A Case Of Severe Hydronephrosis Managed As Cystic Kidney Disease

Summary

Congenital anomalies of the genitourinary tract, including congenital hydronephrosis, are the most common morphological abnormalities diagnosed by fetal ultrasound, but there are many cases in which it is difficult to differentiate them from cystic kidney disease. The case was a 2-year-old boy. Hydronephrosis was noted during the fetal period, but due to a family history of cystic kidney disease, it was diagnosed as the same disease.

Although he was under observation, he was referred to our hospital for re-evaluation. Close examination revealed obstruction of the left renal pelvic ureteric junction and severe renal pelvic dilatation, and a diagnosis of severe hydronephrosis was made. MAG3 scintigraphy revealed renal function <40% and poor urinary drainage, and pyeloplasty was performed at the age of 1 year. One year has passed since the surgery, and although left renal pelvis enlargement remains, renal function, including urine and blood tests, is within normal range. In this case, the influence of family history and the unconfirmed diagnosis resulted in excessive anxiety about the genetic disease. In cases where differentiation is difficult, careful evaluation and diagnosis, including the timing of diagnosis, are necessary.

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Preface

The widespread use of fetal ultrasound (US) has enabled early detection and diagnosis of congenital anomalies of the kidney and urinary tract (CAKUT) such as cystic kidney disease and hydronephrosis during the fetal period. Now you can do it 1).

However, in some cases, it is difficult to differentiate between severe hydronephrosis and cystic kidney diseases such as multicystic dysplastic kidney (MCDK), which may lead to a pitfall in prenatal diagnosis. This time, I experienced a case of severe hydronephrosis in a man with a strong family history of polycystic kidney disease (PKD), which was managed as cystic kidney disease.

Report.

case

Case: 2 years old, boy

Chief complaint: Close examination of renal cyst

History of oral medication during pregnancy: No angiotensin-converting enzyme inhibitors or angiotensin receptor antagonists. Levothyroxine was taken due to subclinical hypothyroidism.

Pregnancy course: No abnormalities were noted until the 29th week of pregnancy, and the course of pregnancy was uneventful. At 31 weeks and 4 days of pregnancy, fetal US revealed hydronephrosis on the left side (Society for Fetal Urology: SFU grade 3) and on the right side (SFU grade 1) for the first time. Although the family history showed a strong history of PKD, it was also explained that there was a possibility of MCDK in the family, as large and small cyst-like dilations were observed in the fetal left kidney in late pregnancy. ．． Perinatal history: Natural pregnancy. The baby was born at a gestational age of 39 weeks and 4 days, weighing 2862 g, and was delivered normally. Apgar 9/10 points (1 minute/5 minutes). Past medical history: Nothing noteworthy

Family history: see Figure 1

History of present illness: After giving birth, the patient was under observation as his MCDK at a nearby doctor. When the patient visited a local doctor for a cold, his family became concerned about his strong family history of cystic kidney disease, and at 8 months of age, he was referred to our hospital for detailed examination. Physical findings (first visit): Height: 67.6 cm (-1.5 SD), weight: 7.6 kg (-1.0 SD). Blood pressure: 90/45 mmHg. The patient's general condition was good, and no abnormalities were found in cardiopulmonary or abdominal findings. There were no abnormalities in facial appearance, limbs, skeleton, or external genitalia. Laboratory findings (Table 1): Urinalysis showed specific gravity 1.004, protein (±), occult blood (-), red blood cells <1/HPF, white blood cells <1/HPF, casts (-), protein/Cr67 (67) day old kidney. Magazine Vol. 34 No. 1

●Case report●

A case of severe hydronephrosis that was managed as cystic kidney disease Yuichi Morimoto, Rina Oshima, Takuji Shioya, Kohei Miyazaki, Mitsuru Okada, Keisou Sugimoto (Reception date: September 29, 2020 Hiring date: February 2021 (Monday 2nd) Key words: Fetal ultrasound examination / hydronephrosis / cystic kidney disease /

Mild urine protein and elevated 2-MG were observed, with 0.19 g/gCr and 2-microglobulin (2-MG)/Cr 1.04 g/mgCr. Blood tests showed Na 141 mEq/L, K 5.0 mEq/L, total protein 7.4 g/dl, albumin 4.9 g/dl, urea nitrogen 7 mg/dl, creatinine (Cr) 0.13 mg/dl, and estimated glomerular filtration rate. (eGFR) was 154.82 l/min/1.73 m2, cystatin C was 0.7 mg/L, and no decline in renal function was observed. eGFR was calculated by multiplying the quintic equation by the coefficient R (1.619) according to the pediatric CKD diagnosis guide.

