic vaginitis and potentially cure urethral stenosis. 

MICTURITION SYNCOPE: A RARE CASE

A 23 year old patient came with history of syncope while passsing urine three times in a period of 2 months.The first episode happened when he got up during the sleep.

The other two episodes happened without any provokative factors.

His routine biochemical and hematological tests were normal.ECG and ECHO tests were too normal.His VMA levels in urine were normal.

His USG KUB and uroflowometry were within the normal limits.

Finally he was given reassurance and advised some life style modifying factors.

To sit while urinating 

To sit on the edge of the bed for a while before getting up and going to the toilet 

To avoid micturition while feeling sleepy. 

Review of the literature:

Micturition syncope fainting shortly after or during urination.

The patients can have concomitant nausea; vomiting and can feel dizzy before loss of consciousness. Some people can have syncope following or during vomiting, defecation or coughing (About 61% with micturition syndrome also suffered from other kinds of syncope).

It is more common in male. It is responsible for 2.4 to 8.4 percent of all syncope. The events often occur at night or after awaking. A combination of postural hypotension and straining is always found in such cases. Defecation syncope is a relatively rare disorder that occurs typically in middle-aged or older individuals and affects women more often than men. More than one third of patients with defecation syncope die within 2 years of complications of their underlying diseases.

The mechanism of micturition syncope is still unknown but believed that it is related to vasovagal syncope.

During micturition there is increased vagal tone as a result from straining (Valsalva maneuver) which results in bradycardia leads to syncope due to decreased cerebral blood flow. As clinician we should rule out cardiac causes, anemia and while does the patient strain at urination (whether he has underlying stricture disease,

PROSTATOMEGALY especially on alpha blockers).

A rare tumor –pheochromocytoma of the bladder can lead to such condition. In all cases it would be safer to do imaging of the bladder, check for urine VMA as a routine.

• Diagnosis mainly relies on patient history but other investigation like ECG, echocardiogram, TMT /Holter monitoring and blood tests need to rule out other diseases.
• There is no specific treatment for micturition syncope. General advice to men who have a micturition syncope includes: 
• To sit while urinating

• To sit on the edge of the bed for a while before getting up and going to the toilet
• To avoid micturition while feeling sleepy
• If it is happening again and again and interfering with the life of the patient then beta blockers can be administered.
• Some urologists prescribe SSRI /antidepressants in such conditions .it may offer some help.
• If any sinus block is present cardiology intervention in the form of pace makers may be needed in rare cases.

Direct trocar insertion in Laparoscopic cholecystectomy

The direct trocar insertion technique (DTI) for the creation of pneumoperitoneum has been described as an alternative to open and Veress needle (VN) techniques. 

After adequate patient relaxation, a 10-mm skin incision is made at the level of the umbilicus to allow the introduction of a 5-mm trocar . With the patient in a supine position, the abdominal wall is elevated by grabbing the portion of the abdominal wall around the proposed site of the entry of the trocar. Care is taken to make the incision length slightly greater than the diameter of the trocar, and all layers of skin must be cut down to the peritoneum through the entire length of the umbilical incision. These simple manoeuvres allow easier introduction of the trocar with minimal force and maximal control. 

In contrast to Veress needle insertion, where one can feel the penetration through the fascia and peritoneum separately, a distinct and single “pop” signifies that the trocar has pierced the fascia and peritoneum. Once a 5 mm trocar is placed and pneumoperitoneum is established we change the trocar to 10 mm trocar.

The contra-indication that we follow is previous abdominal surgery as adhesions will make DTI risky.In that case we follow open technique.

    

MINI PCNL FOR LOWER CALYCEAL CALUCLUS

A 55 year old gentleman with no comorbidties presented with lower calyceal calculus blocking the infundibulum of lower calyceal group of calyces.The stone was about 2 cm in size.

Stone in right lower calyx blocking the infundibulum  of lower calyceal  group

RGP

meticulous puncture of lower posterior calyx and the guide wire being placed in the ureter 

Mini PCNL sheath being placed

The patient was taken up for Mini PCNL and stone fragmentation was done with Holmium LASER lithotripsy.

Cranberry juice/ extract in prevention of Urinary tract Infections in women

As many as 50–80% of women in the general population acquire at least 1 UTI during their lifetime.— Approximately 7% of patients require hospitalization.Around — 20–30% of pregnant women with asymptomatic bacteriuria subsequently develop pyelonephritis. Between 1 year and ~50 years of age, UTI and recurrent UTI are predominantly diseases of females.

It has been estimated that around 8.3 million doctor visits all over the world are for the UTI. It constitutes the second most common type of infection in the body.

The most common strategy employed for UTI prevention is chemoprophylaxis where low dose continuous suppressive antibiotics are administered to the patient.A natural protection against a broad range of uropathogenic microorganisms is there now available in the form of cranberry juice or its extract.

Many a times UTI in pregnancy poses a problem and also lactating mothers because there are limited antibiotics that Urologist can try.In such cases these natural protectants can be a handy tool for urologists.

