Pathophysiological clinical features of an infant with hypertension secondary to multicystic dysplastic kidney: a case report

Abstract Background The association of hypertension with congenital renal hypoplasia has been established. We report a case of an infant who underwent nephrectomy for hypertension. Case presentation Magnetic resonance imaging for the mother revealed fetal renal masses, and fetal multicystic dysplastic kidney was suspected. Following birth, the baby developed hypertension. Numerous investigations revealed that the left kidney was non-functional, and she was initiated on benazepril hydrochloride. However, because the drug response was poor, the left kidney was removed at the age of 7 months. Examination of the renal specimen revealed abrupt transition from normal to atrophic cortex with lobar atrophy and cysts. Tubular atrophy, marked abnormal blood vessels with wall thickening, gathered immature glomeruli, and parenchymal destruction were observed. Renin was partially localized in the proximal tubules and the parietal epithelium of the Bowman’s capsule in the immature glomeruli. We speculated that an abnormal vascular structure and irregular renin localizations may be the cause of hypertension. Serum renin and aldosterone levels gradually reduced post-surgery, reaching normal levels on the 90th postoperative day. A long follow-up is needed due to the possibility of the child developing hypertension in the future. Conclusion This is a case of an infant with MCDK, which discusses the clinicopathological features based on the pathophysiological analysis, including renin evaluation.

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Laparoscopic transposition for crossing vessels (vascular hitch) in pure extrinsic pelvic-ureteric junction obstruction: a successful case report of a 2-year-old infant with horseshoe kidney

Surgical Case Reports ◽

10.1186/s40792-021-01190-y ◽

2021 ◽

Vol 7 (1) ◽

Author(s):

Satoshi Ieiri ◽

Kouji Nagata

Keyword(s):

Laparoscopic Surgery ◽

Left Kidney ◽

Horseshoe Kidney ◽

Renal Scintigraphy ◽

Multicystic Dysplastic Kidney ◽

Extrinsic Compression ◽

Dysplastic Kidney ◽

Renal Vessels ◽

The Right ◽

Crossing Vessels

Abstract Background Pediatric hydronephrosis induced by pelvic-ureteric junction obstruction (PUJO) is treated by dismembered pyeloplasty (DP) via open and laparoscopic surgery. The etiology of PUJO involves both intrinsic stenosis and extrinsic compression of crossing vessels (CVs). PUJO owing to CVs is also treated by DP, as there is no consensus concerning this vascular condition. We encountered a 2-year-old infant with pure extrinsic PUJO combined with horseshoe kidney who successfully underwent laparoscopic transposition for CVs (vascular hitch). Case presentation A 2-year-old boy was prenatally diagnosed with left multicystic dysplastic kidney (MDCK) and right hydronephrosis and received a definitive diagnosis after birth. At 6 months old, renal scintigraphy revealed a non-functioning pattern in the left kidney and an obstructive pattern in the right, showing no response to furosemide loading. The patient also had recurrent urinary tract infection, and his right hydronephrosis gradually worsened. We decided to perform surgery for the right PUJO. Preoperative enhanced computed tomography detected three right renal vessels independently branching from the abdominal aorta. The middle renal vessels were located at the ventral side of the pelvis and coincident with the site of PUJO. These vessels were suspected of being CVs. The patient underwent laparoscopic surgery electively. A 5-mm trocar was inserted at the umbilicus for a 5-mm, 30° rigid scope. Two additional ports were then inserted under laparoscope inspection. The dilated right pelvis and CVs were detected after ascending colon mobilization. To confirm the pathogenesis of PUJO, the CVs were dissected and taped. After taping the CVs, an intraoperative diuretic test was performed using furosemide loading. Peristalsis of the right ureter was recognized, and the extrinsic PUJO owing to the CVs was definitively confirmed. We therefore performed transposition for the CVs (vascular hitch procedure). The CVs were mobilized in the cranial direction and those were wrapped by dilated pelvis. The post-operative course was uneventful. The renal scintigraphy findings improved and showed a favorable response of furosemide loading. Conclusions The laparoscopic vascular hitch procedure is minimally invasive and effective for extrinsic PUJO due to CVs. Anastomotic stricture after Anderson and Hynes DP can be prevented by appropriate patient selection.

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Multicystic Dysplastic Kidney: Conservative Management and Follow-Up

Renal Failure ◽

10.1081/jdi-48221 ◽

2005 ◽

Vol 27 (2) ◽

pp. 189-192 ◽

Cited By ~ 9

Author(s):

Said Al-Ghwery ◽

Abdulrahman Al-Asmari

Keyword(s):

Conservative Management ◽

Multicystic Dysplastic Kidney ◽

Dysplastic Kidney

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575: Is Follow Up Necessary in Patients with Unilateral Multicystic Dysplastic Kidney?

The Journal of Urology ◽

10.1016/s0022-5347(18)32821-0 ◽

2006 ◽

Vol 175 (4S) ◽

pp. 186-186

Author(s):

Bulent Onal ◽

Barry A. Kogan

Keyword(s):

Multicystic Dysplastic Kidney ◽

Dysplastic Kidney

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Early Nephrectomy in Unilateral Multicystic Dysplastic Kidney in Children Cures Hypertension Early: An Observation

European Journal of Pediatric Surgery ◽

10.1055/s-0037-1599837 ◽

2017 ◽

Vol 27 (06) ◽

pp. 533-537 ◽

Cited By ~ 2

Author(s):

Vijai Upadhyaya ◽

Manish Gupta ◽

Laxmi Bharti ◽

Ram Rao ◽

Sheo Kumar ◽

...

