Multiple squamous hyperplastic-fibrous inflammatory polyps of the oesophagus: a new feature of eosinophilic oesophagitis?

This report describes the unusual case of a 12-year-old boy with multiple polyps in the oesophagus and concurrent eosinophilic oesophagitis (EoE). Polyps were of a fibrous-inflammatory composition featuring eosinophils, mast cells, hyperplastic epithelium and fibrosis, which are all features described with EoE. EoE is an increasingly recognised clinicopathological disorder characterised by large numbers of eosinophils infiltrating the oesophageal mucosa. Polyps in the oesophagus are rare, have not previously been associated with EoE, and may represent a new feature of the disease.

Clinical correlation of radiological spinal stenosis after standardization for vertebral body size.

AIM: To determine the relationship between the degree of radiographic lumbar spinal stenosis, adjusted with an internal control for vertebral body size, and disability from lumbar stenosis. MATERIALS AND METHODS: one hundred and twenty-three consecutive patients with clinical and radiological confirmation of neural impingement secondary to lumbar stenosis were enrolled prospectively. Thecal sac anteroposterior (AP) diameter (TSD) and cross-sectional area (CSA), and vertebral body AP dimension (VBD) were determined. These parameters were then correlated with patients' symptoms using the modified Roland-Morris questionnaire (RMQ) disability score. RESULTS: No statistically significant inverse correlation was found between the TSD and RMQ score (p=0.433), between the CSA and RMQ score (p=0.124), or between the TSD:VBD ratio and RMQ score (p=0.109). There was a significant positive correlation between the CSA:VBD ratio and RMQ score (p=.036), and therefore, there was no statistical support for an inverse relationship between the two. There was a significant difference in mean RMQ scores when the patients were divided into those with CSA greater than or equal to 70 mm(2) and those less than 70 mm(2), with T=-2.104 and p=0.038. CONCLUSION: The degree of radiographic lumbar spinal stenosis, even with the use of an internal control of vertebral body size and standardized disability questionnaires, does not correlate with clinical symptoms. However, patients with more severe stenosis below a CSA critical threshold of 70 mm(2), have significantly greater functional disability.

New mental retardation syndrome associated with ocular colobomas, cleft palate, and genital, skeletal, and craniofacial abnormalities.

We describe three Canadian brothers of Cree origin, with a previously undescribed pattern of malformation including distinctive craniofacial abnormalities with triangular facies, hypertelorism, low-set and posteriorly rotated ears, ocular colobomas, ptosis, brachycephaly with widely separated sutures, cleft soft palate, undescended testes, bifid scrotum and hypospadius, wide webbed neck, webbed fingers, pectus excavatum and hypersegmented sternum, and severe psychomotor retardation. The presence of normal brain imaging and physical growth distinguishes them from other syndromes with overlapping abnormalities. This is either an X-linked or autosomal recessive condition.

Development and duration of radiographic signs of bone healing in children.

OBJECTIVE: Few articles report the evaluation of pediatric fracture healing and dating based on radiographic appearance. We established a timetable for expected radiographic changes visible during bone healing in otherwise healthy children. MATERIALS AND METHODS: We examined 707 radiographs of fractured forearms in 141 patients. Each fracture was assessed by a pediatric radiologist who was unaware of the timing of the initial injury. Assessment included the following features: fracture margins, fracture gap, periosteal reaction, callus, bridging, and remodeling. The time interval between injury and the appearance of the radiographic features and the duration of radiographic signs were determined and correlated with age, sex, angulation, displacement, and location. RESULTS: Sclerosis at the fracture margins was evident in 85% of fractures 5 weeks after injury. Widening of the fracture gap was observed in 62% of fractures at 6 weeks. Periosteal reaction was evident on all images by 4 weeks, and after 7 weeks, periosteal reaction was separable from cortex in only 10% of fractures. Fracture callus had a density equal to or greater than that of adjacent cortex 10 weeks after injury in 90% of fractures. CONCLUSION: A wide variation exists in the appearance and duration of the radiographic signs of bone healing. Marginal sclerosis should be an expected radiographic sign of normal bone healing. Applying maximum and minimum time spans to objective radiographic signs may aid in fracture dating.

