Skip to content Skip to sidebar Skip to footer

Polycystic Disease

Clinical
Autosomal-dominant polycystic disease, also called adult polycystic kidney disease and Potter type III, is the most common hereditary renal disorder. It is not a simple entity; no family history of renal disease is obtained in about half the patients, and variable penetrance and expressivity are apparent. Many of these patients develop hypertension. Renal failure is a late finding. These patients are also prone to developing aortic and cerebral aneurysms with their related complications.
A rare disorder called oral-facial-digital syndrome type 1 includes polycystic kidney disease as one component. The syndrome is X-linked, with affected males dying before birth. Although renal findings resemble autosomal polycystic disease and a dominant inheritance pattern is evident, histopathology reveals mostly glomerular cysts. There are two subtypes of autosomaldominant polycystic disease. Polycystic kidney disease type 2 (PKD2) has a milder clinical phenotype than PKD1; the median age at death or onset of end-stage renal disease is 53 years for individuals with PKD1 and 69 years for those with PKD2. About half of these patients also develop liver cysts; far fewer develop pancreatic or splenic cysts or even adrenal cysts. A rare patient also has Caroli’s disease, but this association is less common than with autosomalrecessive polycystic disease. Polycystic kidney disease can develop in a horseshoe kidney. Histologically, cystic changes occur both in nephrons and collecting ducts. Eventually multiple cysts of varying size are found in both the renal cortex and the medulla. Subcapsular cysts also develop.
Abdominal pain is a common presentation. Many patients develop hypertension. An occasional cyst rupture into the collecting system leads to hematuria. Enlarged kidneys are palpable in some individuals. Renal stones with their related complications are more prevalent than in the general population. Eventually renal failure ensues. The rare emphysematous pyelonephritis is probably due to superimposed infection rather than polycystic disease. Likewise, renal cell carcinoma developing in a setting of polycystic disease is probably fortuitous.
Typically few cysts are detected early in life, but gradually multiple bilateral cysts enlarge and interstitial fibrosis develops with loss of normal renal tissue. Not uncommonly progressive worsening of renal function manifests in early adulthood. The cysts compress and distort adjacent calyces and eventually little renal
parenchyma is detected.Why the loss of normal renal tissue is part of this condition is puzzling. Still, the kidneys are larger than normal. Several criteria are used to identify patients at risk for developing adult polycystic kidney disease. One scheme is that the presence of at least two renal cysts (either unilateral or bilateral) n individuals at risk and younger than 30 years is regarded as sufficient to establish the diagnosis; in those 30 to 59 years of age at least two cysts in each kidney and in those above 60 years of age at least four cysts in each kidney are required. Ultrasonography can be used to screen for these cysts.
Imaging
Initially cysts are small, but eventually imaging identifies bilateral enlarged kidneys, at times markedly asymmetrical, and containing multiple cysts. The cysts vary in size and distort surrounding parenchyma. Computed tomography shows most cysts to resemble simple cysts, although hemorrhage into cysts is common and these are hyperdense to water. Some have a fluid–fluid level or even appear as a solid tumor. Renal calcifications are generally secondary to calculi, prior hemorrhage into a cyst, or cyst wall calcifications.
Computed tomography or MR is useful to evaluate for any associated liver disease. The sensitivity of renal US in individuals under 30 years of age at risk for autosomaldominant polycystic kidney disease was 95% for those with PKD1 but only 67% for those with PKD2, but US sensitivity for either PKD type in individuals aged 30 years or older was 100%; DNA genetic linkage analysis was the gold standard.
image
Radiology images of polycystic disease. Transverse CT image through the kidneys reveals numerous cysts varying in size. Little renal parenchyma is left.


These cysts vary from hypo- to hyperintense both with T1- and T2-weighted images, presumably due to prior hemorrhage. A fluid-fluid level is identified in some.

