Spontaneous compressive orbital emphysema of rhinogenic origin

We report the case of a young patient who developed spontaneous compressive orbital emphysema after an attack of coughing. At admission the patient presented left proptosis, diplopia, vision impairment and headache. Computer tomography showed air in the lateral part of left orbit compressing the eyeball and the optic nerve medially. It also revealed a sphenoid bone dysplasia with hyperpneumatization of the left greater wing and with two dehiscences in its wall. It was very intriguing to discover that this sphenoid dysplasia and the flap of mucosa covering one dehiscence were causing a ball-valve effect, allowing air to enter but not leave the orbit. Endoscopic sinus surgery was successfully used to treat this case. Received: 3 April 2000 / Accepted: 5 September 2000     

P^21和PCNA在肝硬变组织中表达的研究

目的：探讨Ｐ２１、ＰＣＮＡ的相关关系及它们在肝硬变、肝细胞不典型增生（ＬＣＤ）、肝细胞癌（ＨＣＣ）形成中的作用。方法：运用Ｓ－Ｐ法对６０例单纯肝硬变（不伴癌）、３０例癌旁肝硬变及２７便ＨＣＣ的Ｐ２１、ＨＢｓＡｇ及ＰＣＮＡ表达情况进行实验研究。结果：癌旁肝硬变组织、单纯肝硬变组织、癌组织Ｐ２１阳性率分别为９０％、６８．３３％、６２．９６％,癌旁肝硬变与单纯肝硬变,癌旁肝硬变与癌组织之间判别有显著性意     

全髋关节置换术治疗成人发育性髋关节发育不良的难点及处理策略

发育性髋关节发育不良（developmental dysplasia of the hip, DDH）是成人继发性髋关节骨关节炎的最常见病因之一,早期干预不及时或治疗不当常引起髋关节严重畸形,晚期致残率较高。全髋关节置换术（total hip arthroplasty, THA）是治疗晚期成人DDH较有效的方法,但是由于髋臼和股骨近端解剖形态病理性异常使THA手术难度较大。本文就DDH的分型、手术难点及处理策略等结合近年来相关文献报道进行综述,以期为THA治疗成人DDH提供一定的理论依据。     

Facial haemangioma,agenesis of the internal carotid artery and dysplasia of cerebral cortex: case report

We describe a girl with a facial haemangioma, associated with other vascular anomalies: agenesis of the internal carotid artery, cerebral cortical dysplasia and hypoplasia of the cerebral hemisphere on the same side of the angioma. We studied the patient by conventional arteriography, T1- and T2-weighted MRI and magnetic resonance angiography.     

How to improve histopathological results in the biopsy diagnosis of gut dysganglionosis

In a methodological survey, the technical prerequisites for optimal histopathological diagnosis of gut dysganglionosis are presented. To make a proper diagnosis, the pediatric surgeon or gastroenterologist and the pathologist must consider certain preconditions. The most important steps for the optimal biopsy diagnosis of an aganglionosis, an ultrashort Hirschsprung segment, a intestinal neuronal dysganglionosis (IND), a ganglioneuromatosis, a hypogenesis, or immaturity of the vegetative gut innervation are: (1) taking 3–4 biopsies the size of a peppercorn (3–5 mm³) with submucosa; (2) the best instruments for taking rectal mucosal biopsies are forceps and scissors or a conventional large biopsy forceps; and (3) biopsies may be taken 1 cm, 3–4 cm, 6–9 cm, and 9–12 cm (or from a preternatural anus) above the pectinate line. A biopsy containing mucosa, muscularis mucosae, and submucosa guarantees a satisfying histopathological diagnosis. The native biopsies can be transported on water-ice if the distance to the pathologist takes no longer than 4–6 h. For long distances, biopsies have to be frozen on dry ice (CO₂ –80 °C) and transported in a sufficient amount of dry ice (adapted to the time of transportation). For biopsy processing, the following points are important: a total of 122 to 160 15-m-thick native cryostat serial sections have to be cut per biopsy and distributed on four microscope slides. Forty sections are used for lactic dehydrogenase reactions, 32 for succinic dehydrogenase reactions, and the rest for an acetylcholinesterase (AChE) reaction. An AChE reaction alone is sufficient for the diagnosis of Hirschsprung's disease (HD), but never for IND or other developmental malformations of the submucous and myenteric plexuses. Enzymehistotopochemical reactions allow the assessment of functional parameters. These reactions, in contrast to immunohistochemical staining, offer information about the functional activity of special gut structures, e. g., increased AChE activity in nerve fibers of the rectal wall in HD or a lack of dehydrogenase activity in immature ganglia.     