Isolated tubal torsion at menarche- a case report

Background: Adnexal torsion is a well-recognized cause of acute pelvic pain. Isolated tubal torsion with ovarian sparing has certainly been documented, but is uncommon. Although risk factors for the latter include a menstrual period, menarche in particular is not known to predispose a patient to this event. Severe unilateral pelvic pain with first menses is more likely to herald a congenital mullerian anomaly and cryptomenorrhea, particularly when accompanied by a pelvic mass. We present a case of tubal torsion where a coincidental, yet misleading temporal relation to menarche led to a delay in laparoscopy and ultimate diagnosis.Case: KG, an eleven-year-old female, experienced severe right-sided dysmenorrhea with her first and second menses in August and September 1999 respectively. Between episodes, pain, although still present, was more tolerable and the patient never required hospitalization. Ultrasound revealed a lobulated inhomogeneous mass posterior to the uterus and extending from one normal ovary to the other (Figures). MRI further described the mass as pseudoencapsulated with inhomogeneous areas of high attenuation on T1 and T2 images (Figures). Findings were consistent with an endometrioma, but admittedly could have represented a hemorrhagic cystic mass. No definite mullerian anomaly was seen to explain advanced endometriosis, but two focal areas within the endometrial canal raised the possibility of a uterine septum. Examination of the patient (one week after presentation) was not very helpful although she was pubertal, did have a hymenal septum and was mildly tender on bimanual examination in the Pouch of Douglas. The patient had been started on continuous oral contraceptives while undergoing investigations. Pain only recurred during an episode of break-through bleeding. Ultimately she came to laparoscopy and hysteroscopy where chronic right tubal torsion and necrosis was identified with an inflammatory/hemorrhagic reaction in the pelvis (Photos). There were no identifiable fimbria of the right tube which was densely adherent distally to perirectal fat (Photo). No obvious precipitant was found. Laparoscopic lysis of adhesions and right distal salpingectomy was performed (Photo). Her uterine cavity was in fact normal (Photo)Conclusion: Whether or not this patient's right tube was originally normal will never be known. Congenital abnormalities of fallopian tubes do occur and can predispose to torsion. Nonetheless, adnexal torsion must always be kept in mind whenever a woman presents with unilateral pelvic pain. Early diagnosis is paramount in children and women of reproductive age in order to improve the likelihood of adnexal salvage and future fertility. A "gold-standard" radiological investigative tool continues to elude us. Laparoscopy, albeit more invasive, remains an invaluable procedure in this context with relatively low morbidity as compared to the consequences of delayed diagnosis.

Anterior rectal duplication: a diagnostic challenge.

The authors present an anterior rectal cyst in a 14-month-old girl. This rare variant of rectal duplications presented with recurrent urinary infections. The diagnosis was challenging in view of the multiple differential diagnoses to be considered. Magnetic resonance imaging appeared to be the most accurate preoperative investigation. The cyst was removed uneventfully by partial excision and mucosal ablation. An awareness of this variant can lead to early diagnosis and curative resection.

Impact of magnetic resonance imaging on the surgical management of cystic hygromas.

BACKGROUND: The surgical management of cystic hygromas can be challenging, and accurate diagnosis and anatomic localization is essential. The literature on the use of magnetic resonance imaging (MRI) in pediatric cystic hygromas is sparse and mostly limited to radiological descriptions. METHODS: The authors present five cases of cystic hygromas in children ranging in age from 1 to 13 years. The preoperative MRI scans and patient charts were reviewed with attention to the clinical, radiological, surgical, and histological findings. RESULTS: MRI produced highly detailed multiplanar renderings of the cystic hygromas that were both diagnostic and predictive of the subsequent intraoperative findings. This modality helped specifically in some cases to identify lesion extensions that required specific surgical attention. There were no recurrences or complications at a mean follow-up of 18 months. CONCLUSION: The use of MRI in cystic hygromas can facilitate accurate diagnosis and assist in the preoperative planning, thereby contributing to the successful treatment of these lesions.

High-resolution sonography of the abnormal cranial suture.

