ABC IN UROLOGY CATALIN PRICOP DAN MISCHIANU MARTHA ORSOLYA VIOREL BUCURAS EDITURA PIM Tagi, 2012Autors : Catalin Pricop — MD, PhD, Assoc. Prof,, Department of Urology, University - of Medicine ans Pharmacy ,,Gr.T, Popa”, asi Dan Mischianu — MD, PhD, Professor of Urology, Prorector of University of Medicine and Pharmacy "Carol Davila", Bucharest Martha Orsolya - MD, Ph.D, Assoc. Prof,, Department of Urology, University of Medicine and Pharmacy Targu Mures, Viorel Bucuras - MD, PhD - Prof of Urology, Department of Urology, "Victor Babes" University of Medicine and Pharmacy, Timisoara Coautors: Alia Adrian Cumpanas - MD, PhD, FEBU, Assist. Prof of Urology, Department of Urology, "Victor Babes" University of Medicine and Pharmacy, Timisoara Catalin Marian Ciuta, MD, PhD Candidate, Department of Urology, Clinical Hospital Cl Parhon, Iasi, Petre-Cristian Hie, MD, PhD, Assistant Professor of Urology, University of Medicine and Pharmacy"Caro! Davila", Bucharest Adriana Pricop ~ MD, PhD Candidate, Consultant radioiogist, Department of Radiology, St Spitidon Hospital, lasi, Emilia Patraseanu - MD, PhD Candidate, Anesthesia and Intensive Care Unit, St Spiridon Hospital, Iasi, Christopher Luscombe - MB ChB, FRCS (Urol), Department of Urology, University Hospital of North Staffordshire, Stoke on Trent, UK. Anurag Golash - FRCS (Urol), Department of Urology, University Hospital of North Staffordshire, Stoke on Trent, UK. Lyndon Gommersall - FRCS (Urol) Department of Urology, University Hospital of North Staffordshire, Stoke on Trent, UK. Witold Lukianski - FEBU PhD Department of Urology, Queen Elizabeth Hospital King's Lyan NHS Foundation Trust, Kings Lynn, UK Rupert Calleja - FRCS (Urol) Department of Urology, Queen Elizabeth Hospital King's Lynn NHS Foundation Trust, Kings Lynn, UKA note on the text y f For many years now, more and more young people from all the comers , of the world and even Europe choose to pursue medical studies in Romania, in ° English, which has become the language of the medical universe... r It is therefore an opportunity for the established medical universities in our country to, on the one hand, test the European standards in medical education that they have achieved and, on the other side, it is a challenge to adapt their curricula to future doctors who will not be practitioners in Romania... Mark Twain once said: “my books are like water; those of the great : geniuses are wine. Fortunately everybody drinks water”, Colleagues and friends of the urological clinics of the Medical University of Iasi, Bucuresti, \ Tirgu Mures and Timisoara, together with urologists from the United Kingdom, we have all gathered around the spring of moder urology in order to bottle a “water” that is as easy to drink and as clear as possible for our students. Our main purpose was that of offering students a textbook comparable to the one they would have had in the UK, had they followed urology course there ... ‘This task was not an easy one, but, placing at the forefront the idea that a student should be able to find in this book a “guide wire” to help him in medical practice, we have managed to overcome all obstacles... we are proud to be the first to annex, to a urology book published in Romania, a DVD containing images of the machines and instruments used in urological practice, urographical and echographical aspects, etc., images that we hope will make reading even more exciting. Our concer for a book that is as good as it can possibly be is also proved by the included feedback form... Everyone who reads the book and sends us their comments will be of great help in preparing a next edition, so that we may perhaps make the water “taste” even better... Céitdlin Pricop Dan Mischianu Orsolya Martha Viorel BucurasA note on the text “With great expectations I looked forward to reading this book and I must truly say I enjoyed it, From the beginning till the end it leads the reader through almost all urological problems and this in a clear, understandable and brief manner, Very interesting to read, detailed, yet not too simple. The reader's interest is even more raised by several questions and the clinical cases at the end of each chapter, this really made me want to read more. I must congratulate the authors with this excellent book, This book offers such a complete and solid basis of general urology that it could (and should) well be a urological manual for students in medical school and even young residents in urology. Prof. Dr. H. Van Poppel Director of the European School of Urology EAU Board Member ICUD Treasurer Past President B.V.U. Chairman Dept. of Urology University Hospitals LeuvenA note on the text I warmly welcome the publication of “ABC in Urology" book, which I consider exemplary in several ways. First, the book is remarkable because, by content, form and style is adapted to the real needs of the student's knowledge, providing the essential package of knowledge necessary for the future young doctor starting out. The book aims to induce to the student the style of "learning to know” an approach which unfortunately became increasingly rare in an university environment dominated by the learning style of teaching performance itself. In colleges, as well as throughout life, from childhood to his professional career, operates almost entirely the outside system of motivation, the rewards and / or punishments, "Do this and you will get that" sums up this doctrine that directs our actions based on purely external motivation, inhibiting/ cancelling the profound inner