Biomedical subjects
Publications and source records attributed to E M Azouz.
Clinical assessment of the sacroiliac joints is difficult. Conventional radiography and radionuclide scanning have not afforded optimal sensitivity and specificity in the diagnosis of sacroiliac involvement in spondyloarthropathies. This report describes a technique for computed tomography of the sacroiliac joints in children; the method involves coronal scanning and allows assessment of the entire surface of the joints.
We report on a son and daughter of Ashkenazi-Jewish parents with postaxial polydactyly of the hands and feet associated with syndactyly and brachydactyly, mental retardation, cerebellar hypoplasia, pectus excavatum, mesomelic shortness of the upper and lower limbs, and pretibial dimples. Although this appears to be an example of one of the OFD syndromes and has many similarities to OFD type II, III, IV and VI, it does not fit satisfactorily into any of the types previously described. Thus this may be a new OFD syndrome, although we cannot exclude a possibility that most or all autosomal recessive OFD syndromes are the result of pleiotropy of a single mutation in a homozygous state.
A girl who presented at 3 months of life with severe developmental delay, blindness, and hearing impairment was found to have intracerebral calcifications. Skeletal films showed craniotubular bone modeling consistent with dysosteosclerosis. The nosology of this disorder is discussed.
We present a 3-generation family, ascertained after the birth of a child with cleidocranial dysplasia (CCD). The propositus presented with respiratory distress (due to a narrow thorax) and hypoplasia and discontinuity of both clavicles. The mother, aunt, and grandmother had varied features of the condition. This intrafamilial variation illustrates the need for clinical assessment of family members following the birth of an apparent sporadic case of CCD.
Neuroblastoma is the third most common type of cancer seen in children, after leukemia and tumours of the central nervous system. Although bony metastasis to the skull and the orbits has been well described, metastasis to the mandible is exceptional; 32 cases have been reported. Two more are presented here, along with a short review of the topic emphasizing the radiographic features and the differential diagnosis.
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We report on a boy with platyspondyly and metaphyseal manifestations of enchondromatosis with severe involvement of hands and feet, compatible with generalized enchondromatosis (enchondromatosis Spranger type VI). His father has only moderate platyspondyly. The latter is either coincidental or a milder, more localized expression of the disease. Father and son have consanguineous parents suggesting autosomal recessive inheritance of the trait. However, autosomal dominant inheritance with variable expressivity is also possible.
We have presented the clinical and radiographic findings (plain films and CT study) of a 22-month-old boy with multiple destructive cranial and mandibular lesions of LCH. An unusual feature was the presence of adjacent, relatively large, soft-tissue masses, with numerous small discrete calcifications, possibly related to previous intralesional steroid injections.
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Computed tomography (CT) of the abdomen is an important diagnostic tool for detecting abnormalities of the solid organs and, to a lesser extent, the gastrointestinal tract. For an optimal CT study, the gastrointestinal and urinary tracts should be opacified with oral and intravenous radiopaque contrast agents. The authors tested the effectiveness of dilute oral meglumine and sodium ioxithalamate (Telebrix 38, Mallinckrodt Canada Inc., Pointe-Claire, Que.) as a bowel marker in 50 CT examinations of the abdomen and pelvis. The study objective was assessment of patient acceptance, safety of the product, degree of bowel opacification and image quality. Of children who were capable of answering the question, 93% said they would be willing to take the 2% oral solution of Telebrix again if required. No adverse effect attributable to the contrast medium was noted. Opacification of the gastrointestinal tract was excellent in the majority of cases, and the diagnostic quality of the CT images was good. The authors concluded that 2% Telebrix is an effective and safe bowel opacification agent for abdominal and pelvic CT scanning.
Two children, with Beckwith-Wiedemann syndrome and hemihypertrophy who were followed by ultrasonography, developed a large nephroblastoma in the interval between two abdominal ultrasound examinations. The customarily suggested bi-annual examinations are not frequent enough and may give physicians and parents a false sense of security.