Imaging findings: His US at the time of his first visit to our hospital showed marked dilation of the renal pelvis, dilatation of almost all calyces, and thinning of the renal parenchyma in the left kidney, similar to the final examination at the maternity hospital (Figure 2c). ). Abdominal MRI revealed marked dilatation of the renal pelvis and dilatation of almost all calyces in the left kidney. No ureteral dilatation was observed (Figure 2d). His 99mTc-DMSA scintigram, which was performed to determine whether he was suitable for surgery, showed decreased left kidney function, with a left kidney uptake rate of 12.4% and a right kidney uptake rate of 21.4% (Figure 2e). MAG3 scintigram showed that the left kidney was 31.2% and the right kidney 68.8%, and after diuretic loading, both Tmax and T(1/2) were prolonged in the left kidney, showing an obstructive pattern (Figure 2f). Voiding cystourethrography (VCUG) revealed no vesicoureteral reflex (VUR). Progress: Based on the results of various tests, the diagnosis was not MCDK but left severe hydronephrosis. After explaining the surgical indications to the parents and obtaining their consent, pyeloplasty was performed at the age of 1 year. The course was uneventful, including the perioperative period. US at the age of 1 year and 8 months (8 months after surgery) showed that the renal pelvis enlargement of the left kidney had slightly improved (Figure 2g). Currently, there is no worsening of eGFR, and both the urinary protein/Cr ratio (0.13 g/gCr) and the urinary MG/Cr ratio (3.04 þ g/mgCr) are within the normal range.

There are also findings that are useful in differentiating between the two, such as the largest cyst located in the center in renal pelvic-ureteric junction obstruction, whereas it is located peripherally in MCDK8),9). Although the brightness of the fetal US in this case was unclear, the location of the largest cyst was central, a finding that suggested hydronephrosis. The obstetrician and gynecologist who actually examined her initially diagnosed hydronephrosis, but because she had a strong family history of polycystic kidney disease, she was later diagnosed with cystic kidney disease, including MCDK. He explained to his family the possibility of kidney disease. In this case, the father was not confirmed to have developed cystic kidney disease (only US was performed; genetic testing was not performed), and the child's lesions were localized to the left kidney. The possibility of a genetic abnormality was thought to be low. Genetic testing was performed on her using the CAKUT panel (including PKD1, PKD2, PKHD1, and HNF1B) at the Department of Pediatrics, Kobe University School of Medicine, and no genetic mutations were found in the child. The medical institution where the patient was requested to undergo follow-up after birth did not have a specific policy for testing or treatment to differentiate between the two, which increased the patient's family's anxiety about the genetic nature of the disease. It is possible that In this case, we should have explained that the possibility of hereditary cystic kidney disease was low based on the unilateral nature and postnatal ultrasound findings, and then proceeded to differentiate between severe hydronephrosis and MCDK. It was done. Even when follow-up is performed as MCDK, contralateral renal urinary tract abnormalities are observed in approximately 1/3 of cases, and hydronephrosis, ureterocele, hypoplastic kidney, and internal genital organ abnormalities other than the renal urinary tract may also occur. It was considered desirable to plan early imaging tests, taking into consideration the gender of the patient.

The indication for surgery for severe hydronephrosis (SFU classification grade 3 to 4) is determined based on the comprehensive judgment of partial kidney function <40%, poor urinary drainage, and rate of decrease in partial kidney function >5 to 10%10) ．． In this case, no decrease in VUR, serum Cr, or eGFR was observed, and the patient had asymptomatic hydronephrosis, but an evaluation of renal function showed a decrease of less than 40% in the left kidney and poor drainage. was used as the criterion. The postoperative course, including the perioperative period, was uneventful, and no renal dysfunction has appeared or progressed, although dilatation of the left renal pelvis and calyx remains. It is known that hydronephrosis, which occurs late in the fetus or due to partial obstruction, also causes thinning of the renal cortex, fibrosis, and a decrease in the number of nephrons11),12) and that children diagnosed prenatally with CAKUT. In a cohort study of 822 people, hydronephrosis was found to be a high risk factor for chronic kidney disease (hazard ratio 5.20), which suggests that careful follow-up is necessary13).

Conclusion

The significance of fetal diagnosis using fetal US is very useful, but there are many diseases that can cause pitfalls in the diagnosis of CAKUT, including cystic kidney disease, and more careful judgment and sufficient explanation are required in consideration of family background. It is.

Acknowledgment

We would like to express our deepest gratitude to Dr. Ijima Issei, Dr. Toro Nozu, and Dr. Naoya Morisada of the Department of Pediatrics, Department of Internal Medicine, Kobe University Graduate School of Medicine, for conducting the genetic testing for this case. "There are no disclosures regarding conflicts of interest based on the standards set by the Japanese Society of Pediatric Nephrology."

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