motivations. So, it is obvious that students lear in college to get a good grade and a deserving classification / prizes (reward) or to avoid a declassification (punishment), ignoring almost entirely the intrinsic and profound motivation, the real reason why they should learn, for which they came to college: the one of knowing what to do when they will graduate, in front of clinical cases that they will face. The student leams to be able to check the correct answers and not to become a good doctor and that is also because the value of the inner I is confused with a grade, with a figure, with the position where is classified in the teaching competition. Serious psychological studies proved that in the colleges where the excessive emphasis is on classification charts, students copy/cheat more. It also induced the idea that only some, those few "rewarded" are the winners and the majority are rather "losers", a false and dangerous idea, because the meaning of a school, especially the medical school is to develop programs through which the vast majority of students to become doctors capable of fulfilling their mission. Overall, we can say that this system creates a risk that the students and us, the teachers to become "punished by rewards"- like the inspired title of a very famous book- and, realizing this risk, we need a new approach for the teaching process. This book is an example for this. 7The modernism is the style in which this book was conceived. The information is presented in an alert lariguage, sitaply and directly, fresh as T would say, so it can be easily understood and retained. At the end ‘of cach chapter there are inserted three headings- key points, MSQ and Case Presentation-extremely useful in the learning process and which highlights the practicality of the book. Providing for the first time @ DVD and the possibility of expressing a feedback by readers, highlights more the modem style of the book. { also noticed that the volume is reduced in size, concise, synthetic, a quality which is increasingly rare in the academic publishing environment, Although the book is published by a large intemational team of valuable authors, they have resisted the temptation of giving an excessive volume of information interesting for the urology specialist, but not for the young graduate, Another fact that | want to note is the inclusion of a chapter dedicated on the anaesthesia in urologic interventions. I find very useful for the student to be familiar not only with the pre-anesthetic consultation, a defining stage for any successful intervention but also with the postoperative care from the intensive care unit, specific to each urologic intervention. The interested student will find in the same place the information about the entire process of treating an urologic patient from the diagnosis, pre-anesthesic consultation, surgery, postoperative care and will understand the usefulness of the collaboration between surgeon - anzesthesiologist as the premise of the therapeutic success. I am convinced that the English medical students will appreciate this book, 1 also think the Romanian students will be equaily interested, so the authors should think of the Romanian edition now! Browsing through this book a thought with which I want to end crossed my mind: when proceeding to write a book, we should remember that we were once students and we should design the book with the thought to the expectations and the real needs of students; the university system should be less the teachers arena in which we express our own way and rather a workshop in which the skills, the experience and our knowledge to be the tools with which we forge, through the superb material, the first represented by the young mind and soul , careers and characters. Prof Dr Dorel Sandese President of the Romanian Society of Anaesthesia, Intensive Care Director of X Department, Surgery II Pharmacy “V. Babes "TimisoaraUrological History and Examination In urology, functional signs can be grouped into three main categories : pains, urine aspect, and micturition disorders. In fact, these are precisely the reasons for patients coming in to have the urologist examine them. : The survey and the quaiity of the clinical examination are : fundamental stages of diagnosing that ‘decide’ the medical investigation plan. PAINS = Lumbar pains are the motivation of the medical consult in most patients with renal/ uretheral lithiasis, Typical renal colics manifest through relatively suddenly occurring lumbar pains that are very intense, typically radiating into the hypogastric/ testicular area, pains that the patient cannot alleviate by changing his stance. Most often, these pains are accompanied by macro-/ microscopic hematuria, The pains can also be associated with more serious signs: fever or anuria, which impose emergency hospitalization in a specialized urology ward. Associated pollakduria can signal the migration of the calculus onto the uretero-pelvic junction (sce the chapter on lithiasis). Any known or suspected lithiasis patient should be asked whether he has eliminated calculi, what their chemical composition/ aspect was, whether he has known endocrine/ metabolic problems. Furthermore, whether he has been diagnosed with multiple myeloma/ leukemia’ lymphoma, or he bas had chronic steroid, D-vitamin, or Calcium-based treatment. Whether he has had any urine infections, and if so with what bacteria. Isolated lumbar pains, without the characteristic radiation described above, can be found in patients with coraliform lithiasis, but