Keyword(s):

Blood Pressure ◽

Antihypertensive Drugs ◽

Case Series ◽

Multicystic Dysplastic Kidney ◽

Time Duration ◽

Level Of Evidence ◽

Variable Time ◽

Dysplastic Kidney ◽

Diagnosis Of Hypertension

Objective Surgery is still indicated as the treatment of choice in subset of patients with unilateral multicystic dysplastic kidney (UMCDK) because of its potential complications and malignant change. The purpose of this study is to present our observation that early nephrectomy may cure hypertension early in children with UMCDK and review the literature. Materials and Methods We report here four children (two males and two females) with antenatally diagnosed UMCDK with hypertension, treated in the past 4 years. All have antenatal diagnosis of UMCDK and referred to us after their birth. Diagnosis of hypertension (blood pressure > 95th percentile) was made after 3 months of follow-up in all patients. Nephrectomy was performed in all children in variable time duration. Hypertension, urinary tract infection, and desire of parents were the main indication of surgery. Results All children showed decrease in the size of their dysplastic kidney with time, but hypertension was persistent and needed antihypertensive drugs; even increase in the doses in successive follow-up. After nephrectomy, three children who were operated early got cured having normal blood pressure within variable time duration, while a child, operated late, still had high blood pressure and needed antihypertensive drugs. Conclusion Early nephrectomy in recently diagnosed hypertension in UMCDK is advisable and can cure hypertension early. Levels of Evidence The level of evidence is IV (case series with no comparison group).

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Simple multicystic dysplastic kidney disease: end points for subspecialty follow-up

Pediatric Nephrology ◽

10.1007/s00467-007-0635-7 ◽

2008 ◽

Vol 23 (1) ◽

pp. 111-116 ◽

Cited By ~ 20

Author(s):

Adam Weinstein ◽

T. Rob Goodman ◽

Sandra Iragorri

Keyword(s):

Kidney Disease ◽

Multicystic Dysplastic Kidney ◽

End Points ◽

Dysplastic Kidney ◽

Multicystic Dysplastic Kidney Disease

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Multicystic dysplastic kidney detected by fetal sonography: conservative management and follow-up

Pediatric Surgery International ◽

10.1007/s003830000449 ◽

2001 ◽

Vol 17 (1) ◽

pp. 54-57 ◽

Cited By ~ 29

Author(s):

E. A. Oliveira ◽

J. S. S. Diniz ◽

A. S. Vilasboas ◽

E. A. S. Rabêlo ◽

J. M. P. Silva ◽

...

Keyword(s):

Conservative Management ◽

Multicystic Dysplastic Kidney ◽

Dysplastic Kidney

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Congenital solitary functioning kidney

10.1093/med/9780199592548.003.0351 ◽

2015 ◽

Author(s):

Michiel F. Schreuder

Keyword(s):

Renal Injury ◽

Renal Agenesis ◽

Multicystic Dysplastic Kidney ◽

Kidney Donation ◽

Adult Age ◽

Solitary Functioning Kidney ◽

Dysplastic Kidney ◽

Functioning Kidney

The main cause for unilateral non-formation or non-functioning of a kidney can be found in renal agenesis/aplasia and multicystic dysplastic kidney. Even though kidney donation at adult age is considered safe, studies in recent years have shown that this may be different in congenital solitary functioning kidneys. Whether this is based on dysplasia in the remaining kidney or based on glomerular hyperfiltration damage, follow-up has shown renal injury, defined as hypertension and/or proteinuria, in up to 32% of children with a congenital solitary functioning kidney. Therefore, long-term infrequent follow-up of all patients with a congenital solitary functioning kidney seems to be indicated.

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Presence of an Isolated Hydatid Cyst in the Left Kidney: Report of a Case of This Rare Condition Managed Surgically

Case Reports in Urology ◽

10.1155/2016/6902082 ◽

2016 ◽

Vol 2016 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Daniel Paramythiotis ◽

Petros Bangeas ◽

Konstantinia Kofina ◽

Vassileios Papadopoulos ◽

Antonios Michalopoulos

Keyword(s):

Hydatid Cyst ◽

Left Kidney ◽

Rare Condition ◽

Clinical History ◽

Cystic Mass ◽

Renal Masses ◽

Case Presentation ◽

Radiologic Imaging ◽

Laboratory Examinations ◽

Upper Abdominal

Introduction.Hydatid cyst disease caused byEchinococcus granulosusis rarely presented in the kidneys, whereas isolated renal occurrence is estimated to be about as low as 2–4% of all cases. We present a case of a female patient suffering from this condition that was treated successfully in our department.Case Presentation.A 44-year-old woman was incidentally diagnosed with a 14 cm left renal cystic mass through ultrasound imaging performed during upper abdominal pain investigation. Laboratory examinations were normal and CT imaging set the diagnosis of an isolated left renal hydatid cyst. The cyst was excised and the postoperative period was uneventful.Discussion.Isolated renal hydatid cyst is a very rare condition and could possibly be misdiagnosed with other renal masses. The clinical history, laboratory tests, and thorough radiologic imaging are crucial for the accurate preoperative diagnosis.

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