OBJECTIVE: The purpose of this investigation is to elucidate the sonographic features of abnormal major cranial sutures. MATERIALS AND METHODS: Eight excised synostosed suture specimens were evaluated. The high-resolution sonographic appearance was correlated with the histological section, plain radiographs, CT and MRI. Diastatic and molded sutures were also evaluated with sonography and compared with the normal cranial suture appearance. RESULTS: Synostosed sutures demonstrated one or more of the following features: (a) loss of echo-poor fibrous gap between bony plates (five sagittal and coronal synostoses); (b) irregular thickened inner sutural margin (three lambdoid synostoses); (c) loss of bevelled edge (one lambdoid synostosis); (d) asymmetric anterior fontanelle (one coronal synostosis). Cranial molding results in an overlap of echogenic bony plates. Sutural width (the distance between bony plates) is increased in cases of elevated intracranial pressure. CONCLUSION: Sonography is an inexpensive, radiation-free modality which can confirm synostosis versus molding versus an underlying intracranial lesion as a cause of plagiocephaly. The high-resolution sonographic images also provide a relatively easy means to assess sutural width and may provide information in regard to increased intracranial pressure.

Sonography of normal cranial sutures.

OBJECTIVE: The purpose of this study was to describe the normal sonographic appearance and measurement of normal major cranial sutures in neonates and infants. SUBJECTS AND METHODS: High-resolution sonograms of sagittal, coronal, and lambdoid sutures were obtained for two autopsy specimens and correlated with histologic sections obtained at identical locations. Also, 50 neonates and infants (0-5 months old [corrected age]) who had normally shaped craniums underwent sonography of the brain that produced normal findings. These neonates and infants also underwent sutural sonograms. The width and thickness of each of the major cranial sutures (sagittal, coronal, and lambdoid) were measured, with mean values established. Measurements were analyzed with paired t tests for interobserver variability. Linear regression was used for correlation of measurements with age. RESULTS: With a scan plane perpendicular to the suture line, sonograms revealed sutures as hypoechoic gaps between two hyperechoic bony plates. On sonograms, sagittal sutures had an end-to-end appearance instead of the beveled junction seen throughout most of the coronal and lambdoid sutures. In the 50 patients, sonograms revealed the mean width to be 0.89 +/- 0.35 mm (mean +/- SD) for coronal sutures. 0.93 +/- 0.28 mm for sagittal sutures, and 0.96 +/- 0.39 mm for lambdoid sutures. On sonograms, mean thickness was 1.97 +/- 0.54 mm for coronal sutures, 1.88 +/- 0.56 mm for sagittal sutures, and 2.49 +/- 0.86 mm for lambdoid sutures. We found no interobserver variability (p < or = .05). With linear regression analysis, we found no correlation between suture width or thickness and patient age (r = .01). CONCLUSION: In our study, high-resolution sonography proved to be a reliable and inexpensive technique capable of defining cranial sutures. Preliminary normative data obtained for cranial suture width and thickness showed no correlation with age in our population group. The normative data obtained will allow recognition of abnormal sutures, particularly synostotic or diastatic sutures.

Diagnosis from single-digit radiography.

This pictorial assay illustrates the value of plain radiographs of the digit in diagnosing disease in children. Although the hand is regarded as a mirror of disease, the finger often points to the correct diagnosis. The authors present a series of cases for which a coned-down view of a single digit is given. In some cases the findings indicate a specific diagnosis, whereas in others only a differential diagnosis is possible. The radiographic findings and a brief differential diagnosis, as well as the definitive diagnosis, are presented for each case.

Sonographic diagnosis of systemic venous gas in a patient with pneumatosis intestinalis.

The detection of systemic venous gas due to pneumatosis intestinalis in patients with portal hypertension has not been described. Since sonography is commonly used to investigate conditions associated with pneumatosis intestinalis, it is important to recognize their sonographic appearance and clinical implications.

Tailoring the small-bowel follow-through examination postoperatively in gastroschisis patients.

The purpose of this paper is to establish a small-bowel follow-through (SBFT) protocol in post-operative gastroschisis patients. In 15 years, 19 SBFT examinations have been performed to diagnose or exclude obstruction in 61 gastroschisis patients. The average examination required 6.7 overhead films (range 3-15) and lasted 34 h (1-190 h). The diagnosis of intestinal obstruction was supported on SBFT in only 1 of 19 patients who underwent this examination. In the other 18, the examination showed no obstruction (13 patients) or was nonconclusive (5 patients). The inherent dysmotility associated with gastroschisis can result in redundant overhead films being made during SFBT. We recommend that an SBFT examination in gastroschisis patients consist of (1) fluoro-evaluation of esophagus, stomach, and duodenum; (2a) if normal peristalsis is noted, then an overhead film at 30 min, or (2b), if altered peristalsis or little movement of contrast medium is noted on the 30-min overhead film, then an overhead film at 4 and 12 h. This is followed by overhead films every 24 h if required. This protocol can result in a decrease in patient radiation, department costs, and staff work load.