After a short review of our method, we compared the results of the radiographs of 18 cases of CdLS to the results of our normal material. All of our normal statistical data was obtained from AP and lateral view radiographs at a distance of 1 m, in the prevailing conditions of everyday practice in a radiological department. We studied quantitative changes such as microcephaly and brachycephaly, and for the first time we introduced qualitative changes such as occipital rotation and the study of obliquity of the foramen magnum (line BAO). We found microcephaly in all our cases of CdLS and brachycephaly in only 72%. Furthermore, we found a lack of positive occipital rotation in all our cases of CdLS and the line BAO turned posteriorly with O higher than BA. The same conditions were observed on our normal material only in an early postnatal stage.
Medulloblastoma is a relatively common intracranial neoplasm in childhood. Its extraneural spread was, until recently, thought to be a rare occurrence. Metastases are most commonly to bone. Five patients with medulloblastoma metastatic to bone are presented, and findings are compared to those of previous reports. Two of the five cases showed patchy extensive osteosclerosis of the pelvis and/or proximal femora. One case had concurrent lymph node involvement. In patients with past or present medulloblastoma and bone pain, metastases to bone should be excluded. Medulloblastoma metastatic to bone is a rare cause of extensive osteosclerosis.
We have retrospectively reviewed the radiographic and clinical features of 56 cases of pelvic rhabdomyosarcoma seen in three Pediatric Hospitals between 1960 and 1986. There were 35 boys and 21 girls. The study aimed at better defining the role played by the various imaging techniques in the investigation of these tumors. The role of diagnostic radiology is the detection and delineation of the primary tumor, its local spread and distant metastases at the time of diagnosis and on follow-ups. Intravenous urography (IVU) and to a lesser extent barium enema (BE) and cystography (VCUG) were used even after the availability of ultrasonography (US) and computed tomography (CT). These two modalities have only partly replaced the traditional radiographic techniques. All patients seen or followed after 1977 (28 patients) had both US and CT examinations. We have focused our discussion on these relatively new imaging modalities namely US and CT. Magnetic resonance was not utilized in any patient in this series. US was by far the best imaging technique available for lesions of the urinary bladder and those invading the bladder wall in children as it was possible to visualize, measure and follow these tumors. Scrotal US was used to confirm the clinical diagnosis of a paratesticular mass. CT showed to best advantage the tumour and its relationship to pelvic organs, musculature and bones.
Six boys with traumatic ischial apophysis are reported. Two cases were diagnosed as stress apophysis and four as apophyseolysis. Two of our patients were referred to the hospital as malignant bone tumours--Ewing sarcoma and osteosarcoma.
Bismuth subgallate is used occasionally as a hemostatic agent for packing the adenoidal and tonsillar beds after an adenotonsillectomy. Containing a heavy metal, it is relatively insoluble and poorly absorbed. It produces faint opacities on plain radiographs and these may mimic a foreign body or pharyngeal calcification. We report a 13-year-old girl with linear radiopacities in the nasopharynx and in the palatine tonsillar bed. Careful and complete clinical information pinpointed the exact (iatrogenic) cause of these radiodensities.
We report 23 children with soft-tissue tumors of the hand and wrist. Tumors of blood and lymph vessel origin accounted for nine patients; giant-cell tumors of tendon sheaths and tumoral calcinosis occurred in four patients each and soft-tissue chondroma in three. Synovial osteochondromatosis and aggressive fibromatosis were each represented by one patient. Finally, there was one child with a malignant soft-tissue tumor, an embryonal rhabdomyosarcoma of the hand. Hand and wrist ganglia were excluded. Imaging methods used in investigating hand and wrist lesions are discussed but plain radiography remains the first and often only examination necessary.
Deep venous thrombosis is a frequent, well-recognized complication of spinal cord injury. Patients with myelomeningocele often have similar weakness of the lower extremities. Following orthopedic surgery, they may also be immobilized at a time when they are hypercoagulable. In addition, as with patients with spinal cord injury, patients with myelomeningocele are prone to urinary tract infection, which may cause local inflammation in the pelvic veins. For the first time, three patients with myelomeningocele complicated by deep venous thrombosis are described. The differential diagnosis (deep venous thrombosis vs osteomyelitis vs fracture) is also discussed in a child with myelomeningocele and a warm, swollen leg, as are the diagnostic methods available. Finally, the issue of antithrombotic prophylaxis in patients with myelomeningocele who are to undergo extensive orthopedic surgery is discussed.