There are now medications available where cranberry juice extract is combined with D-mannose.

Cranberry juice extract condensed tannins or proanthocyanidins exhibiting anti-adhesion properties.The juice can be taken around 250 ml/day.As it a low calorie drink; it is convenient for females who are diet conscious.This drink is available in India commonly in supermarkets.

The repeated UTIs are due to adherence of bacteria to the cells of the genitourinary tract with their hair like fimbriae. D. Mannose works by coating free floating E. Coli and prevents it from attaching to the epithelium of the genitourinary tract.

It has been established that daily dose of cranberry extract is effective in reducing the UTIs in females.(J Nutr.2010;104:1181-89)

ESWL: TRIAD OF GOOD MACHINE, PRESENCE OF UROLOGIST TO AID IN FOCUSSING AND SEDATION TO DELIVER MAXIMUM POSSIBLE INTENSITY OF WAVES ALWAYS WORKS

ESWL is the least invasive of the commonplace modalities for definitive stone treatment, but provides a lower stone-free rate than other more invasive treatment methods, such as ureteroscopy with LASER lithotripsy/RIRS  or PCNL. The passage of stone fragments may take a few days or a week and may cause residual fragments to remain in kidney needing multiple sessions or sometimes auxilliary procedures like RIRS or PCNL.

The success or failure of ESWL depend upon many factors- density of the stone, how good is the focussing and the strength of the waves(intensity). 

We are proud to have Dornier alpha lithotripsy machine.The success rate of ESWL in our hospital is better.

We also ensure a fully trained and competent technician to do it and being monitored by one of our in house urologists.

We do ESWL under sedation to avoid pain and movements so that focussing would be good and also the intensity of the shock waves can be increased to the optimum. The vitals and colour of urine are being monitored continuously.

   

SIMULTANEOUS BOARI FLAP AND URETEROCALYCOSTOMY IN A CASE OF GUTB

A 38 year old lady presented to us with lower urinary tract symptoms and right flank pain for 15 days.She had history of undergoing left nephrectomy in 2002.The histopathological evaluation had shown granulomatous nephritis.After the surgery; she was advised scrupulous follow-up but she could not regularly visit the surgeon.On presentation to our hospital; she had deranged creatinine(2.7 mg%) with sonographic evidence of right moderate hydroureteronephrosis. Non contrast CT scan evaluation confirmed the sonographic findings.She was taken up for retrograde pyelography and stenting. The findings on RGP were hydroureteronephrosis with a stricture at pelvi-ureteric junction.The bladder capacity was small around 90 ml.She was subjected to bladder biopsy.

She underwent silicon stenting(Cook) and on follow up she had improvement of renal function to 1.7 mg%.
She underwent AKT for a period of 18 months.In the interim period she had undergone stent exchanges twice; finally she opted for reconstructive option.
This time her cystoscopic capacity was surprisingly good (around 200 ml; we suppose the initial low capacity must be because of concomitant cystitis.)But her RGP revealed additional lower ureteric stricture.

LONG SEGMENT PUJ STRICTURE

She was taken up for exploration; the pelvis was intrarenal. We didnot wish to dissect inside the renal hilum for fear of injury to the hilar vessels as this was the only kidney.
We found a thinned out parenchyma at the anterior calyceal region of the inferior pole.We made a nephrotomy there and the renal capsule and the inner mucosa were tagged in view of preventing future ureterocalycostomy closure/stenosis.

INFERIOR CALYCEAL NEPHROTOMY 

 We dissected the ureter and widely spatulated it and anastomosed to the calycotomy with 3-0 vicryl sticthes with intervening stent.

The bladder was then distended and a Boari flap was raised and tubularised over a 10 Number infant Feeding tube.This was then suture to the healthy ureter excising the lower segment of the ureter (stricture part) 

BOARI FLAP FROM THE BLADDER

GUTB: A REVIEW


Genitourinary tuberculosis is hematogeneous infection of the kidneys. The kidney being a primary organ the rest of the organs are affected by direct extension. The disease progression depends upon the host immune response.
The urologist many a times consider the GUTB as the diagnosis of exclusion. Any longstanding lower urinary tract symptoms with obvious cause detected makes the urologist suspicious about the disease.
Recurrent UTIs, frequency, dysuria, painless hematuria, painful ejaculation, anejaculation etc are the predominant symptoms.
Pathology: Tuberculosis results in development of Caseating granulomas - Langhans giant cells surrounded by lymphocytes and fibroblasts. The course of the infection depends on the virulence of the organism and the resistance of the host.
The healing process results in fibrous tissue and calcium salts being deposited, producing the classic calcified lesion. The disease because of fibrotic/calcific nature results in development of strictures,deformed calyces,small capacity bladder(so called thimble bladder).The irony of the treatment is that the starting of the antiKochs medications results in further fibrosis.This can lead to further narrowing of the strictures and / or further decrease in bladder capacity.
Small capacity bladder, nonfunctioning kidney, multiple ureteric strictures etc are the manifestations of the GUTB as in our case.