|Year : 2022 | Volume
| Issue : 2 | Page : 110-112
Bilateral Poland anomalies with horseshoe kidney: A rare association
Partha Chakraborty1, Priti Majumder2, Rahuldeb Mondal3, Pankaj Halder1
1 Department of Pediatric Surgery, R G Kar Medical College, Kolkata, West Bengal, India
2 Department of Pediatric Medicine, R G Kar Medical College, Kolkata, West Bengal, India
3 Department of Anesthesiology, R G Kar Medical College, Kolkata, West Bengal, India
|Date of Submission||02-Sep-2022|
|Date of Acceptance||12-Nov-2022|
|Date of Web Publication||10-Jan-2023|
Dr. Pankaj Halder
Department of Pediatric Surgery, R G Kar Medical College, Saroda Pally, Baruipur, Kolkata - 700 144, West Bengal
Source of Support: None, Conflict of Interest: None
Poland syndrome (PS) is a rare congenital disorder characterized by ipsilateral syndactyly and unilateral hypoaplasia of the chest wall. It may also be accompanied by dextrocardia, vertebral deformities, and renal malformations such as undescended testes and pyelonephritis. The degree of intrathoracic visceral compression and chest wall involvement dictate the course of treatment. Surgical drainage is occasionally required to treat sepsis and chronic infection. We present the case of an 11-month-old infant who had bilateral PS, horseshoe kidneys, and right-sided hydronephrosis, which is quite a rare association as per existing literature.
Keywords: Association, horseshoe kidney, hydronephrosis, Poland syndrome, surgery
|How to cite this article:|
Chakraborty P, Majumder P, Mondal R, Halder P. Bilateral Poland anomalies with horseshoe kidney: A rare association. Muller J Med Sci Res 2022;13:110-2
|How to cite this URL:|
Chakraborty P, Majumder P, Mondal R, Halder P. Bilateral Poland anomalies with horseshoe kidney: A rare association. Muller J Med Sci Res [serial online] 2022 [cited 2023 May 28];13:110-2. Available from: https://www.mjmsr.net/text.asp?2022/13/2/110/367411
| Introduction|| |
Poland syndrome (PS) was first described by Alfred Poland in 1841. The incidence varies from 1/20,000 to 1/30,000 live births. A wide range of anomalies, from mild hypoplasia to aplasia of the breasts and ribs, as well as chest wall depression, thin subcutaneous fat, sternal anomalies, and the absence of axillary hair, are included in the spectrum of thoracic malformations in PS. The term acro-pectoral-renal field defect indicates unilateral renal agenesis or duplication of the urinary collecting system or pyelonephritis with PS. These patients usually remain asymptomatic and hence routine renal investigations get skipped, ultimately leading to an underreporting of the actual number of cases. We report a case of a PS in an 11-month-old child who with right-sided hydronephrosis, a horseshoe kidney (HSK), and right ureteric atresia needing surgical intervention.
| Case Report|| |
An 11-month-old boy presented to the department with a cystic space-occupying lesion (SOL) on the right side of the abdomen that was slightly moveable perpendicular to the mesenteric axis. A massive cystic SOL measuring 13 cm × 6.7 cm was found by ultrasonography (USG) on the right side of the abdomen, extending from the xiphisternum to the right side of the pelvis. The right kidney was not visible, while the left kidney was normal. On exploration, we found that the right inferior vena cava had been pushed to the right by a hydronephrotic right kidney that had emerged from the left retroperitoneal location. The right pelviureteric junction and right ureter could not be accurately defined. The renal parenchyma was so much thinner and larger. Hence, a tube nephrostomy was performed.
