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Year : 2002  |  Volume : 12  |  Issue : 3  |  Page : 448-450
Pelvic pancake kidney in klippel feil syndrome


9th Officer Colony, Pink Avenue, Mogapair, Chennai 50, India

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How to cite this article:
Kumar K S, Singh S, Govindan A, Jagadeesan K. Pelvic pancake kidney in klippel feil syndrome. Indian J Radiol Imaging 2002;12:448-50

How to cite this URL:
Kumar K S, Singh S, Govindan A, Jagadeesan K. Pelvic pancake kidney in klippel feil syndrome. Indian J Radiol Imaging [serial online] 2002 [cited 2020 Aug 8];12:448-50. Available from: http://www.ijri.org/text.asp?2002/12/3/448/28512
Sir,

A 21-year-old man was seen in our Emergency Room with a history of injury to the left foot following a road traffic accident. On examination the patient was conscious oriented, well built and with a short webbed neck. The neck movements were restricted and abduction of the upper limbs was not possible beyond 120 degrees. Examination of the left foot showed a lacerated wound 2" x 1" exposing the cuneiform bones.

The radiographs of the left foot showed fractures of the heads of the 2nd, 3rd and 4th metatarsals [Figure - 1]. A Lisfranc's fracture dislocation of the left mid foot was diagnosed with the fracture of the cuboid and dislocation of all the cuneiform bones. The webbed neck, limitation of neck movements and arm abduction prompted us to take radiographs of the chest and cervical spine which showed spina bifida C4 and C5, block vertebra involving C6 and C7 and hypoplasia of the left first rib. A large completely ossified omovertebral body was seen on the left side between C7 and the scapula [Figure - 2], probably the reason for the inability to abduct the arms beyond 120 degrees. The ultrasound of the abdomen and pelvis failed to locate the kidneys in their normal position instead these were found in the pelvis beyond the bladder as one single mass of renal tissue. An Excretory Urogram [Figure - 3] and Computed Tomogram [Figure - 4] were performed for further classification of the sonographic findings and these too demonstrated a single pelvic pancake kidney and two ureters. The bladder and rest of the abdominal viscera were normal. Based on the skeletal findings a diagnosis of Klippel Feil Syndrome was made

Klippel Feil Syndrome consists of complex viscero skeletal anomalies which include skeletal manifestations like block vertebra [1], spina bifida, hypoplastic rib and visceral anomalies involving the urogenital tract and the heart. The common renal anomalies reported in Klippel Feil Syndrome are unilateral renal agenesis, crossed renal ectopia, duplication of the collecting system or very rarely bilateral absence of kidneys which when present is incompatible with life [4]. Fusions of kidneys and rarely separate kidneys with fused pelves have also been reported [5]. During intrauterine growth (4th-5th week) the blastoma of the cervical vertebrae, scapula and the genito urinary system have an intimate spatial relationship which explains the high incidence of congenital anomalies in the scapula and the genitourinary system in association with Klippel Feil Syndrome. The incidence of renal anomalies has been recorded in 33% to 52% of the cases and Sprengel shoulder in 42% of the cases [3]. Congenital heart disease has been reported in 14% of the cases.

Our patient had fused C6 and C7 vertebrae with high placed scapulae on both sides and a very large omovertebral bone almost resembling an accessory scapula. A review of published data does not show an exact guideline for measurement of omovertebral bone size but on comparison our patient had a very large omovertebral bone. He also had a 'pancake' kidney in the pelvis, a hitherto unreported renal anomaly, with separate ureters joining the bladder on either side. An echocardiogram was done to look for any congenital heart disease. It reported a mitral valve prolapse syndrome.

The high incidence of renal anomalies in Klippel Feil Syndrome, especially absence of one kidney and congenital heart disease make the assessment of the genitourinary system and the cardiovascular system mandatory in any case of Klippel Feil Syndrome. The avoidance of nephrotoxic drugs and advice regarding urinary tract infection go a long way in protecting the solitary kidney. We have presented the case because of the rare occurrence of a pelvic pancake kidney and a large omovertebral bone in this patient with Klippel Feil Syndrome who had sustained a Lisfranc's fracture dislocation, which also happens to be an orthopaedic rarity.[6]

 
   References Top

1.Hadristy Hishikawn T, Suzuke A, Yasin N: Dept of Surgical Neurology, Research Institute for Brain and Blood vessels. Akila No Shinkai Geka 1991 (Febl 19 (2) 191-5 (A case of Klippel Feil Syndrome with crossed renal ectopia with fusion and unilateral artery occlusion.)  Back to cited text no. 1    
2.Bernard TN Jr, Burke SW, Johnston CE, Roberts JM. Orthopedics 1985 June: 8 (6) 777-83 Congenital Spinal Deformities - A review of 47 cases.  Back to cited text no. 2    
3.Grise P, Cefort J, Dewald M, Metrofanoff P, Bonde J. Ann Urol (Paris) 1982 Jun-July; 18 (4): 232-5: Urinary malformations in synostosis of cervical vertebrae (Klippel Feil Syndrome).  Back to cited text no. 3    
4.Birth defects Org Artic Ser 1977; 13 (3D); Embryonic Pathogenesis of renal agenesis associated with cervical vertebral anomalies (Klippel Feil Pheno type)  Back to cited text no. 4    
5.J. Urol 1976 Jul; 116 (1): 103-4 Crossed fusion of renal pelves and Klippel Feil Syndrome. Gehring GG, Shinasky JH 2d.  Back to cited text no. 5    
6.J Bone Joint Surg (Am) 1974 Sep; 56 (6): 1246-53 Klippel Feil Syndrome; a constellation of associated anomalies. Mensinger RN, Laig JE, Macerea GD.  Back to cited text no. 6    

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Correspondence Address:
K S Kumar
9th Officer Colony, Pink Avenue, Mogapair, Chennai 50
India
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Source of Support: None, Conflict of Interest: None


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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]

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