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SELF ASSESSMENT ANSWERS Congenital Renal Anomaly in A Postgrad Med J 2003;79:359–362 359 to 10 years. The mode of spread of intramed- Postgrad Med J: first published as 10.1136/pmj.79.932.359 on 1 June 2003. Downloaded from SELF ASSESSMENT ANSWERS ullary cysticercosis is either haematogenous or ventriculoependymal.2 MRI studies help in diagnosing and correctly correlating the Congenital renal anomaly number of cases the band of renal tissue may 4 pathological diagnosis of neurocysticercosis evade ultrasonic detection. Computed tom- 3 in a patient with situs ography may be necessary to confirm the (including intramedullary cysticercosis). inversus diagnosis. Treatment modalities like drug therapy Intervention is required because of obstruc- (cysticidal drug)/surgery, or both, can be Q1: Name the congenital renal tion or calculi. The combination of horseshoe planned according to the pathological stage kidney with an aortic aneurysm presents a and location of the cyst as seen on MRI. Since anomaly identified in fig 1 (see p 355) the cysticidal drugs albendazole and Horseshoe kidney. The lower poles of the kid- diagnostic and therapeutic challenge to the vascular surgeon.5 praziquental were shown to be effective in ney being displaced towards the midline, parenchymal brain cysticercosis, these drugs joined by either functioning renal tissue or a Final diagnosis have been considered potentially useful in fibrous band. patients with intramedullary cysticercosis. Horseshoe kidney with situs inversus. Successful management of intramedullary Q2: What is the incidence and sex cysticercosis by cysticidal drugs alone has also ratio of this anomaly? References been reported in the literature.4 Incidence is one in 400. It is more commonly 1 Bauer SB, Perlmutter AD, Retik AB. Anomalies In the present case when the diagnosis of found in males at a ratio of 2:1. of the upper urinary tract. Campbell’s urology. intramedullary cysticercosis was established 6th Ed. Philadelphia: WB Saunders, 1992: 1376–81. on MRI, surgery was undertaken due to its Q3: What complications occur with 2 Matsusshita K, Veda S, Kegami K. location in cervical segment, and this was fol- this condition? Horseshoe kidney in a patient with situs lowed by albendazole therapy (15 mg/kg × 28 Thirty percent of cases are asymptomatic and inversus totalis. J Urol 1982;128:604–5. days). The patient showed complete neuro- are identified incidentally. Stasis of urine due 3 Krishnan B, Troung LD, Saleh G, et al. logical improvement with resolution of the to the malrotation of the kidneys, and Horseshoe kidney is associated with an intramedullary lesion. increases relative risk of primary renal It is concluded that with present generation impaired ureteric drainage result in infection 157 – carcinoid tumor. J Urol 1997; :2059 66. MRI and also successful surgical/drug man- and stone formation. 4 Banerjee B, Brett I. Ultrasound diagnosis of horseshoe kidney. Br J Radiol agement, the outcome of intramedullary cyst- Q4: Name three other genitourinary 1991;64:898–900. icercosis is not as dismal as was reported ear- anomalies that can be associated with 5 Stroosma OB, Kootstra G, Schurink GWH. lier, and patients with paraplegia also have a Management of aortic aneurysm in the favourable outcome. this condition presence of a horseshoe kidney. Br J Surg These are: (1) hypospadiasis; (2) undescended 2001;88:500–9. Final diagnosis testis; (3) ureteral duplication. Vaginal septa- Spinal (cervical) intramedullary cysticercosis. tion and bicornuate uterus can also be associ- ated with this condition. A man with numbness and References 1 Corral I, Quereda C, Moreno A, et al. Discussion limb weakness Intramedullary cysticercosis cured with drug Horseshoe kidney was first recognised during treatment. A case report. Spine a necropsy by DeCarpi in 1521, but Botallo in Q1: What is the diagnosis? 1996;21:2284–7. 1564 provided the first description and illus- The diagnosis is spinal (cervical) intramedul- 2 Mathuriya SN, Khosla VK, Vasishta RK, et al. tration of a horseshoe kidney.1 Horseshoe kid- lary cysticercosis. The MRI scan (fig 1; see Intramedullary cysticercosis: MRI diagnosis. Neurology India 2001;49:72–4. neys are believed to result from the median p 355) shows a cyst located in the intramedul- lary region. Cervical laminectomy with re- 3 Mohanty A, Venkatrama SK, Das S, et al. fusion of metanephric tissue due to mechani- Spinal intramedullary cysticercosis. cal forces. However studies have suggested moval of the cyst was done. Histopathology Neurosurgery 1997;40:82–7. http://pmj.bmj.com/ that abnormal fusion of tissue associated with examination (fig 2; see p 355) proved the 4 Sotelo J, Guerreo V, Rubio F. the parenchymatous isthmus of horseshoe lesion to be a cysticercus cyst with scolex (lar- Neurocysticercosis. A new classification kidney is the result of a teratogenic event val cyst). based on active and inactive forms—a study involving the abnormal migration of posterior of 753 cases. Arch Intern Med 145 – nephrogenic cells. Q2: What are the treatment options? 