they usually signal strain on the spine level. swe= Testicular pains can be secondary to a testicular torsion, or can signal the apparition of an acute orchiepididymitis. The pain radiates into the testicle during violent renal colies. : . * Pelvic pains, located in the hypogastric area, sometimes radiating into the genital organs, can be associated with vesical tenesmus and can be invoked by patients with cystitis or prostatitis, but can be mistaken for gynecological, digestive, or neurological pains. Signs that indicate it is in fact an urological problem are the radiation of the pain into the glans, the association of hematuria, burning sensations or urination stings, pollakiuria, urgency of urination. * Funiculo-scrotal pain can be explained by local traumatic, tumoral, or inflammatory pathology of the testicle, of the epididymis, or of the spermatic cord, URINE ASPECT. Normally, urine appears clear and of a light yellow colour (granted by the biliary hydrosoluble pigments, eliminated through the kidneys). Concentrated urine, during a low diuresis, will have dark yellow colouration, while in patients with renal insufficiency, urine has very little colour (like water), even in the case of low diuresis. = Hematuria is defined as the presence of red blood cells during urination. One must make a distinction between hematuria and urethrorrhagia, which is the presence of urethral bleeding between urination. Hematuria is a highly valuable semeiotic sign in urology, for various urological and nephrological conditions. ‘When dealing with a patient whose urine is coloured red, one must assess the following: a. Is this a case of hematuria? For guidance, the bandelette test can be used, and the urine test confirms it. b. Is this a case of urological hematuria? We must look for/ signal the post presence of clogs, of urinary symptoms (lumbar pains, urination disorders, etc.) or the existence of urological antecedents (lithiasis, tumours, ete.). 10tal ¢, Is this a case of nephrological hematuria? We must look for a recent te » | “infection in the ENT area, arterial hypertension, edemas, and whether the lab “test results indicate the presence of proteinuria or hematic cylinders. to @. What is the possible cause of the hematuria? ed _ al, . q ! | Urethra Location | Kidney Ureter | Bladder ineluding the al | | i |__ prostate) of Tumors | l of | Tumors Tumors Lithiasis | | | i Lithiasis Lithiasis Traumas | al Tumors Trauma =| Trauma Infections . ne Condition | Lithiasis | Infections | Infections Papillary | | . | . ; | , Trauma | Foreign bodies | Foreign bodies | necrosis | . i | Idiopathic | Idiopathic | Renal artery | | . . | Bladder neck | Prostatic xe | | aneurism : | od m The “ten commandments” of hematuria postulated by Proca : nm Hematuria is always an alarm signal that cannot be ignored. 1. Hematuria is always caused by an anatomic lesion of the urinary " sytem, 2. Hematuria is never physiological or functional, é 3. There is no hematuria without cause! The so-called essential hematuria bas an unknown cause. +s 4. Hematuria is a cardinal point in the pathology of the urinary system, but is not exclusive to it, as it can be an expression of other visceral or systemic conditions. 2 5, Whether it is visible only under the microscope or not, hematuria has the same meaning. 1e 6. Important macroscopic hematuria must be hemodynamically s assimilated with any other exteriorized internal hemorrhage (hematemesis, melena, hemoptysis, etc.) i7. Regardless of the clinical context in which it becomes manifest, hematuria is always a diagnosing emergency. . 8. Hematuria rarely becomes a treatment emergency, ‘but when it does, it” imposes treating its causes, 9, The symptomatic treatment of hematuria can become a serious treatment error, when it is limited to just that. = Pyuria can be defined as the cloudy urine during urination. Pathological leukocyturia is defined by the Addis-Hamburger test as being above 2000/ml/min. As a general rule, leukocyturia indicates an evolving urinary infection, or ‘one that is being treated. In the absence of an infection with banal bacteria or MIB, we must also consider nephrological causes of leukocyturia: interstitial glomerulopathy, severe diabetes, systemic lupus erythematosus. The bandelette test in current medical practice allows diagnosing pathological leukocyturia (>10,000 GA/ml). It is based on the esterasic activity of the multinucleate cells. In this situation, it is important to monitor the colouring agents' effect on nitrites. Most urinary pathological bacteria reduce nitrates to nitrites (absent in normal urine!), False negative results are given by antibiotics therapy or by ascorbic acid. Warning! Streptococei and MTB do not haye the ability to reduce nitrates to nitrites! © Notany turbid urine signals a urinary infection: a salt-rich urine can have a similar aspect. It must be determined whether the urine becomes limpid through heating, whether the ‘turbidness' is owed to urate salts; if the urine becomes limpid after acidification, this means that the turbid aspect was caused by an outpour of phosphates of carbonates. © Women suffering from vulvovaginitis can display turbid urine in the context of the gynecological condition. Obtaining a urine sample through vesical sampling can clarify the situation. 