Renal Hypoplasia: Case Literature Review

Introduction: Congenital anomalies of kidney and urinary tract (CAKUT) presents a broad range of disorders including abnormal renal parenchymal development, disrupted parenchymal development, abnormalities of lower urinary tract, urinary collecting system. The knowledge of these anomalies will help the clinicians and surgeons during the procedures of kidney. The purpose of this article is to present one of the cases of renal hypoplasia and the review that highlights renal hypoplasia cases. Methodology: A vertical incision has been made in midline from xiphoid process to pubic symphysis encircling the umbilicus, another incision from pubic symphysis laterally up to the anterior superior iliac tubercle along the iliac crest to expose the abdominal cavity. Details of the position suprarenal gland and the upper poles of the kidneys and external appearance of kidneys in situ were noted. The arrangement of the attached structures such as the hilum with structures passing through, ureter, bladder, abdominal aorta and the inferior vena cava were also noted and recorded. Observation and Result: The size of the left kidney was found small in comparison to right kidney. Coronal section of left kidney also showed a smaller number of renal pyramids, major calyces and minor calyces. Conclusion: The knowledge of anatomical variations of urinary system has a significant role for clinicians and surgeons for diagnosing diseases including congenital anomalies, radiological interpretations, procedures, surgical interventions and management of clinical conditions.

Two Cases of Hypertension Associated with Congenital Unilateral Renal Hypoplasia / 대한신장학회잡지

Renal hypoplasia is defined as a subnormal growth of the kidney with a distinct cortex and medulla and is recognizable as a kidney. It is known that renal hypoplasia can induce hypertension by reduction of functioning nephron. Patients with reduced functioning renal mass by surgical or medical causes have a risk to develop hypertension. Patients with congenital reduction of functioning nephron have more frequently showed renal insufficiency. But renal hypoplasia induced hypertension is very rare and reported rarely in international literature. We report two cases of hypertension associated with congenital renal hypoplasia.

A Case of Fryns Syndrome

Fryns syndrome is a lethal syndrome of multiple congenital anomalies first described by Fryns et al in 1979. A recently developed major diagnostic criteria includes abnormal face, small thorax with widely spaced hypoplastic nipples, distal limb and nail hypoplasia, lung hypoplasia with diaphragmatic hernia, central nervous system anomalies and congenital heart disease. The pathogenesis of Fryns syndrome is not clear. Of the major immediate life-threatening abnormalities of this syndrome, lung hypoplasia associated with diaphragmatic hemia has usually proven to be fatal. We report a case of Fryns syndrome, which has the prenatal ultrasonographic findings of Dandy-Walker malformation and renal hypoplasia.

Vas anomaly associated with ipsilateral renal hypoplasia / 대한비뇨기과학회지

Congenital anomaly of the vas deferens is often easily diagnosed by careful examination of the scrotum as well as by present popularity of vasectomy. By the fact that both systems originate from a common urogenital ridge of mesoderm, anomaly of the kidney has long been recognized to coexist with anomaly of the vas deferens. So, inability to palpate the vas deferens in routine examination or absence of vas deferens in vasectomy should alert the physician to study the patient for associated renal anomaly. We report one case of vas anomaly associated with ipsilateral renal hypoplasia with the brief review of literatures.

Aortorenal Bypass with Saphenous Vein in the Patient of Left Renal Arterial Stenosis and Right Renal Hypoplasia / 대한비뇨기과학회지

Renal hypertension is found to be the cause in 5-15% of patients with hypertension, and may be vascular or renal parenchymal in nature. Recent advances in diagnostic technique and vascular reconstructive techniques now enable successful management in many patients with renal hypertension. We observed one case of renovascular hypertension due to right renal hypoplasia and left renal arterial stenosis, in 22 year-old female patient with complaints of headache and dizziness for 10 days. The treatment was done by right nephrectomy and left aortorenal bypass procedure with saphenous vin. Herein we reported a case of renovascular hypertension with a review of the literatures.

A Case of Renal Cell Carcinoma with Contralateral Renal Hypoplasia / 대한비뇨기과학회지

One patient who underwent radical nephrectomy for renal cell carcinoma participated in a follow up study of the contralateral hypoplastic renal function. Compensatory renal enlargement was demonstrated on follow excretory urogram. The enlargement was 12mm in length, 16mm in width. Immediately, postoperative renal function, GFR, was decreased to minimum value, 8.6 ml/min., but GFR of the remaining kidney was increased to maximal value, 37 ml/min. on post-op 5th day, and then decreased. After post-op 2.6 month the GFR was maintained as the level of 13-14 ml/min. Plasma BUN and creatinine was increased to maximum value, BUN 46.1 mg/dl Cr. 4.1mg/dl, on post-op 9th day and then decreased continuously. But these BUN and creatinine level were maintained as 29.3 mg/dl, 3.4 mg/dl on post-up 6th month. We present a case of this renal cell carcinoma with contralateral renal hypoplasia.

Two Cases of Unilateral Renal Hypoplasia / 대한비뇨기과학회지

Renal hypoplasia is a relatively rare congenital disease. We report two cases of unilateral renal hyperplasia with review of literature.

A Case of Replacement Lipomatosis Associated with Unilateral Renal Hypoplasia / 대한비뇨기과학회지

Replacement lipomatosis of kidney which has been recognized as invasion of kidney by fat and extensive replacement of cortex with lipomatous growth, is always associated with kidney infection and with calculus disease of most cases. Lipomatosis in congenital renal hypoplasia is a very rare condition. In a 37 year old female patient suffering from left pyelonephritis, we incidentally found that right kidney was a fuctionless hypoplastic one by serial studies. Right nephrectomy was taken to prevent from possible renal hypertension and other complications. On pathologic examination, right kidney revealed severe replacement lipomatosis on hypoplastic kidney. Herein we report this case with review of some literatures.

Two Cases of Congenital Renal Hypoplasia / 대한비뇨기과학회지

The incidence of congenital unilateral renal hypoplasia is extremely rare.Two cases of congenital unilateral renal hypoplasia were presented with review of literature.

Congenital Hypoplastic Kidney associated with Tuberculosis / 대한비뇨기과학회지

One case of congenital hypoplastic kidney associated with tuberculosis in 28 year old man is presented and related literatures reviewed.

Ectopic Ureter Associated with Congenital Hypoplasia of the Kidney / 대한비뇨기과학회지

A 14-year old girl presented with urinary incontinence along with normally secured voluntary voiding. Careful physical examination revealed leakage of urine through a small opening near the vestibule, ventral to the urethral meatus. Cystoscopically, the right ureteral orifice was absent but otherwise normal. Nonfunctioning right kidney was found on excretory urography. A #4 ureteral catheter was inserted into the ectopic opening as high as 10cm, and the retrograde pyelogram showed dilated right ureter ending blindly at the level of lumber vertebra II with stricture at the level of the tip of the catheter. After the right nephroureterectomy, she became dry and was discharged on the 12th postoperative day. The removed kidney weighed only 8 Gm. and pathological diagnosis was congenital hypoplasia of the right kidney with ectopic ureteral opening.