1/36. Functional intestinal obstruction due to deficiency of argyrophil neurones in the myenteric plexus. Familial syndrome presenting with short small bowel, malrotation, and pyloric hypertrophy.In 3 infants functional intestinal obstruction, associated with a short small intestine, malrotation, and pyloric hypertrophy, was shown to be due to failure of development of the argyrophil myenteric plexus, with the absence of ongoing peristalsis. 4 infants with similar clinical features have been described previously, and there is evidence for an autosomal recessive mode of inheritance of this syndrome.- - - - - - - - - - ranking = 1keywords = plexus (Clic here for more details about this article) |
2/36. Congenital hemihypertrophy and epithelioid haemangioendothelioma in a 10-year-old boy: a case report.Epithelioid haemangioendothelioma has not been previously described in a patient with congenital hemihypertrophy and diabetes mellitus. Hepatic nodules were incidentally discovered on a routine US examination searching for known associated abnormalities. Pulmonary nodules were present on chest X-ray and CT of the lungs. The diagnosis was confirmed by open biopsy of a hepatic nodule. Despite significant disease progression the patient remains symptom free.- - - - - - - - - - ranking = 0.0014506596904314keywords = chest (Clic here for more details about this article) |
3/36. Hypertrophic inflammatory neuropathy involving bilateral brachial plexus.BACKGROUND: The present case is an example of hypertrophic inflammatory neuropathy (HIN). This entity is a rare tumor-like, chronic inflammatory, focal or multifocal, mainly demyelinating neuropathy of unknown origin, most frequently involving the brachial plexus. CASE DESCRIPTION: The authors describe a 67-year-old man presenting with a nodular mass in his right supraclavicular fossa. A nodular mass grossly resembling a schwannoma originating from a single nerve fascicle was surgically removed from the right C6 spinal nerve. Histologically, endoneurial edema, fibrosis, focal chronic inflammation, and extensive "onion bulb" formation were seen. Electron microscopy studies and immunohistochemistry proved that the onion bulb-forming cells were schwannian in nature and that the whorls of onion bulbs surrounded a generally demyelinated axon. Three months following surgery the patient developed acute painless paralysis of his right biceps brachii muscle that rapidly reversed; after that he remained neurologically asymptomatic. MRI revealed multiple fusiform mass lesions involving the brachial plexus bilaterally. Electrophysiologic studies demonstrated a bilateral, asymmetrical, mainly demyelinating neuropathy involving the brachial plexus; they failed to reveal any abnormality suggestive of generalized neuropathy. CONCLUSION: HIN is different from other focal tumor-like neuropathies and in particular from localized hypertrophic neuropathy (LHN).- - - - - - - - - - ranking = 1.4keywords = plexus (Clic here for more details about this article) |
4/36. Chronic demyelinating hypertrophic brachial plexus neuropathy.A patient with unilateral, painless, chronic progressive upper limb sensorimotor deficit showed electrophysiological evidence of a focal demyelinating neuropathy with almost complete conduction block across the brachial plexus. magnetic resonance imaging disclosed marked brachial plexus hypertrophy. Intravenous immunoglobulin led to fast and complete recovery, maintained by intermittent perfusions. Hypertrophic brachial plexus neuropathy can be a presentation of focal chronic inflammatory demyelinating polyradiculoneuropathy. Objective and quantitative assessment of hand function is useful to evaluate treatment results and to optimize treatment regimens.- - - - - - - - - - ranking = 1.4keywords = plexus (Clic here for more details about this article) |
5/36. Primary hypertrophic colonopathy.We report a 45-year-old man and a 60-year-old woman who presented with features of intermittent intestinal obstruction. barium enema revealed narrowing at the pelvic-rectal junction in the man, and from the pelvic colon to the anal verge in the woman. histology of the resected sections showed marked hypertrophy of the muscularis propria in both cases, with normal mucosa, submucosa and myenteric plexus. Both patients are asymptomatic at 4 years' and 2 years' follow up. This entity of primary hypertrophic colonopathy may be a variant of primary visceral myopathy.- - - - - - - - - - ranking = 0.2keywords = plexus (Clic here for more details about this article) |
6/36. Sonographic detection of diffuse peripheral nerve hypertrophy in chronic inflammatory demyelinating polyradiculoneuropathy.Chronic inflammatory demyelinating polyradiculoneuropathy is an autoimmune disease characterized by recurrent demyelination and remyelination with resultant thickening of the peripheral nerves. We report a case in which sonography was instrumental in demonstrating diffuse peripheral nerve hypertrophy. On sonography, both brachial plexuses were found to be diffusely hypertrophic and hypoechoic. Similar findings were noted for the median, sciatic, and femoral nerves. The brachial plexus findings were confirmed by MRI.- - - - - - - - - - ranking = 0.4keywords = plexus (Clic here for more details about this article) |