12st, it us og or or ial ng ity he ice rot ne aes the vas in © The urine that becomes turbid after a certain time from its emission is not considered pyuria. Urine samples brought by patients in @ container that they filled before coming to the doctor are of no diagnosing value. As in the case of hematuria, it is important to analyze the urine when it is emitted! Most of the time, urine is turbid due to pus, which is produced by the urinary infection. The mandatory examination for bacterial pyuria is quantitative uroculture, Amicrobial pyuria (abundant leukocyturia, turbid urine with negative uroculture) and acid urine pH must direct our examinations towards urogenital tuberculosis or towards parasitoses. = Pneumaturia represents the passage of gas in urine. This release occurs at the end of the urination, and is accompanied by a noise that is unmistakable. Pheumaturia can have external cause (most often, after vesical sampling), internal (in cases of uro-digestive fistulae), or endogenous (CO2 produced by gram-negative bacteria in the context of toxic shock syndrome in a diabetes patient), = Fecaluria is the presence of feces in urine at the time of urination. ‘The urine is turbid, gray-brown, foul-smelling, and the clinical examination confirms the diagnosis. The location of the fistula must be established through radiological imaging. * Chyluria is the presence of eliminated lymph in the urine at the time of urination. It indicates an abnormal communication between the urinary system and the lymphatic system. The aspect of the urine is characteristically milky. This sign is rare, but its diagnostic value is enormous. The origin of chyluria can be parasitic, in the case of parasitosis (filaria bancrofti) or unparasitic (very rarely). * Hematospermia deserves separate mentioning, even though it is more seldom in occurrence, Usually unaccompanied by pain, this manifestation 13imposes detailed questioning of the patient (blood from the partner?). If pain or stings are associated with it, we can consider a potential inflammation of the prostate or prostate lithiasis. In young patients, hematospermia must also orient- our diagnosis towards a possible urogenital tuberculosis, and in older patients towards prostatic neoplasm. URINATION DISORDERS = Normal urination is a reflex act that allows the intermittent evacuation of the urine contained in the urinary bladder. It is supposed to be a voluntary, rapid, complete, effortless, unpainful process. Normal urination requires that the bladder ‘pump! and the sphincter system to work harmoniously. = Dysuria, urination pains, pollakiuria, overactive bladder, and incontinence will be dealt with at length in the chapters dedicated to tumor and prostate pathology, and urethral strictures. EXAMINING THE UROLOGY PATIENT This is an important stage of diagnosing and, warning! It cannot be YY ¥ Initial hematuria (Urethro-prostatic) YY Terminal hematuria (Bladder) replaced by any technology, no matter how sophisticated. The clinical examination of the micturition must provide us with answers to the following important questions: is the patient straining himself to urinate? Is the jet of urine insufficiently projected? Is he only urinating drop by drop (through overflow)? Is the urine jet interrupted? Is the patient unable tolor ent onts: ion wy, ‘hat and and control passing urine? Is the urine jet normal/ filamentary/ scattered? What is the macroscopic aspect of the urine? In case of hematuria, is it initial (the first container), terminal (occurs only at the end of the urination), ie. the third container, or total (in all three containers). “ The clinical examination of the kidneys An examination of the lumbar area can outline: = lumbar scars after previous interventions «the patient's alleviating body stance (this is possible in the context of a perinephric suppuration) = the unilateral enlargement or deformation of the Jumbar area, in thin patients, in the context of renal tumor pathology or voluminous cyst). Palpation = All four anterior quadranis of the abdomen must be palpated, as weil as the two costovertebral angles. During this procedure, the doctor must closely observe the patient's facial expression to detect tenderness, while causing minimum pain. Any mass must be detected and recorded: location, size, consistency, and mobility. Warning! Do not palpate too energetically the lumbar area of a patient with renal trauma, in order to prevent aggravating potential lesions! = Percussion and auscultation of the lumbar area do not provide significant information. ‘THE CLINICAL EXAMINATION OF THE HYPOGASTRIC AREA « The urinary bladder is reflected onto the hypogastric area. When the bladder is empty, it cannot be palpated or percussioned, which is due to its retrosymphysial location. In thin patients with chronic retention with distension, inspecting the hypogastric area outlines a round, ovoid deformation, located medially, that cannot be moved in lateral decubitus, sometimes painful, 15most of the times well-tolerated. The palpation and percussion contribute, in this case, to diagnosing the ‘globular vesica’. ‘THE CLINICAL EXAMINATION OF THE GENITALIA ‘The penis is examined in order to potentially outline: * Urethrorrhagia (blood leaks at the level of the urinary meatus); = Urethrorthea (the presence of purulent urethral secretion. in urethrites, prostatites); = The opening of the urinary meatus on the dorsal side of the penis (cpispadias); * The decrease in calibre of the urinary meatus (meatal stenosis of inflammatory or post-traumatic cause); = The presence of fistulous penile or pseudo-scrotal tract, starting in the urethra, an indication of fistulized periurethral abscesses; * Phimosis (the inability to retract the foreskin over the glans); = Pataphimosis (the