Placental tissue stem cells and their role in neonatal diseases

Neonatal diseases such as hypoxic ischemic encephalopathy, diseases of prematurity and congenital disorders carry increased morbidity and mortality. Despite technological advancements, their incidence remains largely unabated. Stem cell (SC) interventions are novel therapies in the neonatal world. In pre-clinical models of neonatal diseases, SC applications have shown encouraging results. SC sources vary, with the bone marrow being the most utilized. However, the ability to harvest bone marrow SCs from neonates is limited. Placental-tissue derived SCs (PTSCs), provide an alternative and highly attractive source. Human placentas, the cornerstone of fetal survival, are abundant with such cells. Comparing to adult pools, PTSCs exhibit increased potency, decreased immunogenicity and stronger anti-inflammatory effects. Several types of PTSCs have been identified, with mesenchymal stem cells being the most utilized population. This review will focus on PTSCs and their pre-clinical and clinical applications in neonatology.     

Surgical treatment of a case of early infantile epileptic encephalopathy with suppression-bursts associated with focal cortical dysplasia

We report a surgically treated case of early infantile epileptic encephalopathy (EIEE) with suppression-bursts associated with focal cortical dysplasia. Tonic-clonic seizures followed by a series of spasms occurred about a hundred times a day at a few days of age. Interictal electroencephalogram (EEG) revealed a suppression-burst pattern that was predominant in the left hemisphere. Magnetic resonance imaging (MRI) suggested focal cortical dysplasia in the left prefrontal area. Combination therapies with antiepileptic treatments showed only partial efficacy. The patient underwent lesionectomy at age 4 months, after which he gradually showed psychomotor development and a decrease of spasms to 0-2 series daily. In cases of EIEE with focal cortical dysplasia, surgical treatment may have beneficial effects on both psychomotor development and seizure control.     

Progressive ECG changes in arrhythmogenic right ventricular disease Evidence foran evolving disease

Electrocardiography results were used to assess diagnosis andevolution of arrhythmogenic right ventricular disease. The initialECG presentation and long-term changes were analysed in 74consecutivepatients with symptomatic ventricular tachycardia and arrhythmogenicright ventricular disease. On first available tracings, a left axis deviation of the QRSwas found in 18 patients. The QRS length in V¹ was 110 ms in39 patients, an epsilon wave was present in 17, and a completenght bundle branch block in four patients. The T wave was negativein V₁–V₃ in 37 patients (50%). In 36 patients, long-term electrocardiographic follow-up of9.5 ± 3.2 years was available. During this period, ECGchanges were observed in 20 patients (56%):negative T wavesin 11 patients, a new left axis deviation in three, QRS enlargementin 13 (including eight right bundle branch block), right atrialhypertrophy in three, and paroxysmal or established atrial fibrillationin three. On studying all 110 ECG tracings (74 initial recordings +36follow-up ECGs), we found a strong correlation between QRS orT wave changes and the length of follow-up after the first symptom;mean time interval between first ventricular tachycardia andECG recording was significantly longer in patients with negativeT waves in the right precordial leads, QRS enlargement, or leftaxis deviation, than in patients without such abnormalities.ECG abnormalities were more frequent at 10 year and 5 year follow-upthan on initial tracings. A normal ECG was found in 40% of patientsduring the first year of follow-up, 8% at 5 years, and neverlater than the 6th year. In conclusion, electrocardiographic diagnosis of arrhythmogenicright ventricular disease may be difficult in the initial stageof the disease, since a normal ECG is found in up to 40% ofpatients. During the follow-up, progressive and characteristicECG changes will occur. Arrhythmogenic right ventricular diseasecan be excluded if the ECG is found to be normal 6 years orlater after a first ventricular tachycardia attack.     

Experimental approach to the pathogenesis of the anomalies of amniotic disease

By intra-adnexal injection of glucose in the rabbit embryo, we were able to stimulate all the anomalies associated with "Amniotic Disease". Since we were even able to obtain amniotic bands, this study provides an excellent experimental model of this disease. Resulting lesions occur early in development, corresponding to the first trimester of human gestation. All of the anomalies can ultimately be explained by the destruction of the most superficial cells: epiblastic cells of the embryo and the amnion, subjacent mesenchyme, and endothelial cells. The subsequent lack of interaction between these cells and the importance of the anatomical localizations of resulting hematomas can lead to the pathogenetic approach to this disease. In light of the present study, the disease appears to be caused by an external factor within the amniotic fluid. The exact nature of the destructive agent(s) remains a mystery in man.