Joanna J. Seibert

Cranial Ultrasound Lesions in the NICU Predict Cerebral Palsy at Age 2 Years in Children Born at Extremely Low Gestational Age

Our prospective cohort study of extremely low gestational age newborns evaluated the association of neonatal head ultrasound abnormalities with cerebral palsy at age 2 years. Cranial ultrasounds in 1053 infants were read with respect to intraventricular hemorrhage, ventriculomegaly, and echolucency, by multiple sonologists. Standardized neurological examinations classified cerebral palsy, and functional impairment was assessed. Forty-four percent with ventriculomegaly and 52% with echolucency developed cerebral palsy. Compared with no ultrasound abnormalities, children with echolucency were 24 times more likely to have quadriparesis and 29 times more likely to have hemiparesis. Children with ventriculomegaly were 17 times more likely to have quadriparesis or hemiparesis. Forty-three percent of children with cerebral palsy had normal head ultrasound. Focal white matter damage (echolucency) and diffuse damage (late ventriculomegaly) are associated with a high probability of cerebral palsy, especially quadriparesis. Nearly half the cerebral palsy identified at 2 years is not preceded by a neonatal brain ultrasound abnormality.

Transcranial Doppler, MRA, and MRI as a screening examination for cerebrovascular disease in patients with sickle cell anemia: an 8-year study

Objective. The authors previously reported five transcranial Doppler ultrasonography (TCD) findings as significant in detecting clinical cerebrovascular disease in a 4-year study in patients with sickle cell disease. This is a follow-up to evaluate the validity of the original findings over another 4-year period during which the study population doubled. A clinical follow-up of the original asymptomatic sickle cell patients with positive TCD, MRA, and MRI was also made. Materials and methods. Over an 8-year period TCD, MRI, and MRA were prospectively performed in 90 sickle cell patients who were clinically asymptomatic for stroke and in 27 sickle cell patients with clinical stroke. Results. Of the 4 out of original 46 control patients in 1992 who had positive MRA and TCD, 3 have subsequently had clinical stroke. None of the 9 original patients with positive TCD and positive MRI but negative MRA have developed stroke. All five original TCD indicators of disease were still significant (P < 0.05) for detecting clinical disease: maximum velocity in ophthalmic artery (OA) > 35 cm/s, mean velocity in middle cerebral artery (MCA) > 170 cm/s, resistive index (RI) in OA < 50, velocity in OA greater than in MCA, and velocity in posterior cerebral (PCA), vertebral, or basilar arteries greater than in MCA. Four additional factors were also significant: turbulence, PCA or ACA without MCA, RI < 30, and maximum velocity in MCA > 200 cm/s. Conclusion. Positive MRA with a positive TCD in an asymptomatic patient in long-term follow-up suggests a trend for developing clinical stroke. A 4- to 8-year follow-up of nine patients with positive TCD, positive MRI, but not positive MRA did not show development of clinical stroke. Nine Doppler findings are significant in screening for clinically symptomatic vascular disease in sickle cell patients. It is recommended that children with sickle cell disease be screened for cerebrovascular disease with TCD. If one or two indicators of abnormality are present, MRA is recommended. If the MRA is positive, the patient may be considered for transfusion therapy or other treatment for prevention of stroke.

Transcranial Doppler (TCD) screening for stroke prevention in sickle cell anemia: pitfalls in technique variation

Background. The Stroke Prevention Trial in Sickle Cell Anemia (STOP) identified children as being at high stroke risk if the time-averaged maximum mean velocity (TAMMV) of the middle cerebral or intracranial internal carotid arteries measured ≥ 200 cm/s. These values were obtained utilizing a 2-mHz dedicated nonimaging pulsed Doppler technique (TCD) and manual measurements. Questions have been raised as to the comparability of results obtained with different ultrasound machines and measurement techniques.¶Objective. The purpose of this study was to compare nonimaging (TCD) and transcranial duplex imaging (TCDI) findings in children potentially at risk for stroke with sickle cell disease.¶Materials and methods. Twenty-two children with sickle cell disease and no history of stroke were evaluated by both TCD and TCDI. Examinations were performed on the same day without knowledge of the other modality results and read independently using manually obtained measurements. Mean velocities, peak systolic velocities, and end diastolic velocities obtained by the two techniques were compared. In a subgroup, manual measurements were compared to electronically obtained measurements. ¶Results. TCDI values were lower than TCD measurements for all vessels. TCDI TAMMV values were most similar to the TCD values in the middle cerebral artery (–9.0 %) and distal internal cerebral artery (–10.8 %), with greater variability in the anterior cerebral artery (–19.3 %), bifurcation (–16.3 %), and basilar arteries (–23.1 %). Risk group placement based on middle cerebral artery TAMMV values did not change when comparing the two techniques. Measurements obtained electronically were lower than those obtained manually. ¶Conclusion. Velocities obtained by TCDI may be lower than TCD measurements, and these differences should be taken into consideration when performing screening for stroke risk and selection for prophylactic transfusion based on the STOP protocol.