situation when a forced retraction of the foreskin occurs in a phimosis patient, which cannot be undone due to edema). = Balanopreputial suppurations; * Palpating the penis can indicate the presence of plastic induration of the corpora cavernosa (Peyronie's Disease); ™ Female urethral pathology can consist, in rare cases, of urethral meatal stenosis, and of the presence of inflammatory lesions at this level. * The urology specialist often encounters cervical ectropion cases, visible as red-violet coloured tumorette which is prolabial, irreducible, on the anterior segment of the urethra, labeled as ‘urethral polyp’. The valve vaginal examination, completed in the vaginal tract, can help detect urethral fistulas. The examination and palpation of the scrotum can determine: = A potential volume increase of the hemiscrotum: ° Painfiel - in the inflammations of the testicular epididymis, and in orchiepididymal traumas; 16‘ites, denis skin nof zatal ses, sinal md © Unpainfirl ~ in testicular and epididymal tumors, in idiopathic hydrocele, hematocele or epididymal cysts, peritoneo-vaginal channel persistence (communicating hydrocele). = The presence of a fistulous orifice through which pus is leaking out ~ in the case of an orchiepididymal abscess that fistulized at the scrotum: = The presence of celsian signs (rubor, tumor, calor, dolor, finctio Jaesa) in epididymites and orchiepididymites; * The irregularity of the two scrotum chambers, with, potentially, the absence of one testicle from its chamber. This is called cryptorchidlism (bilateral in up to 25% of cases), when the testis is stopped in its normal trajectory (descensus testis) or ectopic testis — if the testis is located on an abnormal trajectory; * yascular diseases ~ idiopathic varicocele, testicular torsion, manifested as scrotal edema; * solid (testicular, epididymal) or figuid (epididymal or cord cysts) tumors; = inguinoscrotal hernia vera or hernia through persistence of the peritoneo-vaginal channel. Palpating the scrotal chambers, in both ortostathism and clinostatism, reinforces the same semeiotic signs mentioned above: * uni- or bilateral anorchia, the absence of the epididymis, the presence of Morgagni's hernia; * diagnosing elements of various specific (tuberculosis, syphilis, gonorrhea) or non-specific inflammatory diseases; = vascular diseases — idiopathic varicocele, testicular torsion manifested as scrotal edema; = solid (testicular, epididymal) or liquid (cpididymal or cord cysts) tumors; 17™ inguinoscrotal hernia vera or hernia through persistence of the peritoneo-vaginal channel. . : Testicular palpation will indicate its consistency (homogenous, firmly- retinent), its volume, surface, sensitivity, atrophy, or even absence (congenital or post-surgical). The dilated vascular tissues in cases of varicocele have been compared to ‘bird intestines’. One must not omit palpating the inguinal superficial orifice, precisely in order to identify potential types of inguinal hemia. RECTAL EXAMINATION ‘This is an important diagnosing maneuver, not only for the urologist, but also for the surgeon or the gynecologist. Elements of this examination that are of interest include: * Exploring the prostate (size, consistency, median duct, the presence of nodules). It is best performed under rachianesthesia. * Exploring the urine bladder — palpation under rachianesthesia being essential to an evaluation of a tumor infiltration to the bladder, = Exploring the sphincter and the anal channel (fistulas, anorectal suppurations, hemorrhoids, low rectal tumors). Examining fecal debris is mandatory ~ melena, rectorthagia? * The perianal tegument and the anal sphincter are innervated starting with S2, $3, and S4. If there is a suspicion of a neurological condition, it will be helpfull to examine the state of the sphincter. * Exploring the Douglas cul-de-sac ~ ‘Douglas’ scream! in peritonites and pelvic peritonites. * Occasionally, patients will refuse digital rectal examination: in such a case, they must be informed that we cannot form an opinion about their prostate pathology and we cannot determine a treatment path. 18the amly- nital veen inal inal but ve of eing ectal is is sting will aites cha their i Key points: » Lumbar pains ate the motivation of the medical consult in most patients with renal/ uretheral lithiasis. » One must make a distinction between hematuria and urethrorrhagia, which is the presence of urethral bleeding between urinations. Hematuria is a highly valuable semeiotic sign in urology, for various urological and nephrological conditions, » Pyuria can be defined as the cloudy urine during urination. Pathological leukocyturia is defined by the Addis-Hamburger test as being above 2000/mi/min. Not any turbid urine signals a urinary infection: a salt-rich urine oan have a similar aspect. = The clinical examination of the micturition must provide us with answers to the following important questions: is the patient straining himself to. urinate? Is the jet of urine insufficiently projected? Is be only urinating drop by drop (through overflow)? Is the urine jet interrupted? Is the patient unable to control passing urine? Is the urine jet normal/ filamentary/ scattered? What is the macroscopic aspect of the urine? * RECTAL EXAMINATION is an important diagnosing maneuver, not only for the urologist, but also for the surgeon or the gynecologist: exploring the prostate (size, consistency, median duct, the presence of nodules); exploring the urine bladder — palpation under rachianesthesia being essential to an evaluation