Sonographic diagnosis of developmental dysplasia of the hip: importance of increased thickness of acetabular cartilage.

OBJECTIVE: Dynamic and static sonography has become the imaging technique of choice in the diagnosis and follow-up of developmental dysplasia of the hip. The purpose of this study was to determine the value of using the thickness of acetabular cartilage as measured on sonograms as a discriminating factor in the diagnosis of developmental dysplasia. SUBJECTS AND METHODS: We evaluated 220 consecutive hips in 110 infants referred for sonography because of possible developmental dysplasia (referred group). We also evaluated 66 hips in 33 infants who had normal findings on physical examination and no clinical or sonographic evidence of the abnormality (control group). The thickness of the acetabular cartilage, the alpha angle, and the presence of subluxation or dislocation on sonograms were recorded. Follow-up clinical and sonographic examinations were performed between 6 weeks and 6 months after the initial examinations in 19 patients in whom the thickness of the acetabular cartilage was increased (> 3.5 mm) and in 20 patients in whom the thickness was in the normal range (< 3.5 mm). All 39 patients who had follow-up examinations were from the referred group, and all these hips were normal according to conventional sonographic criteria on the initial examination. RESULTS: Of the 220 hips in the referred group, 170 were initially classified as normal according to traditional sonographic standards, and 50 were classified as dysplastic (alpha angle < 55 degrees with or without subluxation or dislocation). The mean thickness of the acetabular cartilage in the control group was 2.6 mm (1 SD = 0.37) as compared with 4.6 mm (1 SD = 0.71) for the dysplastic hips in the referred group. The mean thickness in the hips classified as normal in the referred group was 3.0 mm. Nineteen hips in the referred group in which the thickness of the acetabular cartilage was greater than in the control group (i.e., > 3.4 mm) were imaged again during follow-up examinations. Sixteen of these 19 hips had become clinically unstable or dysplastic according to conventional sonographic criteria. CONCLUSION: The results suggest that sonographic evidence of increased thickness of the acetabular cartilage is an early sign of developmental dysplasia of the hip and that close follow-up of infants with this abnormality is indicated.

Synthesis and calcium channel antagonist activity of nifedipine analogues containing 1,2-dihydropyridyl in place of the 1,4-dihydropyridyl moiety.

Calcium channel antagonist activities of the 1,2-dihydropyridines (9) were determined using the muscarinic-receptor mediated Ca2+ dependent contraction of guinea pig ileal longitudinal smooth muscle. The relative potency order for 4-substituted analogs 9 was phenyl greater than 3-trifluoromethyl(nitro)phenyl greater than 4- and 2-trifluoromethyl(nitro)phenyl. Increasing the size of the alkyl ester substituents (9h) enhanced activity. The test results indicate that the 1,2-dihydropyridyl ring system (9) is partially bioisosteric with the 1,4-dihydropyridyl ring system (10). Competitive [3H]-nitrendipine binding studies indicated that the affinities of the 1,2-dihydropyridine analogues were much lower than expected from their ID50 activities, suggesting that these analogues inhibit calcium channels by a different receptor mechanism. In the synthetic work, Hantzsch condensation of aldehydes (3) with alkyl acetoacetates (4) afforded 3,5-dialkyl 2,6-dimethyl-4-(substituted-phenyl)-1,4-dihydropyridine-3,5-dicarboxylat es (5). Oxidation of 5 gave the aromatic pyridines (6) which were elaborated to the l-methylpyridinium methyl sulphates (7) and then to the perchlorates (8). Sodium borohydride reduction of 8 in aqueous ethanol gave 3,5-dialkyl 1,2,6-trimethyl-4-(substituted-phenyl)-1,2-dihydropyridine-3, 5-dicarboxylates (9).