Multicystic Dysplastic Kidney with Twice Duplicated Distal Ureter Having Ectopic Blind Endings

j o u r n a l o f t h e a n a t o m i c a l s o c i e t y o f i n d i a 6 4 ( 2 0 1 5 ) 1 7 8 – 1 8 1

Available online at www.sciencedirect.com

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journal homepage: www.elsevier.com/locate/jasi

Case Report

Multicystic dysplastic kidney with twice duplicated

distal ureter having ectopic blind endings

* 1

A. Amar Jayanthi , K.T. Jisha

Government Medical College, Thrissur, Kerala, India

a r t i c l e i n f o a b s t r a c t

Article history: Congenital abnormalities of the kidneys, urinary collecting system, and bladder come at an

Received 10 January 2014 incidence of roughly 1% of total live births. These urinary abnormalities form a major

Accepted 31 August 2015 problem in urological studies both of interest to the surgeons and anatomists, of which

Available online 26 September 2015 inverted ‘‘Y’’ shaped ureter, ectopic ureter, blind ending ureter, and multicystic kidney are a

very rare spectrum of anomalies. Coexistence of such a complex spectrum of anomalies in

Keywords: the same individual is of immense surgical importance.

During dissection, retro peritoneum of intra uterine dead (IUD) male fetus of 28 weeks

Multicystic dysplastic kidney

(MCDK) gestation a combination of multicystic dysplastic kidney with a rare ureteral anomaly that is

not seen frequently was observed. The normal left renal anatomy was completely distorted.

Intra Uterine Death (IUD)

Ureter divided twice in an inverted ‘‘Y’’ branching pattern, the two left limbs entered the wall

Duplicated ureter

of rectum and urinary bladder of same side respectively and right limb opened ectopically

Ectopic ureter

into urinary bladder. The gross and microscopic features were in favor of Multicystic

Blind ending ureter

Dysplastic Kidney and Ureter. Congenital renal dysplasia may be explained by an abnormal

induction of metanephric blastema by migrating ureteric bud. The embryological basis of

inverted ‘‘Y’’ shaped ureter is not clearly understood, we suggest that it is due to longitudinal

splitting of ureteric bud. This type of anomaly of the ureter may represent real traps in the

interpretation of abdominal imaging, particularly in CT scanning.

# 2015 Anatomical Society of India. Published by Elsevier, a division of Reed Elsevier

in which the renal cortex is replaced by numerous cysts of

1. Introduction

multiple sizes, resembling a bunch of grapes, occurring in 1 in

1000 to 4300 live births, most often on the left side. MCDK

Congenital malformations represent defects in morphogene- shall be predominant in boys than girls. Most common age of

sis during early fetal life. Congenital abnormalities of the presentation was within ﬁrst month of life, and MCDK can be

kidneys, urinary collecting system, and bladder come at an diagnosed by high resolution ultrasonography in the neonatal

incidence of roughly 1% of total live births. Multicystic period. Multicystic kidney is usually unilateral, segmental or

dysplastic kidney (MCDK) is a congenital maldevelopment, focal associated with abnormalities of collecting system. It

* Corresponding author at: Additional Professor of Anatomy, Mulankunnathukavu Govt. Medical College, Medical College P.O. Thrissur –

680596, India. Tel.: +91 0487 2306972; mobile: +91 9447698881.

E-mail address: [email protected] (A. Amar Jayanthi).

Address: Assistant Professor of Pathology, Mulankunnathukavu Govt. Medical College, Medical College, P.O. Thrissur – 680596, India.

http://dx.doi.org/10.1016/j.jasi.2015.08.001

j o u r n a l o f t h e a n a t o m i c a l s o c i e t y o f i n d i a 6 4 ( 2 0 1 5 ) 1 7 8 – 1 8 1 179

may remain clinically silent throughout life or may involute

with time. In children who survive, there is an increased risk of

Wilm's tumor and renal cell carcinoma. 50% of MCDK are

associated with other urological defects such as ureteral

atresia, reduplication, meckel syndrome, neural tube defects,

Kallman syndrome, agenesis or dysplasia of the kidney, and

agenesis or cysts of the seminal vesicles.