7/36. Villous hypertrophy versus choroid plexus papilloma: a case report demonstrating a diagnostic role for the proliferation index.Diffuse bilateral enlargement of the choroid plexus has long been appreciated as a cause of shunt-resistant hydrocephalus in infants, most often secondary to cerebrospinal fluid overproduction. Despite the fact that such lesions have interchangeably been termed 'villous hypertrophy' (VH) or 'bilateral choroid plexus papilloma' (CPP), little attention has been paid to the pathological distinctions that characterize these two entities. We present a case of presumed VH in a 2-week-old female presenting with cerebrospinal fluid production-dependent hydrocephalus that ultimately required bilateral surgical ablation of the choroid plexus. Although the preoperative radiological diagnosis was consistent with VH, postoperative histological analysis suggested bilateral CPP. The neoplastic nature of the lesion was confirmed by specific staining for the proliferation index, showing an MIB-1 labeling index of 4%, characteristic of papilloma. Routine imaging and histological grading of choroid plexus hypertrophy and papilloma have not provided a reliable means of predicting malignant behavior or recurrence after surgical resection. Moreover, none of the previously reported cases of VH in the literature have analyzed pathological specimens for cellular proliferative potential. Therefore, we review the literature on VH and bilateral CPP and discuss the diagnostic and possible prognostic implications of distinguishing these variants by utilizing the MIB-1 marker for the proliferation index.- - - - - - - - - - ranking = 1.6keywords = plexus (Clic here for more details about this article) |
8/36. Marble impaction in the nasopharynx following oral ingestion.Foreign body ingestion is an important emergency occurring in childhood. In this article, we report the case of a 5-year-old girl with marble impaction in the nasopharynx, which had not been discovered by routine x-rays of the chest and abdomen at the time of ingestion. The patient presented 4 months after the event with typical symptoms of adenoid hypertrophy and sinusitis, and the diagnosis was established on the basis of a plain film of the nasopharynx. This rare situation is potentially dangerous, since the foreign body may descend and cause sudden airway obstruction. Therefore, in all cases with vanishing foreign bodies in the aerodigestive system, nasopharyngeal impaction and its fatal consequences should be kept in mind and endoscopic examination of the region should be considered.- - - - - - - - - - ranking = 0.0014506596904314keywords = chest (Clic here for more details about this article) |
9/36. Chronic gastric volvulus and hypertrophic pyloric stenosis in an infant.Chronic secondary mesenteroaxial gastric volvulus associated with hypertrophic pyloric stenosis has rarely been mentioned in the general medical literature. We report the case of a 2-month-old boy whose clinical symptoms, which included distension of the stomach and projectile vomiting, suggested the diagnosis of hypertrophic pyloric stenosis but who was later diagnosed with chronic secondary mesenteroaxial gastric volvulus. Sonographic examination revealed findings consistent with a rotated stomach, and subsequent plain chest radiography demonstrated a left diaphragmatic eventration. An upper gastrointestinal series radiographic examination with barium contrast enhancement confirmed the diagnosis of mesenteroaxial gastric volvulus. Surgery was performed, and the boy recovered well. A follow-up sonographic examination performed 3 months post-operatively revealed no abnormalities. This case demonstrates that primary or secondary gastric volvulus, although rare in children, should be considered in the differential diagnosis of pediatric patients with a history of vomiting.- - - - - - - - - - ranking = 0.0014506596904314keywords = chest (Clic here for more details about this article) |
10/36. A case of superficial esophageal cancer complicated with idiopathic muscular hypertrophy of the esophagus.We hereby reported a case of 60-year old man with superficial esophageal cancer complicated with idiopathic muscular hypertrophy of the esophagus. Endoscopic ultrasonography and CT showed the thickness of esophageal muscular layer, but the accurate diagnosis could not be entertained before operation. Idiopathic muscular hypertrophy of the esophagus is an entity rarely encountered, and most cases are diagnosed at postmortem examination. Only a few cases have been reported regarding its clinical symptoms and images. The etiology remains to be elucidated, and the pathologic features are characterized by significant thickness of inner circular muscular layer of esophagus without degeneration of plexus and ganglionic cells. This case report deals with superficial esophageal cancer complicated with idiopathic muscular hypertrophy of esophagus. literature review is also included.- - - - - - - - - - ranking = 0.2keywords = plexus (Clic here for more details about this article) |
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