Congenital Congenital Chorioretinitis renal abnormalities arise from embryologic/genetic defects and cause a variety of isolated or syndromic renal disorders, including renal agenesis Agenesis Teratogenic Birth Defects, dysgenesis, and ectopia. Congenital Congenital Chorioretinitis renal abnormalities are generally identified prenatally and represent approximately ⅓ of all prenatal anomalies. Because of the fetal kidney’s role in the production of amniotic fluid Amniotic fluid A clear, yellowish liquid that envelopes the fetus inside the sac of amnion. In the first trimester, it is likely a transudate of maternal or fetal plasma. In the second trimester, amniotic fluid derives primarily from fetal lung and kidney. Cells or substances in this fluid can be removed for prenatal diagnostic tests (amniocentesis). Placenta, Umbilical Cord, and Amniotic Cavity, oligohydramnios Oligohydramnios Oligohydramnios refers to amniotic fluid volume less than expected for the current gestational age. Oligohydramnios is diagnosed by ultrasound and defined as an amniotic fluid index (AFI) of ‰¤ 5 cm or a single deep pocket (SDP) of < 2 cm in the 2nd or 3rd trimester. Oligohydramnios detected on prenatal ultrasounds often prompts the workup that identifies congenital Congenital Chorioretinitis renal anomalies. Unilateral renal involvement in the presence of a functioning contralateral kidney may only be an incidental finding later in life. In many cases, treatment is supportive.
Last updated: 7 Oct, 2021
The kidney develops in the pelvis Pelvis The pelvis consists of the bony pelvic girdle, the muscular and ligamentous pelvic floor, and the pelvic cavity, which contains viscera, vessels, and multiple nerves and muscles. The pelvic girdle, composed of 2 “hip” bones and the sacrum, is a ring-like bony structure of the axial skeleton that links the vertebral column with the lower extremities. Pelvis: Anatomy and migrates cranially. Three separate renal systems form in sequence, giving rise to the kidney, in association with the urinary tract Urinary tract The urinary tract is located in the abdomen and pelvis and consists of the kidneys, ureters, urinary bladder, and urethra. The structures permit the excretion of urine from the body. Urine flows from the kidneys through the ureters to the urinary bladder and out through the urethra. Urinary Tract: Anatomy and urogenital system:
Metanephros Metanephros Development of the Urogenital System
Image by Lecturio.Renal embryologic disorders affecting the size, the shape, or the structure of kidney parenchyma (renal dysgenesis):
Incidental discovery of renal agenesis Agenesis Teratogenic Birth Defects in a 12-year-old boy: Magnetic resonance imaging (MRI) of the abdomen on the coronal Coronal Computed Tomography (CT) plane shows right renal agenesis Agenesis Teratogenic Birth Defects (red arrow) and left ectopic pelvic kidney over the urinary bladder Urinary Bladder A musculomembranous sac along the urinary tract. Urine flows from the kidneys into the bladder via the ureters (ureter), and is held there until urination. Urinary Tract: Anatomy (blue arrow).
Image: “Figure 2” by Altun et al AL Amyloidosis. License: CC BY 2.0.Sagittal Sagittal Computed Tomography (CT) (left) and coronal Coronal Computed Tomography (CT) (right) T2-weighted MRI views of the abdomen and pelvis Pelvis The pelvis consists of the bony pelvic girdle, the muscular and ligamentous pelvic floor, and the pelvic cavity, which contains viscera, vessels, and multiple nerves and muscles. The pelvic girdle, composed of 2 “hip” bones and the sacrum, is a ring-like bony structure of the axial skeleton that links the vertebral column with the lower extremities. Pelvis: Anatomy, showing right renal agenesis Agenesis Teratogenic Birth Defects and complete absence of MĂĽllerian/ paramesonephric duct Paramesonephric duct A pair of ducts near the wolffian ducts in a developing embryo. In the male embryo, they degenerate with the appearance of testicular anti-mullerian hormone. In the absence of anti-mullerian hormone, mullerian ducts give rise to the female reproductive tract, including the oviducts; uterus; cervix; and vagina. Uterus, Cervix, and Fallopian Tubes: Anatomy structures in an 18-month-old infant
Image: “Figure 2” by William Mifsud et al AL Amyloidosis. License: CC BY 3.0.Multicystic kidney: T2-weighted HASTE imaging of monochorionic, monoamniotic twins. Panel A demonstrates that the affected twin is on the right with a thickened and distended
bladder
Bladder
A musculomembranous sac along the urinary tract. Urine flows from the kidneys into the bladder via the ureters, and is held there until urination.