ofa tumor infiltration to the bladder; exploring the sphincter and the anal channel (fistulas, anorectal suppurations, hemorrhoids, low rectal tumors). Examining fecal debris is mandatory ~ melena, rectorrhagia?; exploring the Douglas cul-de-sac ~'Douglas' scream’ in peritonites and pelvic peritonites, MCQ 1, All the afirmations bellow are correct except one : a. a.Testicular pains can be secondary to a testicular torsion, 19b. The bandelette test in current medical practice allows diagnosing pathological leukocyturia (+10,000 GA/mil) c. Hematuria can be in some cases physiological or functional. d. Women suffering from vulvovaginitis can display turbid urine in the context of the gynecological condition. e. The presence of plastic induration of the corpora cavernosa is called Peyronie's Disease; (CASE PRESENTATION ‘A 52 year-old patient is callin the ambulance for acute lumbar pain after phisical effort. 1. How can the doctor differentiate a renal colic from a lumbago painonly by clinical means? 2, Is it helpful to see the urine at emission? Why? i ;sing Malformations of the urinary tract the lied Malformations of the genitourinary tract are represented by the anatomic and/or histologic anomalies which are present at the new-bom. There is a relatively rare pathology, sometimes life-threatening (e.g. bilateral renal agenesia), sometimes with potential risk for the health/life (ureteropelvic junction stenosis) and sometimes without tisks (e.g, simple ster cortical renal cyst). For this reason, it is important for the physician to diagnose ihe malformation as early as possible and to act accordingly. »aQ0 EMBRYOLOGY OF THE URINARY TRACT Following fertilization, a blastocyte results, which implants into the uterine endometrium on day 6. The early embryonic disc of tissue develops a yolk sac and an amniotic cavity, from which are derived three parts: the ectoderm, endoderm, and mesoderm. The kidneys develop from the intermediate part of the mesoderm and , in the embryonic life, there are three kidneys which appear successively: pronephros, mesonephros and metanephros (this last one is the definitive kidney). Pronephros - is the first, transitory and non-functional kidney which appears in the 3-rd week of intrauterine life and disappears in the 5-th week. Its structure is quite similar with the kidneys from the primitive fish. It appears in the future neck and thorax region. Mesonephros- is the second kidney which appears from the S-th week of intrauterine life and lasts until the 4-th months. It represents the first functional renal filtrating unit for the embryo and is associated with two duct systems — the mesonephric duct and the paramesonephric duct. 21The mesonephric (Wolffian) ducts develop laterally and advance downward to fuse with the primitive cloaca. By week 5, a ureteric bad grows from the distal part of the mesonephric ducts and induces formation of the metanephtos in the overlying mesoderm After the 4-th month of the intrauterine life mesonephros disappear but there are parts of it which persist in the mature human: ™ the paramesonephric duct essentially forms the female genital system (fallopian tubes, uterus, and upper vagina) meanwhile in males it regresses = the mesonephric duct forms the male genital duct system (epididymis, vas deferens, seminal vesicles, central zone of prostate); in the female, it regresses. Metanepbros - is the third kidney, from which the adult kidney develops. It develops from the 4-th month, in the sacral region. During its development, the ureteric bud (from the mesonephric duct) penetrates the mesenchima of the metanephros, begins to divide and forms the collecting system (collecting ducts, calyces and renal pelvis). Thus, it can be said that the mesonephric duct forms the ureters and renal collecting system. Meantime, renal glomeruli are formed from the metanephros mesenchima. ‘The urine production starts from the 10-th week of intrauterine life. During the growing process, the caudal part of the foetus rapidly grows and the kidneys are pushed upward (renal ascent process from the sacral to the lumbar region - weeks 6-9). In the ascent process, the arteries feeding the metanephros do not follow the kidney, they degenerate, being replaced by other new-ones until the kidney reaches its final position and the definitive renal artery develop. Although the renal maturation process continues after birth, the nephrogenesis process is considered completed at that time. The lower part of the mesonephric ducts (ureters) drain into the cloaca (Latin = sewer). During the weeks 4-6, cloaca divides into the urogenital sinus (anteriorly) and the anorectal canal (posteriorly). 22nital as it stem the ows s the : by tive vaca inus ‘The bladder develops from the upper part of the urogenital sinus Urethra develops from the inferior part of the urogenital sinus in females. In males, urethra has two origins: from the mesonephric ducts (the first part of the urethra-prostatic urethra) and from the closure of the urogenital groove (the anterior urethra) MALFORMATIONS OF THE KIDNEY A. Anomalies of number 1. Unilateral renal agenesia Unilateral renal agenesia (Gr.A=absence, genesia~formation) means the congenital absence of a kidney. The incidence is between 1/500-1/1000 new-borns. In the vast majority of cases there is a renoureteral agenesia (the ureter js lacking too - embryologically there was no mesonephric duct to develop the ureteric bud) although sometimes the ureter can be present, with its upper part being blind (the mesonephric ducts were