Characteristic Radiological Appearance Research Articles

Esophageal atresia: Primary repair of a rare long gap variant involving distal pouch mobilization

This report describes a rare type IIIb variant of long gap pure esophageal atresia treated with delayed primary repair. Preoperative imaging showed a distal pouch with a unusually long tapering end, and a 5-vertebral body gap. Delayed exploration with mobilization of the distal pouch allowed for successful primary repair without the need of myotomy. The characteristic radiological appearance of this rare variant enables its preoperative recognition and warrants attempting primary repair with a good expectation of success.

MESENCHYMAL CHONDROSARCOMA AND SIMULATING LESIONS OF THE ORBIT

Although an extremely rare neoplasm, orbital mesenchymal chondrosarcoma has a characteristic radiologic appearance. Suspicion of this lesion preoperatively can be of great value to the ophthalmologist because it tends to produce profuse bleeding at biopsy. This article reviews the clinical presentation, pathologic features, and radiologic findings of patients with orbital mesenchymal chondrosarcoma.

Unilateral Tubular Obstruction: An Unusual Complication of Urographic Contrast Media

OBJECTIVE. Our purpose was to report our experience with four patients with renal colic who had a rare unilateral complication from urographic contrast media, with a dense and striated nephrogram affecting only the healthy kidney. All cases were reversible. CONCLUSION. An unusual complication of urography with a characteristic radiologic appearance is presented. This complication was transient and reversible in our patients. Unilateral tubular blockage induced by a contrast agent must be considered when the contralateral kidney shows evidence of ureteral obstruction. Editorial Comment: The authors describe 4 patients who had a dense, unilateral, striated nephrogram affecting a normal healthy kidney with contralateral ureteral obstruction. This complication was reversible and transient. They attributed this appearance to protein or uric acid precipitation in the renal tubules. Thus, among the patterns of unilateral striated nephrogram that has been reported, this new entity needs to be considered. Other entities that present such a pattern and that may be unilateral or bilateral include obstructed uropathy, acute pyelonephritis, acute thrombosis of the renal vein and so forth. In 1 patient proteinuria was observed, while the other 3 had hyperuricemia with urates in the urinary sediment after excretory urography. I have not seen this phenomenon but it is important to be aware of and include in the differential diagnosis.

Ophthalmic manifestations of fibrous dysplasia: A disease of children and adults

ObjectiveTo determine whether adults constitute a significant proportion of patients presenting with ophthalmic complications of fibrous dysplasia. DesignA retrospective case series. ParticipantsTwenty patients with biopsy-proven fibrous dysplasia participated. Main outcome measuresPatient demographics, major signs and symptoms, occurrence of visual loss, pattern of bone involvement, and radiographic appearance of fibrous dysplasia were studied. ResultsIn contrast to observations made in the past, the authors found that adults constitute a significant proportion of patients suffering from the ophthalmic complications of fibrous dysplasia. Of 20 patients, 9 were younger than 18 years of age (children) and 11 were 18 years of age or older (adults) at the time of presentation. Changes in facial contour and symmetry were the most common presenting signs and symptoms. Five patients, four of whom were adults, presented because of acute visual loss. Most patients had monostotic lesions that crossed suture lines to involve multiple craniofacial bones. On computed tomographic scan, most fibrous dysplasia lesions had a characteristic, pagetoid appearance, with alternating areas of radiolucency and radiodensity. ConclusionsBecause fibrous dysplasia of the orbital bones can be a cause of significant dysfunction and disfigurement, as well as a treatable cause of blindness in both children and adults, the diagnosis of fibrous dysplasia should not be ruled out based solely on the age of the patient. The characteristic radiologic appearance of this disease allows one to differentiate fibrous dysplasia from other tumors associated with bony expansion or density changes, specifically meningioma.

Cortical malformations: a significant cause of paediatric neurological morbidity.