image
radiology images of polycystic disease. A: Cysts, hyperintense on T2–weighted magnetic resonance imaging (MRI), have replaced most of renal parenchyma. Even more hyperintense cysts are present in the liver. B: Very little renal parenchyma is left to enhance after IV contrast. (Source: Burgener FA,Meyers SP,Tan RK, Zaunbauer W. Differential Diagnosis in Magnetic Resonance Imaging. Stuttgart: Thieme, 2002, with permission.) C: T2– weighted MRI in another patient with polycystic kidneys identifies cyst intensity variations reflecting their content differences.


Therapy Follow-up of these patients is generally by US. Computed tomography is useful to evaluate for a suspected complication. Intravenous urography has no role in follow-up. Open transperitoneal bilateral renal cyst reduction surgery is an option in symptomatic patients; one advantage of this approach is that any liver cysts are also amenable to therapy. Laparoscopic cyst unroofing (decortication or marsupialization) for relief of pain is also an option; intraoperative US aids cyst detection during the laparoscopic procedure. Some of these patients require repeat cyst unroofing efore pain relief is achieved. A number of these patients eventually undergo renal transplantation. Some surgeons recommend nephrectomy prior to renal transplantation, although others disagree and reserve nephrectomy for those with intracystic hemorrhage, significant hematuria, pyonephrosis, or other complications.

Post a Comment for "Polycystic Disease"

Category

3 tesla MRI (3) 3D CT (2) 3d printing (1) 7 tesla MRI (1) abdomen (14) Abnormal Mri Brain (33) about (1) accessory bone (1) ACL reconstruction (1) ACL tear (1) ACLS (1) acute cerebillitis (1) acute pancreatitis (1) aiims (2) aiims may 2011 (1) AIIMS nov 2008 (1) aipg (1) ALPSA lesion (1) Anatomy (29) ANGIOGRAPHY (1) ankle (1) ankle fracture (1) ankle sprain (2) Apk Android (8) apple (1) Arachnoid cyst (2) articular cartilage (1) aspergilloma (1) ATFL (1) atherosclerosis (1) avulsion injury (1) awards (1) b (6) B12 deficiency (1) bankart lesion (1) basics (8) basilar artery thrombosis (1) bilateral phaeochromocytoma (1) biopsy (1) bipolar (1) blog (3) BLS (1) body imaging (8) bohler's angle (1) BOLD (1) bone tumour (3) bone tumours (1) bowel cancer (2) brachial plexus (1) BRAIN (4) brain abscess (2) Brain CT (1) brain tb (1) Brain tumour (14) brainstem stroke (1) branchial cyst (1) breast cancer screening (1) Breast imaging (1) Breast imaging Mammography (1) buford complex (1) business today (1) c (9) C T (8) calcaneal fracture (1) calcium scoring (1) cancer (6) canned reports (1) Carcinoid (1) carcinoma esophagus (1) carcinoma stomach (1) cardiac CT (3) cardiac ct/pet (1) cardiac pacemaker (1) cardiogen 82 (1) cardiothoracic imaging (1) career (3) case study (2) catrotid artery dissection (1) cavernoma (1) cerebral venous thrombosis (3) cervical lymphnode levels (1) chest (23) chest radiographic score (1) chest radiographs (1) chest radiology (6) Chest X Ray (3) Chest X Ray Interpretation (7) Chest X Ray Self Assesment (1) chest xray (3) CHF (1) choledocolithiasis (1) classification (1) Clinicoradiological series (4) cloud computing (1) cochlear implant (1) colloid cyst (1) communication in radiology (1) compare case (1) computers and radiology (2) concha bullosa (1) congenital brain anomalies (1) congenital heart disease (1) contrast (2) Contrast Agent (1) coronary artery disease (1) coronary CT (1) corpus callosum agenesis (1) correct (2) Covid19 (2) craniopharyngioma (1) Creutzfeldt-Jakob Disease (1) cryptococcal meningitis (1) CT (25) ct abdomen and pelvis (1) CT Abdomen with contrast (10) CT angiography (1) CT Cardiac (2) CT coronary angiography (1) CT Paranasal (1) CT Scanner Price (1) CT technology (1) CV junction (1) CXR (5) CXR teaching files (1) cystic renal disease (1) d (3) DAMS (11) Dams clinicoradiological series (3) Dams grand rounds (1) Dams MD/ms (1) dams md/ms coaching (5) dams usmle (1) dams visual treat (1) deep brain stimulator (1) dengue (1) dengue hemorrhagic fever (1) developmental dysplasia (1) devices (1) diagnostic imaging (1) dialysis (1) differential diagnosis (31) diffusion MR imaging (1) diffusion tensor imaging (1) diffusion tractography (1) Diffusion weighted imaging (1) digital radiography (1) Diploma Guid (2) distal facial neuroma (1) dsa (1) duplication gall bladder (1) dynamic MRI (4) Dysmenorrhea (1) E (1) E World Award 2011 (1) echinococcus (1) echocardiography (1) ectopic kidney (1) elearning (1) elevated diaphragm (1) Embolization Imaging (1) Entrepreneur (3) Entrepreneurship (1) ependymoma (2) epidermoid (2) epilepsy (1) Equipment Cost (2) esop (1) EYE (1) facebook (2) facial neuroma (1) FDA (2) FETUS (4) Fibrous dysplasia (2) fibular osteosarcoma (1) FMG India (1) FMGE (1) FNH (1) Focal liver lesions ultrasound (1) Focal nodular hyperplasia (1) fracture (3) Fun (6) functional mri (1) gadolinium (3) Gall bladder (1) gastrointenstinal tuberculosis (1) gastrointestinal (3) Gastrointestinal Tract Images (3) Gastrointestinal x ray (4) gb 3d (1) GBM (1) GenitoUrinary (5) genitourinary radiology (3) germinoma (1) GI (1) GI radiology (4) gifts (1) GIT (1) Glioblastoma multiforme (1) glioma (1) Glomus jugulare (1) google (1) google plus (1) Group Case (1) guest post (2) guide (8) gun shot injury (1) haemangioblastoma (1) HEAD (1) head and neck radiology (7) Head injury (1) Head Neck (30) health 2.0 (1) healthcare consultancy (1) HEART (1) hemangiblastoma (1) hemophilia (1) hepatobiliary imaging (3) hippocampus (1) hirayama disease (1) HRCT (1) HSG (1) hydatid cyst (1) hypercoagulable (1) hypoxic ischemic encephalopathy (1) ICA dissection (1) Iliotibial Band Friction Syndrome (1) Imaging (11) incorrect (1) indian conferences (1) inferior rectus anomaly (1) infrared (1) interior tomography (1) internet journal of radiology (2) interstitial lung disease (1) interventional radiology (14) Interventional Ultrasound Guiding Biopsi (2) intramedullary metastates (1) intramedullary tuberculomas (1) ipad (1) ipad 2 (1) iphone (2) IRIA 2011 (1) IUGR (1) IVC filter (1) jacoud's arthropathy (1) job (15) Job Info (1) kernohan's notch (1) Kidney Cancer (1) kienbock disease (2) knee (2) kohler's disease (1) krukenberg tumour (1) laceration (1) leber's optic atrophy (1) Leptomeningeal cyst (1) lifestyle (4) lipoma (3) Literature (3) liver (2) Liver abscess ultrasound (3) liver segments ct (2) liver transplant (1) locum tenens (2) Lower Extremity (3) lung contusion (1) LV thrombus (1) lymphatics (1) macros (1) mammography (5) MAMOGRAPHY (1) marchifava bingami disease (1) mastoiditis (1) maxillary sinus mucocele (1) MCI Screening (1) MD (1) MD/MS coaching (3) MDCT (2) medial malleolus ossicle (1) medial tibial stress syndrome (1) medical blogs (3) medical writting (1) medicare (1) medicolegal aspects of teleradiology (1) Mesenteric ischemia (1) Middle East respiratory syndrome coronavirus (MERS-CoV) (1) migraine (1) mnemonic (26) mortality (1) motor area (1) Motor neuron disease (1) MPPG (1) MR mammography (1) MR Pancreas (2) MR urography (1) MRA (1) MRCP (1) MRI (45) mri abdomen (2) MRI Cervical (1) MRI contrast (2) mri knee (1) mri lower abdomen (3) mri lumbal (1) MRI update (1) MRI Vertebrae (2) MRI Vertebrae lumbal (2) MRI/PET (1) MRS (2) MRV (1) msk penang course (1) mucinous cystadenoma (1) multicentric Glioblastoma multiforme (1) multislice CT (2) musculoskeletal (21) Musculoskeletal MRI (37) Musculoskeletal radiology (72) mycotic pseudoaneurysm of superficial temporal artery (1) nature (9) NECK (1) nephrogenic systemic fibrosis (1) nephrology (1) Neuroradiology (144) neurosurgery meets (1) nonradiologist (1) Normal Liver US (1) oesophagus (1) olecranon spur (1) On Call Radiology (1) Oncology trial (1) online medical games (1) open access (1) opercular syndrome (1) opinion (1) orbit (1) ORTHOPEDICS (5) os odontoideum (1) os radiale externum (1) osirix (1) osteochondritis (1) osteochondroma (1) osteoid osteoma (1) outsourcing radiology (1) ovarian tumour (1) PAEDIATRICS (2) parameniscal cyst (1) paranasal sinuses (1) parotid vascular mass (1) Partial anomalous pulmonary venous drainage (1) patellar sleeve avulsion fracture (1) patent ductus arteriosus (1) patent foramen ovale (1) patient information (1) PDF Radiology (1) pediatric radiology (9) pediatrics (18) pelvic MRI (1) pelvis (3) pericallosal lipoma (2) pericardial fat (1) Persistent trigeminal artery (1) Pg entrance (1) phaeochromocytoma (1) pharmaceutical writting (1) Physiatrist (1) Physiatry (1) Physician Assistant (1) pineal tumour (1) placenta accreta (1) placenta increta (1) placental insufficiency (1) plain film teaching files (2) plain x ray (1) Plastic Surgery (1) PM and R Physician (1) pneumococcal pneumonia (1) PNS (1) polycystic kidney disease (1) portal Hypertension (1) posterior circulation stroke (1) power point presentation (1) Price MRI Machines 2017 (1) primary complex (1) privacy policy apk (2) Privacy Policy mri (1) Privacy Policy MSCT Cross Sectional Anatomy (1) Privacy policy physio (1) private practice (2) procedure (8) protected health information (1) pseudobulbar palsy (1) pseudomyxoma peritonei (1) psychiatry (3) publications (1) pulmonary edema (1) pulmonary embolism (1) pulmonary tuberculosis (1) Pulmonology (1) Pulvinar sign (1) Quiz (5) rabbit ear sign (1) rad (1) radiation concern (3) radiographic deterioration pattern (1) radiography (1) radiolo (1) radiologist blogs (1) Radiology (22) Radiology 1 (5) Radiology 11 (2) Radiology 14 (8) Radiology 15 (2) Radiology 17 (6) Radiology 18 (5) Radiology 19 (5) Radiology 2 (4) radiology 2.0 (4) Radiology 20 (5) Radiology 21 (5) Radiology 22 (5) Radiology 23 (5) Radiology 24 (10) Radiology 25 (5) Radiology 26 (6) Radiology 27 (5) Radiology 28 (5) Radiology 29 (6) Radiology 3 (4) Radiology 30 (5) Radiology 31 (6) Radiology 32 (5) Radiology 33 (6) Radiology 34 (4) Radiology 35 (5) Radiology 36 (5) Radiology 37 (5) Radiology 38 (5) Radiology 39 (5) Radiology 4 (4) Radiology 40 (3) Radiology 41 (4) Radiology 42 (4) Radiology 43 (3) Radiology 44 (5) Radiology 45 (5) Radiology 46 (5) Radiology 47 (6) Radiology 48 (5) Radiology 49 (6) Radiology 5 (5) Radiology 50 (6) Radiology 51 (6) Radiology 52 (5) Radiology 53 (5) Radiology 54 (8) Radiology 55 (4) Radiology 56 (4) Radiology 57 (4) Radiology 58 (5) Radiology 59 (4) Radiology 6 (5) Radiology 7 (5) Radiology 8 (4) radiology a (4) radiology business (6) radiology city (1) Radiology Conferences (4) radiology debate (1) Radiology grandrounds (1) radiology humour (2) Radiology Images (4) Radiology images of GI Tract (1) radiology images of spleen (1) Radiology Imaging (3) radiology intervensional (1) Radiology Journal (3) Radiology Journal PDF (2) radiology learning (1) radiology links (4) Radiology mcqs (3) Radiology News (23) Radiology News 2021 (1) Radiology PDF (3) Radiology Price List (1) radiology ramblings (10) radiology search engine (1) radiology spotter (1) radiology spotters (1) Radiology Teaching resource (2) Radiology Today (1) Radiology Top (8) Radiology Updates (1) radiolopolis (1) raditudes (1) random ramblings (9) rathke cleft cyst (1) renal case (4) renal imaging (1) renal sinus tumour (1) Renal Ultrasound (1) residency (10) Retinal Surgery (1) Rheumatology (1) right sided aortic arch (1) robot (1) round cell tumour (1) RTA (2) Ryles tube (1) sacral agenesis (1) salary (20) salary 1 (6) scaphoid fracture (1) schatzki ring (1) scientific content (1) screening (1) search engine (1) secondaries (1) secondary ossification centres (1) Seizure (1) Sellar Masses (1) shoulder arthrogram (2) shoulder dislocation (2) silicosis (1) SKULL (1) Sleep Medicine (1) small bowel (2) social media (2) social networking (3) social radiology (1) soft tissue chondroma (1) solitary pulmonary nodule (1) Sonographic Measurements (5) spectroscopy (1) spinal arachnoid cyst (1) spinal cord tumours (1) spinal osteochondroma (1) spine (4) spine tumour (1) spinoglenoid cyst (1) Sports Medicine (1) sternal tuberculosis (1) stress fracture sacrum (1) Stroke (5) stroke mri (1) sub-acute combined degeneration (1) subclavian pseudoaneurysm (1) Subdural hematoma (1) subependymal giant cell astrocytoma (1) sumer sethi (1) Surgery (1) swyer-james syndrome (1) syringomyelia (1) tb (1) TB Hip (2) TBM (1) teaching video (1) Tech (2) TECHNIQUE (2) technology (3) Telemedicine (4) teleradiolo (1) Teleradiology (25) teleradiology business models (1) Teleradiology Providers (7) teleradproviders (1) teleultrasound (1) temporal bone fracture (1) tendoachilles (1) term (4) testicular tumour (1) tethered cord. MRI (1) Thorax (2) tibial stress fracture (1) total knee arthroplasty (1) total shoulder replacment (1) trachea. (1) tracheal diverticulum (1) tracheal stenosis (1) tracheomalacia (1) training (3) Trauma Surgery (1) traumatic lung cyst (1) TRIANGULAR FIBROCARTILAGE COMPLEX (1) triceps tear (1) trigeminal neuralgia (2) triquetral fracture (1) tubercular abscess (1) tuberculoma (1) tuberculosis (5) tuberculosis elbow (1) tuberous sclerosis (2) ultrasound (18) Ultrasound Hepatitis (5) Ultrasound HIV (1) ultrasound image of The segments of the liver (1) ultrasound images (18) Ultrasound in Emergencies (4) Ultrasound Technique (3) Umbilical vein (1) umblical artery doppler (1) Upper Extremity X Ray (1) UPPER LIMPS (4) URI (1) urogenital imaging (3) Urology (1) uroradiology (1) Usmle (1) vascular malformation (1) Vascular Surgery (1) vasovist (1) venous (2) venous angioma (1) ventriculitis (1) Veteran Affairs (1) VHL (1) video (7) Video Anatomy (1) video ct scan (1) video mri (1) viral encephalitis (1) vitamin deficiency (1) Watching the Detectors (1) web 2.0 (8) weblog (3) X Ray Head (1) X Ray Upper Ext (2) x ray Vertebrae (2) X-RAY (34) xray (9)