present bilateral but, in one part the ureteric bud did not stimulated the metanephros mesenchima formation). Sometimes this malformation is associated with other genital malformations, The disease is asymptomatic, thus, if the new-born is not examined by ultrasound the disease can be discovered incidentally (routine ultrasound or TU). The solitary kidney is compensatory hypertrophied (more than 10% from the normal kidney size/volume). The hypertrophy is only limited to the proximal renal tubule of the nephron to compensate the increased ionic changes ai this level and this represents a very useful ultrasound sign (because the absence of the kidney from its normal position at ultrasound is not equivalent with the renal agenesia: it can be an ectopic kidney, present elsewhere in the body). Sometimes, the CT put the diagnosis. 23To confirm the diagnosis is necessary to perform ‘urethrocistoscopy which will reveal the absence of the ureteral orifice (renoureteral agenesia) of a blind ureter (by retrograde ureteropielography). The malformation is not life-threatening, does not need any further treatment but is good that the patient knows it (e.g. renal colic, renal trauma, kidney cancer surgery etc. - on solitary kidney have other prognosis on solitary vs. bilateral kidney). Sometimes renal infections and stones can occur more frequent than in normal kidneys. 2. Bilateral renal agenesia Is a rare disease (500 cases worldwide), not compatible with life, the child often presents hypoplastic tung and facial deformities. ' 3. Supranumerary kidney Js characterized by more than two kidneys and urinary tracts (is different from renal duplication where the patient has a renal unit with duplex or triplex collecting system - as a result of duplication of the ureteral bud as it joints the metanephros mesenchima). It is a rare condition which is usually diagnosed incidentally in adult life and generally has no consequences on renal function. B. Anomalies of volume and structure 1. Renal hypoplasia ‘This malformation implies the presence of a small kidney, with normal collecting system. It can involve the whole kidney or only a part of the kidney, corresponding to a certain segment of the kidney. Sometimes can be bilateral. Alcoholic foetal syndrome and in-utero cocaine exposure are considered predisposing factors. . This malformation can be responsible for secondary renal hypertension (both by vascular and parenchimatous mechanism) or for renal failure (in case of bilateral hypoplasia). On the other hand, the diagnosis should exclude the secondary renal hypoplasia by chronic pyelonephritis, vesicoureteral reflux or by renal artery stenosis with secondary renal hypoplasia. There are many cases which are asymptomatic, 24ascopy 2.. Infant polycystic kidney a)ora | + Is a very rare condition, autosomal recessive transmitted, the kidney structure being completely disorganized, with glomerular and tubular cysts and further islands of metaplasia into the kidney. The kidney appears of larger size, rauma, sometimes being palpable and putting problems of differential diagnosis with olitary abdominal or renal masses. "more The abdominal ultrasound and CT put the diagnosis, The patient presents renal failure after the birth, The treatment includes the management of the xenal failure, respiratory problems and hypertension. The prognosis is poor. fe, the 3, Adult polycystic kidney This is an autosomal dominant disease, being a different disease from the infant form and having a better prognosis. The pathogenesis consists in sts (is joining between normal glomeruli with blind nephronic tubules, The resulting lex or cysts which compress the adjacent renal parenchyma and destroy it mainly by joints ischemia. nosed It appears in the 4-th decade of life with progressive renal failure, on. hypertension (90% of cases) and palpable renal masses. Gross haematuria, infection (loin tenderness, chills, fever) and signs of renal failure can occur. Laboratory findings include: anaemia (the kidney erythropoetin secretion is severely impaired) and high serum creatinine levels. ormal Ultrasound examination/CT scan reveals bilateral enlarged kidneys, dney, with cysts of different sizes which disorganize the normal architecture. of the eral. kidney (in fact, both kidneys are transformed in huge cystic bags). Sometimes, dered in case of intracystic hemorrhage differential diagnosis with a renal tumor should be taken into consideration. Radioisotopic studies reveal cold spots in asion extremely enlarged kidney. 1 case The treatment include diet restriction (low protein intake, low salt diet), le the high fluid intake and, if necessary hemodialysis. In cases or pyonephrosis, Ux oF large cysts which compress the collecting system, severe bleeding or renal cases tumor, surgical treatment could be necessary. Renal transplantation can be ‘useful in patients with renal failure. 