Pyelonephritis and Perinephric Abscess.
Panel B is an
axial
Axial
Computed Tomography (CT) slice demonstrating a multicystic kidney (arrow).
(A) Obstructive MCDK: Notice the hypoplastic
kidneys
Kidneys
The kidneys are a pair of bean-shaped organs located retroperitoneally against the posterior wall of the abdomen on either side of the spine. As part of the urinary tract, the kidneys are responsible for blood filtration and excretion of water-soluble waste in the urine.
Kidneys: Anatomy with the
adrenal glands
Adrenal Glands
The adrenal glands are a pair of retroperitoneal endocrine glands located above the kidneys. The outer parenchyma is called the adrenal cortex and has 3 distinct zones, each with its own secretory products. Beneath the cortex lies the adrenal medulla, which secretes catecholamines involved in the fight-or-flight response.
Adrenal Glands: Anatomy still at the upper poles (arrow). The
aorta
Aorta
The main trunk of the systemic arteries.
Mediastinum and Great Vessels: Anatomy is centrally located and there are bilateral hydroureters.
(B) Nonobstructive MCDK: The
ureters
Ureters
One of a pair of thick-walled tubes that transports urine from the kidney pelvis to the urinary bladder.
Urinary Tract: Anatomy are unremarkable and portions of the two umbilical
arteries
Arteries
Arteries are tubular collections of cells that transport oxygenated blood and nutrients from the heart to the tissues of the body. The blood passes through the arteries in order of decreasing luminal diameter, starting in the largest artery (the aorta) and ending in the small arterioles. Arteries are classified into 3 types: large elastic arteries, medium muscular arteries, and small arteries and arterioles.
Arteries: Histology run parallel to the
urinary bladder
Urinary Bladder
A musculomembranous sac along the urinary tract. Urine flows from the kidneys into the bladder via the ureters (ureter), and is held there until urination.
Urinary Tract: Anatomy. The
kidneys
Kidneys
The kidneys are a pair of bean-shaped organs located retroperitoneally against the posterior wall of the abdomen on either side of the spine. As part of the urinary tract, the kidneys are responsible for blood filtration and excretion of water-soluble waste in the urine.
Kidneys: Anatomy are bi-valved to demonstrate numerous small
cysts
Cysts
Any fluid-filled closed cavity or sac that is lined by an epithelium. Cysts can be of normal, abnormal, non-neoplastic, or neoplastic tissues.
Fibrocystic Change scattered through the cortex and medulla.
Autosomal recessive polycystic kidney disease
Autosomal Recessive Polycystic Kidney Disease
Polycystic kidney disease (PKD) is an inherited genetic disorder leading to the development of numerous fluid-filled cysts in the kidneys. Autosomal recessive polycystic kidney disease is primarily characterized by cystic dilatations of the renal collecting ducts and intrahepatic bile duct dilatation with hepatic fibrosis.
Autosomal Recessive Polycystic Kidney Disease (ARPKD).
A: Baby with a distended abdomen due to voluminous
kidneys
Kidneys
The kidneys are a pair of bean-shaped organs located retroperitoneally against the posterior wall of the abdomen on either side of the spine. As part of the urinary tract, the kidneys are responsible for blood filtration and excretion of water-soluble waste in the urine.
Kidneys: Anatomy that led to respiratory problems and early demise.
B: Abdominal situs of a perinatally demised
ARPKD
ARPKD
Polycystic kidney disease (PKD) is an inherited genetic disorder leading to the development of numerous fluid-filled cysts in the kidneys. Autosomal recessive polycystic kidney disease is primarily characterized by cystic dilatations of the renal collecting ducts and intrahepatic bile duct dilatation with hepatic fibrosis.