Of the ureteric anomalies, biﬁd ureters and double ureters

are more frequently seen, but triple ureters are reported by

Boopathi in 1 out of 11 patients. According to the Committee

on Terminology, Nomenclature, and Classiﬁcation of the

Section on Urology of the American Academy of Pediatrics,

ureteral ectopia is deﬁned as a ureter that terminates into an

abnormal location. Several authors have described the

Fig. 2 – Microsopic appearance of left kidney stained with

possible ectopic endings of ureter in male and females. The

Hematoxylin and Eosin showing immature glomerulus

common sites in decreasing order in males are the posterior

and multiple tubules with dysplasia.

urethra, prostatic utricle, seminal vesicle, ejaculatory duct, vas

deferens and in females urethra, vestibule, vagina, cervix and

uterus, but the list remains without rectum. Ureteral ectopia

is 2–12 times more common in females. We report, to the best

of our knowledge, a rare occurrence of the coexistence of all (Fig. 1). The gross and microscopic appearance of the left

these anomalies in a single individual. kidney and ureters was observed in detail. Normal renal

anatomy was completely distorted. Left kidney measured

10 Â 7 Â 4 cm, dilated and cystic, extending from T10 to L5

2. Observation

vertebra. Cysts were of varying sizes from 2 to 12 mm, walls

thin and contained clear white ﬂuid. The opposite kidney was

Autopsy done in a male intra uterine dead fetus of 28 weeks, normal in shape, size, and structure.

obtained from Obstetrics and Gynecology Department of Three cms distal to its formation left ureter divided into two

Government Medical College, Thrissur, a combination of multi in an inverted ‘‘Y’’ manner. The left division had a normal

cystic dysplastic kidney with ureteral anomaly was observed termination into urinary bladder. The right division after

entering pelvis again had an inverted ‘‘Y’’ branching. Both

limbs ended blindly as well as ectopically, right one into

urinary bladder and left into rectum. Such a variant ureter

presented here was not seen in any of the available literature.

Small diameter of ureter hindered introduction of a probe.

Malformations of other organs were not observed. Photo-

graphs of kidney were taken after injecting two different color

solutions into the cyst. All the ﬁve limbs of ureter, bladder and

kidney sections were taken for histo-pathological studies for

conﬁrmation.

Microscopically kidney showed multiple dilated disorga-

nized tubules and ductules lined by single layer of low

cuboidal epithelial cells with collarrette of mesenchyme;

cartilage conﬁrming dysplasia (Fig. 2). Section of the

duplicated parts of ureter showed lumen lined by transition-

al epithelium and smooth muscle. Smooth muscle in the

walls of ureter conﬁrmed using mallory's trichrome stain

(Fig. 3).

3. Discussion

Anatomical variations and congenital anomalies of kidney and

ureter were well described by Edmund Papin and Daniel

Eisendrath. Usually ureteral reduplication is associated with a

hypoplastic kidney that develops around second ureter which

may end blindly below. In general, the more ectopic the

Fig. 1 – Showing gross appearance of kidney with multiple uereteral oriﬁce, the more the involved moiety of kidney. In

cysts of varying sizes resembling bunch of grapes and this case the multi cystic dysplastic kidney is associated with

duplicated distal part of ureter. partial reduplication of distal ureter which ends blindly below

180 j o u r n a l o f t h e a n a t o m i c a l s o c i e t y o f i n d i a 6 4 ( 2 0 1 5 ) 1 7 8 – 1 8 1

The embryology of inverted Y shaped ureter is incomplete-

ly understood. We suggest that it is due to the longitudinal

splitting of single normal ureteric bud. As ureteric bud grows

cranially toward the metanephros its growing end becomes fused.

4. Conclusion

Majority of abdominal urologic procedures are now performed

laparoscopically. Combination of anomalies in the same

individual may be the source of technical difﬁculties in the

identiﬁcation and dissection for the inexperienced laparo-

scopic surgeons. Hence awareness of such patterns of

Fig. 3 – Sections of the three limbs of distal part of ureter

malformations should arouse the suspicion and search for

showing wall made of smooth muscle lined by transitional

other anomalies.

epithelium and presence of lumen.

Conﬂicts of interest

into urinary bladder as well as into rectum. This may be The authors have none to declare.

explained by an abnormal induction of metanephric blaste-

ma by the migrating ureteric bud. It has been suggested

that displaced metanephric blastema interspersed with

normal zones of nephrogenes generates the irregular Acknowledgements

parenchyma of the multicystic kidney as evidenced in this

case also. The subsequent cystic dilatation of dysplastic The authors' thank Dr. Indira C.K., Associate Professor of

tissue is believed to compress and permanently damage the Anatomy for the help and support offered in doing fetal

normal renal tissue. autopsy and to Mr. Ashok Kumar T.K., Artist of Anatomy

Though there are many theories explaining multicystic department for the photography.

dysplastic kidney, biﬁd and duplex ureters, there is no

information regarding the etiology of double inverted Y

shaped ureter, and blind ending ureter opening into same

r e f e r e n c e s

side rectum and crossing midline to end blindly on opposite

side urinary bladder. The ureter develops from 3 to 5 mm

stage at the end of the 5th gestational week as an

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