Disorders of cortical development are a significant cause of epilepsy, developmental delay and congenital neurological deficits in children of all ages. Disruption of neuronal proliferation, differentiation, migration and organization during the middle trimester are recognized as the basis of most of these disorders. With advances in magnetic resonance imaging and increasing recognition of the characteristic radiological appearance of these lesions, a variety of lesions are now described. Furthermore, detailed clinical and genetic studies have lead to the recognition of clinicopathological syndromes of cortical maldevelopment with specific modes of inheritance. Our understanding of these disorders is increasing rapidly and will continue to do so with improvements in neuroimaging, advances in developmental neurobiology and heightened clinician awareness.

Endoscopic evaluation and management of the lacrimal sump syndrome.

Failed dacryocystorhinostomy caused by the presence of residual lacrimal sac has been termed the "lacrimal sump syndrome." Epiphora occurs when tears collect in this residual pouch and lacrimal drainage is delayed. Irrigation into the nose may seem normal. This condition can be easily diagnosed with nasal endoscopy and has a characteristic radiologic appearance. Six patients with failed dacryocystorhinostomy caused by lacrimal sump syndrome were successfully treated with an endoscopic contact Nd:YAG laser technique. Endoscopic dacryocystorhinostomy using the contact Nd:YAG laser is an effective method for treating patients who have persistent epiphora or dacryocystitis after failed dacryocystorhinostomy.

Unilateral tubular obstruction: an unusual complication of urographic contrast media.

Our purpose was to report our experience with four patients with renal colic who had a rare unilateral complication from urographic contrast media, with a dense and striated nephrogram affecting only the healthy kidney. All cases were reversible. An unusual complication of urography with a characteristic radiologic appearance is presented. This complication was transient and reversible in our patients. Unilateral tubular blockage induced by a contrast agent must be considered when the contralateral kidney shows evidence of ureteral obstruction.

Intraductal papillary-mucinous neoplasm of the pancreas: a report of five cases with immunohistochemical findings

Five cases of intraductal papillary-mucinous neoplasm (IPMN) of the pancreas are here described. This group of tumors represents a distinct clinicopathological entity with characteristic endoscopic, radiological, gross and microscopic appearances. Our five cases all showed marked dilatation of the main pancreatic duct which contained mucin and papillary tumor, sometimes filling and sometimes lining the lumen. The papillae were lined by columnar epithelium showing varying degrees of dysplasia. In one case there was adjacent invasive adenocarcinoma. Immunohistochemical staining for p53 protein was strongly positive in a majority of cells in the three cases with severe epithelial dysplasia and in the invasive adenocarcinoma, while weaker staining in a minority of cells was seen in the remaining two cases. A small proportion of tumor cells expressed PCNA and Ki-67 immunohistochemically in four cases, while the case with severe dysplasia and invasive carcinoma showed positivity for these proliferation markers in most cells. The four cases of exclusively intraductal tumor showed no evidence of disease recurrence at follow-up (median 55 months). The patient with IPMN and associated invasive adenocarcinoma died 21 months post-operatively. In view of its relatively favourable prognosis, it is important that IPMN is recognised.

Intraarticular osteochondroma of the elbow

Osteochondroma is the most common benign bone tumor. The long tubular bones are commonly affected, including the proximal humerus and distal radius. A solitary lesion about the elbow is rare, and from review of the literature, no author has ever reported an intraarticular location as a site of an osteochondroma in the distal humerus:5. 8 We report the case of an intraarticular osteochondroma of the elbow without characteristic radiological appearance that caused progressive elbow ankylosis. (J SHOULDER ELBOW SURG 7994;3:270-2)

Case report 821

Parosteal lipoma with bony excrescences at the periosteal base of the tumor is rare. Only one case has been reported with osseous and cartilaginous nodules throughout the lipoma. We describe an additional case in a 39-year-old man. We emphasize the characteristic radiological appearance of the lesion as well as the possibility of a dual histogenesis for the osteochondromatous component. We also stress the great value of CT in establishing the diagnosis.