Observer variability assessing US scans of the preterm brain: the ELGAN study

BackgroundNeurosonography can assist clinicians and can provide researchers with documentation of brain lesions. Unfortunately, we know little about the reliability of sonographically derived diagnoses.ObjectiveWe sought to evaluate observer variability among experienced neurosonologists.Materials and methodsWe collected all protocol US scans of 1,450 infants born before the 28th postmenstrual week. Each set of scans was read by two independent sonologists for the presence of intraventricular hemorrhage (IVH) and moderate/severe ventriculomegaly, as well as hyperechoic and hypoechoic lesions in the cerebral white matter. Scans read discordantly for any of these four characteristics were sent to a tie-breaking third sonologist.ResultsVentriculomegaly, hypoechoic lesions and IVH had similar rates of positive agreement (68–76%), negative agreement (92–97%), and kappa values (0.62 to 0.68). Hyperechoic lesions, however, had considerably lower values of positive agreement (48%), negative agreement (84%), and kappa (0.32). No sonologist identified all abnormalities more or less often than his/her peers. Approximately 40% of the time, the tie-breaking reader agreed with the reader who identified IVH, ventriculomegaly, or a hypoechoic lesion in the white matter. Only about 25% of the time did the third party agree with the reader who reported a white matter hyperechoic lesion.ConclusionObtaining concordance seems to be an acceptable way to assure reasonably high-quality of images needed for clinical research.

The ultrasonographic diagnosis of typhlitis (neutropenic colitis).

Typhlitis is a necrotizing inflammatory disease of the cecum, usually with secondary infection. It is most often found in acute leukemia patients on chemotherapy but has also been reported in other patients on chemotherapeutic drugs. Diagnostic features of typhlitis have been reported on plain radiographs, barium enema, angiography, CT, and one other reported case with ultrasound. We report three cases of typhlitis with a characteristic echogenic thickening of the mucosa on ultrasound. The sonographic findings in the one previous report were identical to those of our three cases. We believe that the sonographic findings of typhlitis are unique and that ultrasound offers an easy noninvasive method of diagnosing this potentially lethal disease.

Efficiency of the bone scan for occult limping toddlers.

Fifty consecutive occult limping toddlers were prospectively evaluated by acute triphase 99mTc MDP scintigraphy (TTS) at Arkansas Childrens Hospital from 1984 through 1989. Only patients with a limp that could not be diagnosed by an orthopaedist were included. TTS proved essential in localizing the lesion in 27 patients (54%). With only two false negatives and one false positive, this test was shown to be highly sensitive, specific, efficient, and predictive, especially as compared with temperature, white blood cell (WBC) count, erythrocyte sedimentation rate (ESR), and plain radiography. Because no infections were missed by TTS, patients with a normal TTS could be safely observed as outpatients, saving thousands of health care dollars in this small series.

Use of ultrasound in the detection of occult bowel perforation in neonates.

Intestinal perforation is a common and serious complication of NEC in neonates. Plain film evidence often is absent in patients with surgically proved perforation. Some patients with NEC develop a distended, gasless abdomen suitable for sonographic evaluation. Five neonates with gasless abdominal distention and clinical decline were studied with ultrasonography. Four patients demonstrated intraperitoneal fluid‐debris levels and ascites; all had surgical proof of perforation. The fifth patient did not demonstrate these findings and subsequently recovered. The sonographic findings of ascites and intraperitoneal fluid‐debris levels in patients with NEC are suggestive of perforation.

High resolution ultrasound with Doppler: A diagnostic adjunct in orbital and ocular lesions in children

Twenty-six infants and children with orbital and ocular pathology were examined with ultrasound (US) utilizing real-time imaging and Duplex Pulsed Doppler evaluation. Twenty-two of these patients underwent concurrent orbital computed tomography (CT) and two had magnetic resonance imaging (MRI). Orbital and periorbital lesion included hemangioma, dermoid, lymphangioma, rhabdomyosarcoma, encephalocoele and abscess. Ocular lesions included infection, trauma, retinal detachment, retinoblastoma, Coats disease, and persistent hyperplastic primary vitreous. High resolution US with Doppler provided unique diagnostic information in patients with penetrating ocular trauma, orbital and periorbital masses, and intraocular structural abnormalities. High resolution US examination of the eye and periorbital tissues is readily performed using widely available equipment and often delineates subtle structural abnormalities not shown by CT or MRI.

Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome: Case Report and Review of the Literature

We report a case of the megacystis-microcolon-intestinal hypoperistalsis syndrome and review the literature. Generally, this disease affects female neonates and is usually fatal within the first year of life. All patients have microcolon megacystis, intestinal hypoperistalsis, abdominal distention and dilated small bowel. Although the dilated bowel and urinary tract suggest an obstruction, such is not usually the case and surgical intervention is not always necessary. The abdominal distension results primarily from distension of the urinary tract.

High resolution ultrasound characterization of soft tissue masses in children

Forty-two soft tissue masses in infants and children were examined with high resolution ultrasonography. Sonography was diagnostically specific in 17/42 (40%), useful but not diagnostic in 24/42 (58%), and misleading in 1/42 (2%) of soft tissue masses. Lesions with diagnostic sonographic features included cystic hygroma, fibromatosis colli, lymphadenopathy with abscess formation, and one case of osteomyelitis.