In the follow-up clinic, we further assessed the patient and performed a contrast-enhanced computed tomography (CT), which revealed a right kidney that was substantially enlarged (9.2 cm × 5.8 cm), along with a dilated pelvicalyceal system and no visibility of the right ureter. An isotope renogram using diethylenetriamine pentaacetate (DTPA) demonstrated substantially compromised right renal function (glomerular filtration rate of 5.68 ml/min). The lower poles of both the kidneys were fused together with a nonfunctioning isthmus. A little opacification of the right renal artery and vein was visible by a CT angiography [Figure 1]. In addition, we saw symmetrical deformities of the chest walls on both the sides without any actual practical restrictions on shoulder movement. On palpation, ribs appeared missing in the upper part of the chest on both sides, and only nine ribs on each side could be palpated.
|Figure 1: CT and CT angiography images demonstrate gross hydronephrosis with nonvisualization of the right ureter and fused kidney with nonfunctioning isthmus, respectively, in a patient with Poland syndrome. CT: Computed tomography|
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The neck seemed to be short as well. A chest radiograph revealed partial agenesis of the anterior arches of the seventh, eighth, and ninth ribs, as well as bilateral hypoplastic first, second, and third ribs. A USG of the bilateral chest wall revealed no muscle mass in the anterior chest wall and just a thin layer of pectoralis major muscle on both the sides in the upper lateral region [Figure 2]. After 3 months, a second DTPA scan showed no improvement in renal function. The blood hemogram and serum urea/creatinine, however, were both normal. The patient underwent re-exploration for a potential nephrectomy or pyeloplasty 5 months after the initial procedure. The right pelvis was identified and opened in between stay sutures. Further dissection revealed that the right ureter had developed into a string-like structure and had atretic at the distal end [Figure 3]. It was impossible to negotiate a 3-Fr DJ (doubled J) stent beyond the middle part of the ureter. The decision for the right nephrectomy was chosen. The postoperative recovery went without a hitch and an uncomplicated course in the follow-up clinic too.
|Figure 2: Chest X-ray and USG show bilateral hypoplastic ribs (1-3), partial agenesis of the anterior arches (7-9), and a thin layer of pectoralis major muscle on both sides in the upper lateral part in an 11-month-old boy with Poland syndrome. USG: Ultrasonography|
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|Figure 3: Intraoperative picture shows distal atretic right ureter, hydronephrotic right kidney in patient of Poland syndrome with horseshoe kidney|
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| Discussion|| |
The lack of the sternal and costal heads of the pectoralis major muscle, the chest wall, and hand abnormalities are the most distinguishing characteristics of PS (syndactyly, variable degrees of brachydactyly, and severe hypoplasia or aplasia of the middle phalanges). According to reports, the right side exhibits more than 75% of the aforementioned flaws. The numerous other related defects identified are hemivertebrae, renal anomalies, dextrocardia, Sprengel deformity, clubfoot, and submucous cleft palate. In our case, the youngster exhibited a short neck, bilateral chest wall deformity, right-sided hydronephrosis, and HSK but no upper limb abnormalities. According to the traditional definition, PS involves unilateral involvement. In our case, the infant showed bilateral hypoplasia and depression of the chest walls in addition to the absence of the ribs. The bilateral Poland anomaly has been reported infrequently in the literature and termed "Thoracic Dysplasia."
Numerous etiopathogenetic factors, such as congenital vascular maldevelopment, intrauterine insults, drugs, and attempted abortions, have been proposed as potential causes. The name "subclavian artery supply disruption sequence," coined in 1986 by Bavinck and Weaver, suggests a shared etiology for the Poland, Klipple–Feil, Moebius, and Sprengel malformations, which typically manifest in the 5th or 6th week of pregnancy. How much the embryonic blood flow (through the subclavian and vertebral arteries) is constrained determines the range of abnormalities. Renal malformations have been linked to the maldevelopment of the metanephros, which affects the ureteral bud and the mesonephric duct, in addition to the familial type of megacalycosis of the kidney., The mechanical displacement may result in cardiac dextroposition in PS. The thin chest wall and missing or malformed ribs might cause the heart to lean to the right.
As a result of standard renal studies being skipped because these individuals typically do not exhibit any symptoms, the true number of cases is likely underreported. The course of treatment is determined by the degree of functional impairment and chest wall involvement, as well as by any evident compression of the thoracic viscera. A computer-aided design pectoralis muscle implant is part of the surgical treatment for PS. Tissue expansion and lipofilling silicone breast implants are advised for subcutaneous and mammary atrophy. PS has not been associated with any defects that are life-threatening; hence, life expectancy remains unaffected. Thus, we can infer that in addition to a skeleton scan, a thorough evaluation of other systems, such as the genitourinary system, should be performed in a confirmed case of PS.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]