254, Simple renal cysts ‘This malformation is rare in children and yery frequent in adulthood. ‘The incidence of disease increased since the wider use of ultrasound in. daily practice. In many cases it does not put any problem but sometimes can be a harmful condition which if not treated properly, can -have dangerous consequences. That's why we will treat it more extensively. The cyst, delineated by a wall can be situated in the renal cortex or adjacent to the renal sinus (sometimes, if it has large size, could be obstructive for the urinary tract), In cases of multilocular cysts, the risk of being a cystic renal tumor should be taken in consideration. The Bosniak classification of the cysts (see below) defines 4 types of cysts, The higher the Bosniak type is, the higher the probability of a tumor- harboring cyst is. Type I - cysts with smooth, thin wall, with clear fluid ; Type If - cysts with minimal septations and a smail fine rim of calcification, Type IT F (mostly benign but need to be followed); Type HI - cysts with many septations, with more calcification and a thick cyst wall; Type IV - cyst with a thick, irregular wall, with calcifications or with a mass inside the cyst. The guidelines recommends ultrasonic-guided puncture with fluid aspiration and cytological examination or even cyst excision in any case of doubt (type II and IV or whether the cystic fluid obtained at punction is hemorrhagic). Some authors recommend cystography (injection of contrast medium into the cystic cavity after the puncture) and radiographic evaluation for a better visualisation. Symptoms are unremarkable, very often being asymptomatic. Sometimes can lead to fullness sensation in lumbar region, palpable mass (large cyst), lumbar pain (by obstruction of urinary tract - large cysts). Complications are very rare and include: intracystic haemorrhage (with intense pain), cyst infection (with pain and fever). 26 : : a 3ii The treatment for asymptomatic cysts with no doubts regarding its . benign character is not necessary. Cyst removal (laparoscopy) is necessary for din, _ jarger oySts, symptomatic or compressive on the urinary tract. Cystic puncture abe with aspization/drainage is necessary in infected cysts (the antibiotherapy is not ous efficient in these cases due to the low passage of the antibiotic into the cystic cor uid). Because of the high risk of malignancy surgical treatment for Bosniak tive type III and IV is recommended. ‘ste 5, Multicystic kidney This term defines a severe non-genetic dysplasia, with the kidney with *o multiple cysts, losing its reniform shape, without a calyceal system clearly defined. There are reported cases with spontaneous involution antenatally or postnatally. However, it is an evolutive disease, usually the kidney becoming smaller (aplasia), rarely increasing to huge dimensions. «of The disease affect only one kidney (vs. the polycystic kidney where both kidneys are affected) but the contralateral kidney can be affected by other da anomalies (vesicoureteral reflux, obstructive megaureter, ureteropelvic junction obstruction). The patients with multicystic kidney are predisposed to renal tumors and hypertension, requiring a careful monitoring. me 6. Medullary sponge kidney (Cacchi-Ricci syndrome) 1is In this nonheritable malformation, which rarely occurs in children, a rast dilatation and cyst formation occurs in distal collecting tubules, giving the tion urographic characteristic of a bristles on a brush sometimes filled with calcifications (if stones are present, a plain film will reveal small, round caiculi | in the pyramidal regions just beyond the calyces) or a streaky blush extending ttle. into the medulla from the involved calyces. The incidence is between 1/5000 - 1ass 1/20000 in general population. The malformation can, be limited to one kidney or can involve both kidneys. Another term for this malformation is ‘precaliceal vith canalicular ectasia’. Many patients remain asymptomatic. Symptoms include renal colic (but usually with spontaneous passage of the stone due to the small size of the 27stones), urinary tract infections, gross haematuria and hypercalciuria. Stone, formation, although in a large number rarely need surgery (only when a small area ofa kidney is destroyed by the dysplasia process), C. Anomalies of rotation and ascent LL Matlrotation The normal position of the kidney comprises the kidney located in the renal fossa, with the calyces oriented laterally and the pelvis medially. Any other situations of the kidney are considered malrotations. Its incidence is unknown since many cases with small degrees of malrotation or asymptomatic cases are not reported/ undiagnosed. The most frequent situation when this malformation appears is in Turner syndrome. The most common situations (defined by the relative position of the renal pelvis) are: ventral position (renal pelvis facing anterior and the calyces posterior, in the same antero-posterior plane), ventromedial position (with the pelvis situated ventromedially and the calyces posterolaterally), dorsal position and lateral position. Sometimes, other degrees can occur. Foetal lobulation are present and dense fibrous tissue surrounding the hilar area is commonly encountered. Symptoms are not specific, sometimes ureteropelvic junction obstruction can occur due to the excessive fibrous tissue from the hilar region which encompass the renal pelvis and ureter. In other situations, secondary renovascular hypertension by renal artery stenosis can occur. Diagnosis is put by IVU which reveal the abnormal position of the renal pelvis. The malrotation itself does not need any specific treatment. The treatment is addressed to the complications due to the fibrous tissue from the renal hilum (ureteropelvic junction obstruction, renal artery stenosis). 2, Ectopic kidney As described at the beginning of this chapter, the kidney moves upward from its initial sacral position, between the 6-th and the 10-th week of intrauterine life. Any incomplete ascension leads to an abnormal position of the kidney (Gr. ectopia means ec -out, topos-place). Sometimes, the kidney from one side can move upward on the opposite side, resulting crossed ectopia (the 28