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CLINICAL IMAGES
Year : 2014  |  Volume : 139  |  Issue : 4  |  Page : 648-649

Potter's syndrome - a fatal constellation of anomalies


1 Department of Pathology, Armed Forces Medical College, Pune 411 040, India
2 Department of Pathology, Command Hospital Southern, Command, Pune 411 040, India

Date of Web Publication9-Jun-2014

Correspondence Address:
S. Kakkar
Department of Pathology, Command Hospital Southern, Command, Pune 411 040
India
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Source of Support: None, Conflict of Interest: None


PMID: 24927356

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How to cite this article:
Manoj M G, Kakkar S. Potter's syndrome - a fatal constellation of anomalies. Indian J Med Res 2014;139:648-9

How to cite this URL:
Manoj M G, Kakkar S. Potter's syndrome - a fatal constellation of anomalies. Indian J Med Res [serial online] 2014 [cited 2019 Apr 20];139:648-9. Available from: http://www.ijmr.org.in/text.asp?2014/139/4/648/134144

An autopsy requisition of a stillborn, male foetus born to a 23 year old primigravida, was received in the department of Pathology, Command Hospital, Pune, India, in July 2012. Autopsy findings were hypoplastic mandible; left lower limb contracture; hypoplasia of right lower limb; bilateral renal dysgenesis; post prostatic urethral obstruction; and bilateral hypoplastic lungs [Figure 1], [Figure 2], features classical of Potter's syndrome [1],[2] .

After 16 weeks, amount of amniotic fluid depends on foetal urine production. In these cases decreased urine production is caused by bilateral renal agenesis and/or by obstruction in the urinary tract. Urine is critical for development of lungs by aiding in expansion of alveoli by means of hydrodynamic pressure and by supplying Proline, a critical amino acid for lung maturation. Urine also serves to cushion the foetus [1] . There is no known prevention for this fatal condition. Hence, ultrasound screening for oligohydramnios and urogenital anomalies is recommended, between 16-18 wk of gestation [3] .
Figure 1 (A). Hypoplastic mandible. (B). Hypoplasia of right lower limb (below knee) and contracture of left lower limb. (C). Dilated urinary bladder (asterisk) and bilateral hydroureters (black arrowheads).

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Figure 2 (A). Hematoxylin and Eosin stained section of kidney with inset showing the island of immature chondroid tissue (arrow) (1000X). (B). Hematoxylin and Eosin stained section of urinary bladder showing a flattened transitional epithelium and marked hypertrophy of the muscularis propria (arrow) (400X). (C). Hematoxylin and Eosin stained section of lungs showing the un-aerated alveoli (400X), with the inset showing the cuboidal pneumocytes (arrow) (1000X).

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   References Top

1.Hjort C, Larsen CO, Nathan E. Potter's sequence. Phenotype, pathogenesis, etiology and hereditary aspects. Ugeskr Laeger 1992; 154 : 488-91.   Back to cited text no. 1
    
2.Thomas IT, Honore GM, Jewett T, Velvis H, Garber P,Ruiz C. Holzgreve syndrome: recurrence in sibs. Am J Med Genet 1993; 45 : 767-9.  Back to cited text no. 2
    
3.Roodhooft AM, Birnholz JC, Holmes LB. Familial nature of congenital absence and severe dysgenesis of both kidneys.N Engl J Med 1984; 310 : 1341-5.  Back to cited text no. 3
    


    Figures

  [Figure 1], [Figure 2]



 

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