1985; :442 5. In most cases the kidneys are linked at the The treatment of spinal intramedullary cyst- lower poles by a parenchymatous or fibrous icercosis could be surgical, medical (that is, An interesting case of isthmus that crosses the midline of the body. cysticidal therapy) or both, based on location In general isthmus lies anterior to aorta and and stage of the cyst as also on the experience hemiparesis on September 26, 2021 by guest. Protected copyright. vena cava. Because kidneys fail to rotate, the of the physician. Surgical treatment includes calyces point posteriorly. The ureter inserts laminectomy with removal of the cyst. Cysti- Q1: What is the differential diagnosis higher on the renal pelvis and lies laterally cidal drugs given are albendazole in a dose of in this patient? and crosses over and anterior to isthmus. The 15 mg/kg body weight for 14–30 days or The differential diagnosis of HIV patients pre- blood supply can be quite variable.1 praziquantel 50 mg/kg body weight for 15 senting with focal neurological deficits should The horseshoe kidney is frequently found in days along with steroids to reduce the perile- include disorders such as toxoplasmosis, other congenital anomalies, some of which sional oedema and to prevent neurological primary central nervous system lymphoma, are incompatible with long term survival. deterioration during the course of cysticidal cerebral Chagas’ disease, progressive multifo- Most common congenital anomalies involved drugs. Administration of cysticidal drugs cal leucoencephalopathy (PML), central nerv- include skeletal, cardiovascular, and central before or after surgery is a point of personal ous system tuberculosis, and cryptococcosis.1 nervous systems. There is increased occur- preference for the individual doctor as no sys- rence of other genitourinary anomalies. Fe- tematic evaluation has been possible due to Q2: What are the computed males with Turner’s syndrome have a high the rarity of the disease. tomography and MRI findings? incidence of horseshoe kidney. Horseshoe Computed tomography of the head (fig 1; see kidney with situs inversus is a rare and inter- Discussion p 356) shows well defined hypodense areas in esting association.2 Intramedullary cysticercosis is a rare manifes- the white matter in bilateral posterior parietal One third of all patients remain asympto- tation of neurocysticercosis,1 and fewer than regions and in the right temporoparieto- matic. Others present with vague abdominal 50 cases have been reported. The cysts are frontal region without areas of enhancement pain resulting from hydronephrosis, infection, commonly located in spinal subarachnoid or mass effect. Figure 2A (see p 356) shows or calculus formation. Horseshoe kidney is space and rarely at intramedullary locations. the gadolinium enhanced T1 sagittal MRI of associated with an increased relative risk of The majority of reported cases have cysts in the brain showing non-enhancing white mat- Wilms’ tumour, transitional cell carcinoma, the dorsal cord, which is in accordance with ter changes. Figure 2B (see p 356) shows the and renal carcinoid.3 the regional blood flow to the spinal cord. In T2 weighted coronal MRI showing white mat- Ultrasound or an excretory urogram readily 90% of reported cases of intramedullary cyst- ter changes without mass effect. The findings makes the diagnosis. Ultrasound diagnosis icercosis due to neurocysticercosis the pa- of non-enhancing white matter lesions with depends on the demonstration of an isthmus tients were between 20 and 45 years of age. typical increased T2 and decreased T1 signals or band of renal tissue across the midline. In a The duration of symptoms varied from a week on MRI head are highly suggestive of PML.2 www.postgradmedj.com 360 Self assessment answers 3 Q3: How is the diagnosis confirmed? to make the clinical diagnosis of PML. the limb in the abducted positions (fig 3; see p Postgrad Med J: first published as 10.1136/pmj.79.932.359 on 1 June 2003. Downloaded from Though the definitive diagnosis of PML Recently polymerase chain reaction amplifi- XXX) virtual occlusion of the subclavian depends on identification of characteristic cation of JC virus DNA from the cerebrospinal artery can be seen. Venography demonstrated neuropathological abnormalities on brain fluid has become the favoured diagnostic similar positional compression of the subcla- biopsy, it is not necessary to confirm the modality to confirm the diagnosis.4 vian vein. The vascular compression accounts diagnosis.3 Neuroimaging is most helpful in
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