Failed Dacryocystorhinostomy: The Sump Syndrome

ABSTRACT A dacryocystorhinostomy may fail due to a problem along the canaliculus, at the ostomy site of nasolacrimal sac to nose, due to an intranasal problem or related to the stent. The "sump syndrome" occurs when a residual nasolacrimal sac forms, collects fluids, and leads to tearing. This entity, although uncommon, has a characteristic clinical history and radiologic appearance.

Primary sclerosing cholangitis

Primary sclerosing cholangitis is a condition of unknown cause. It is recognized by liver dysfunction and its characteristic radiologic appearance, which is related to portal tract inflammation, bile duct proliferation, and periductal fibroses involving small intrahepatic and large extrahepatic ducts. The disease lasts about 10 years from the time of diagnosis. Primary sclerosing cholangitis is recognized by abnormal results on routine liver function tests or by the development of clinical jaundice. An autoimmune cause has been suggested because of its strong association with inflammatory bowel disease, certain antigens, AIDS, and immunoregulatory abnormalities. Results of medical management of sclerosing cholangitis have been disappointing. Immunosuppressive drugs, copper chelating agents, and antibiotics have failed to alter progression of the disease. Colectomy in patients with inflammatory bowel disease also has no influence. The judicious use of dilations of strictures, bypass procedures, or resection can palliate jaundice in patients with primary sclerosing cholangitis, but liver transplantation is the definitive treatment. Because palliative operations increase the hazards of liver transplantation, percutaneous dilations and stentings are preferred initially. Cirrhosis and portal hypertension are indications for transplantation. In the future, transplantation may be indicated earlier in the course of the disease.

Urinary tract calculi that form on surgical staples: a characteristic radiologic appearance.

An autostapler is frequently used when bowel is interposed in the urinary tract during reconstructive urologic surgery in children. Usually the metallic staples are buried beneath the intestinal mucosa. However, if a portion of a staple is unintentionally left exposed or becomes exposed through migration and is then bathed by urine, it can become the nidus for formation of a calculus. The radiologic studies of 30 patients having surgery of this type in our hospital from 1980 to 1990 were reviewed prospectively, and stones were found to have developed on staples in eight patients. None of the patients had metabolic abnormalities. The stones were easily visible on plain radiographs because they were calcified. They formed in the lumen of the bladder or the bowel segment on the exposed portion of the staple. Thus, the staple was eccentric in the calculus and not central, as is usually the case with a stone forming on a foreign body. This experience suggests that stones forming on surgical staples during reconstructive urologic surgery have a characteristic radiologic appearance that may aid in their identification.

Juxta‐epiphyseal pathological fracture of the proximal femur secondary to metaphyseal chondrodysplasia

Abstract Metaphyseal chondrodysplasia is a rare disorder of which there are five types: the Jansen type, Schmid type, McKusick type, metaphyseal chondrodysplasia and thymolymphopenia, and metaphyseal chondrodysplasia with malabsorption and neutropenia also known as Shwachman's syndrome. All of the bony changes associated with these disorders are considered to be due to a primary defect in the metaphyseal portion of the growth plate. Accurate diagnosis is of importance as the members of the above‐mentioned pentad have varying genetic implications as well as widely varying prognoses. We report a case of juxta‐epiphyseal pathological fracture of the proximal femur secondary to Shwachman's syndrome. The characteristic radiological appearance and management of this complication have not been described. It is important that appropriate management be instituted in this condition because patients with Shwachman's syndrome carry a good prognosis both for length and quality of life.

Giant Epidermoid Cysts of the Skull

The authors describe two cases of giant intradiploic epidermoid cysts of the cranial vault and a case of a large epidermoid arising from the frontal sinus and extending intracranially. The aetiology of epithelial cysts is reviewed. The characteristic radiological and CT scan appearances of diploic epidermoid cysts are described, their clinical presentation and surgical management briefly discussed.

The radiological appearances of telangiectatic osteosarcoma. A study of 14 cases.

Fourteen telangiectatic osteosarcomas are reported. They are rare, clinically and radiologically aggressive lesions, involving mainly the femurs of young patients, often misdiagnosed as aneurysmal bone cysts. An explanation for a characteristic early radiological appearance consisting of regular parallel striations of the shaft is suggested.