Autosomal Recessive Polycystic Kidney Disease (ARPKD) patient with symmetrically
enlarged kidneys
Enlarged kidneys
Autosomal dominant polycystic kidney disease (ADPKD) that maintained their reniform configuration.
Autosomal recessive polycystic kidney disease
Autosomal Recessive Polycystic Kidney Disease
Polycystic kidney disease (PKD) is an inherited genetic disorder leading to the development of numerous fluid-filled cysts in the kidneys. Autosomal recessive polycystic kidney disease is primarily characterized by cystic dilatations of the renal collecting ducts and intrahepatic bile duct dilatation with hepatic fibrosis.
Autosomal Recessive Polycystic Kidney Disease (ARPKD) in a fetus.
A: Gross specimen of the right kidney measuring 10 Ă— 7 Ă— 4 cm. The cut surface is spongy with poor corticomedullary differentiation. There are multiple, tiny
cysts
Cysts
Any fluid-filled closed cavity or sac that is lined by an epithelium. Cysts can be of normal, abnormal, non-neoplastic, or neoplastic tissues.
Fibrocystic Change with some at right angles to the cortical surface.
B: Photomicrograph showing numerous
cysts
Cysts
Any fluid-filled closed cavity or sac that is lined by an epithelium. Cysts can be of normal, abnormal, non-neoplastic, or neoplastic tissues.
Fibrocystic Change lined by a single layer of low cuboidal epithelial cells with thick peritubular mesenchyme. The glomeruli are normal (H and E, Ă—40).
Antenatal ultrasound at 24 weeks showing bilateral, symmetrically enlarged, echogenic kidneys Kidneys The kidneys are a pair of bean-shaped organs located retroperitoneally against the posterior wall of the abdomen on either side of the spine. As part of the urinary tract, the kidneys are responsible for blood filtration and excretion of water-soluble waste in the urine. Kidneys: Anatomy filling the fetal abdomen. The liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver: Anatomy is normal.
Image: “F0001” by the Department of Urology, Kasturba Medical College, Manipal, India. License: CC BY 2.0.A 24-year-old man with recurrent epididymitis Epididymitis Epididymitis and orchitis are characterized by acute inflammation of the epididymis and the testicle, respectively, due to viral or bacterial infections. Patients typically present with gradually worsening testicular pain and scrotal swelling along with systemic symptoms such as fever, depending on severity. Epididymitis and Orchitis and a ureteral insertion into the seminal vesicle Vesicle Primary Skin Lesions. A: A coronal Coronal Computed Tomography (CT) cut of the computed tomography (CT) scan showing an atrophic right pelvic kidney (yellow circle). B: On this coronal Coronal Computed Tomography (CT) cut of the CT scan, the ureter and seminal vesicle Vesicle Primary Skin Lesions complex can be seen (yellow ellipse).
Image: “Atrophic and ectopic right kidney” by the U.S. National Library of Medicine. License: CC BY 4.0.Horseshoe kidney on contrast-enhanced CT: Arrowheads show the isthmus Isthmus Uterus, Cervix, and Fallopian Tubes: Anatomy.
Image: “Enhanced abdominal computed tomography” by the Department of Urology, Hirosaki University Graduate School of Medicine, 5 Zaifucho, Hirosaki 036-8562, Japan. License: CC BY 2.0.A CT showing crossed fused ectopia: The ectopic kidney is situated anterolateral to the orthotopic Orthotopic Tissue grafted to the usual anatomic site (heart, lung, and liver transplants) Organ Transplantation kidney.
Image: “A computed tomography showing crossed fused ectopia” by the Department of Urology and Transplantation Surgery, Institute of Kidney Diseases and Research Research Critical and exhaustive investigation or experimentation, having for its aim the discovery of new facts and their correct interpretation, the revision of accepted conclusions, theories, or laws in the light of newly discovered facts, or the practical application of such new or revised conclusions, theories, or laws. Conflict of Interest Centre, Institute of Transplantation Sciences, Civil Hospital Campus, Asarwa, Ahmedabad, Gujarat, India. License: CC BY 2.0.