Unstable Thoracolumbar Fractures

Two comparable groups with unstable thoracolumbar fractures have been studied: one consisting of 23 patients treated conservatively and one consisting of 20 patients treated surgically with Harrington instrumentation. The vertebral injuries have been analysed radiologically at the time of injury as well as after completion of conservative and surgical treatment and at follow-up on average 6 years and 3 months after injury in the conservatively treated group and 2 years and 2 months after injury in the operated group. The vertebral fractures were often solitary at the L1 level with a characteristic radiologic appearance and mainly caused by flexion-rotation injuries. There were indirect signs of associated ligament injuries in most cases. Indirect signs of associated disc injury were found in 77 per cent of the cases, most of them related to the superior disc. The signs of disc injury were accentuated at the two follow-up examinations. In the operated group, the radiologic appearance of the vertebral body normalised after Harrington instrumentation. A preoperative gibbus angle of 19.4 degrees was reduced to 6.8 degrees on average postoperatively. In 13 of the operated cases the rods were removed. Among these patients the gibbus angle increased to 17.6 degrees at the final follow-up examination, compared with 11.9 degrees in the patients who had their rods retained. This difference in gibbus angle was significant. In the conservatively treated group, the initial gibbus angle of 19.5 degrees continuously increased to 27.6 degrees at the follow-up examination. All vertebral fractures had healed at the follow-up examination.

Periosteal chondroma

The periosteal chondroma (juxtacortical chondroma) is an unusual tumor which usually occurs on the surface of tubular bones in the metaphyseal area. In this study, we reviewed the clinicopathologic features of 22 patients representing 23 instances of periosteal chondroma and discuss the radiologic and histologic features necessary for accurate diagnosis. The characteristic radiologic appearance is of a single cartilaginous mass in the metaphyseal periosteum causing well-defined depression or "saucerization" of the adjacent cortex. The radiologic differential diagnoses include soft-tissue tumors compressing bone, fibrous cortical defect, and periosteal chondrosarcoma or osteosarcoma. Histologic features include lobules of hyaline cartilage with frequent areas of hypercellularity, binucleate chondrocytes, and focal mild cytologic atypia. The histologic features clearly identify the tumor as chondrogenic; however, familiarity with the x-rays may be necessary to recognize the tumor as benign.

Pigmented Villonodular Synovitis of the Temporomandibular Joint

A 39-year-old woman had a large asymptomatic left parotid mass that she had apparently not noticed. The clinical appearance suggested a parotid tumor. Aspirated tissue revealed numerous giant cells, histiocytes, and hemosiderin pigment. At surgical exploration a tumor was found deep to the facial nerve involving the temporomandibular joint, which had a brown-stained roughened synovial membrane. The resected specimen histologically was a proliferative lesion composed of epithelioid histiocytes, spindle cells, and multinucleated giant cells. The appearance was typical of the family of lesions that includes pigmented villonodular synovitis, bursitis, and tenosynovitis (giant cell "tumor" of tendon sheath). In view of the origin from the temporomandibular joint, reinforced by a characteristic radiologic appearance, we interpret this as a case of pigmented villonodular synovitis. This is the fifth case reported from this site.

The Morbid Anatomy of the Skeleton in Osteogenesis Imperfecta

Clinically, the two most prominent features of osteogenesis imperfecta are dwarfing and bone fragility. Radiologic examination reveals severe osteopenia with or without multiple deformities. Histological examination of samples obtained from 16 iliac crest biopsies compared with 21 age-matched controls revealed a significantly diminished amount of bone matrix without an associated decrease in the number of bone cells, suggesting a quantitative deficiency of collagen production. Radiologic studies of 46 affected children and autopsy studies of two children revealed abnormalities in the ossification of the secondary centers of ossification, together with evidence of fracture and fragmentation of the epiphyseal growth plates causing a characteristic radiologic appearance which may be likened to a bag of popcorn. These epiphyseal abnormalities may contribute to the limb shortening in some cases.