Antenatal detection and management of suprarenal masses

The indications for, and timing of, surgical intervention for suprarenal masses detected prenatally are unclear. Also, the definitive diagnosis of suprarenal masses using imaging studies is difficult at best. We report 2 cases of antenatally detected suprarenal masses. One case represents an initial cystic mass expanding and becoming solid that had benign pathologic features. The second case was a stable solid mass that, on exploration, was malignant. Management options are discussed.     

Antenatal and perinatal uro-nephrology: current questions and dilemmas

The development of antenatal ultrasonography and the detection of fetal uropathies has created a new field for paediatric urologists. The embryology and physiology of the fetal renal tract is still poorly understood. It is, however, recognised that delays in the maturation of the renal system can lead to transient dilatation of the urinary tract. Structural and functional anomalies can also result in dilatation, but these are permanent. The ability to distinguish between the transient and permanent impairments to urine flow represents a diagnostic challenge. This review article discusses the pathophysiology of urine flow impairment, antenatal detection and the postnatal management of the common congenital uropathies. Received: 13 February 1998 / Revised: 30 July 1998 / Accepted: 8 October 1998     

MRI评估胎儿神经系统畸形

目的探讨MRI诊断胎儿中枢神经系统畸形的价值及其应用指征。方法回顾性分析450例中枢神经系统畸形胎儿的超声和MRI资料,与产后诊断或尸体检查结果进行对照。结果 450例患者共检出神经系统病变594处,包括单纯侧脑室增宽99处、脑积水21处、小脑延髓池增宽59处、Dandy-Walker畸形/小脑发育不良45处、胼胝体发育不良/缺如67处、透明隔增宽6处、脑白质软化15处、室管膜下囊肿/出血30处、颅内出血26处、颅内实质性病变14处、蛛网膜囊肿60处、孔洞脑13处、脑裂14处、积水型无脑畸形3处、脑水肿1处、平滑脑4处、脑组织发育不良3处、全前脑20处、小头畸形8处、头形异常3处、开放性神经管缺陷68处、脑血管畸形5处、透明隔缺如2处、颅底凹陷症1处、枕骨大孔窄1处、囊性松果体1处、脉络丛病变5处。超声正确诊断487处,准确率81.99%(487/594),MRI正确诊断551处,准确率92.76%(551/594)。结论对于胎儿神经系统各种病变,超声和MRI各有优劣,明确MRI应用指征有助于指导合理地选择产前MRI,从而明确诊断。     

Antenatal diagnosis of neonatal surgical diseases

With advances in noninvasive fetal diagnostic tools, such as ultrasonography and magnetic resonance imaging, the percentage of neonatal surgical patients detected antenatally has increased. Recently, color Doppler as well as 3D imaging has contributed to more accurate diagnoses with ultrasonography. The significance of antenatal diagnosis for neonatal surgical disease and the current progress in fetal surgery are described and discussed. At present, congenital diaphragmatic hernia with extremely severe hypoplastic lung, congenital cystic adenomatoid malformation with hydrops, obstructive uropathy with oligohydramnios, and simple ovarian cyst greater than 5cm in diameter are considered to be appropriate indications for fetal surgery.     

Hand abnormalities in Apert’s syndrome: surgical management

Apert’s syndrome is the most common of the acrocephalosyndactylies with complex malformations of the hands. Treatment of the Apert hand is complex, and numerous procedures are required. The aim of this study is to propose a strategy for hand management. Sixteen Apert’s syndrome hands were submitted to early surgery, which included opening of the first web, separation of the fingers, realigninment of the thumb, and correction of the clinodactylies. We performed an average of six operations per child. Treatment of the first web depended on the classification of Upton: For the severe stages, we used a dorsal hand flap. Radical clinodactyly was treated by osteotomy of the delta phalanx and Z-plasty. We treated nine hands with four fingers and seven hands with five fingers. There was always bilateral opposition and symphalangism excluding the fifth finger. All of the children have a rudimentary but functional pinch grip. Revision of the webs was necessary in 16% of the cases. The Apert hand requires early and specialized treatment that aims to provide a functional hand before 2 or 3 years, with the least surgical complications. The functional prognosis is adversely affected by symphalangism.     

Impact of core-needle breast biopsy on the surgical management of mammographic abnormalities

OBJECTIVE: To evaluate the accuracy of percutaneous, image-guided core-needle breast biopsy (CNBx) and to compare the surgical management of patients with breast cancer diagnosed by CNBx with patients diagnosed by surgical needle-localization biopsy (SNLBx). SUMMARY BACKGROUND DATA: Percutaneous, image-guided CNBx is a less invasive alternative to SNLBx for the diagnosis of nonpalpable mammographic abnormalities. CNBx potentially spares patients with benign lesions from unnecessary surgery, although false-negative results can occur. For patients with malignant lesions, preoperative diagnosis by CNBx allows definitive treatment decisions to be made before surgery and may affect surgical outcomes. METHODS: Between 1992 and 1999, 939 patients with 1,042 mammographically detected lesions underwent biopsy by stereotactic CNBx or ultrasound-guided CNBx. Results were categorized pathologically as benign or malignant and, further, as invasive or noninvasive malignancies. Only biopsy results confirmed by excision or 1-year-minimum mammographic follow-up were included in the analysis. Patients with breast cancer diagnosed by CNBx were compared with a matched control group of patients with breast cancer diagnosed by SNLBx. RESULTS: Benign results were obtained in 802 lesions (77%), 520 of which were in patients with adequate follow-up. Ninety-five of the 520 evaluable lesions (18%) were subsequently excised because of atypical hyperplasia, mammographic-histologic discordance, or other clinical indications. There were 17 false-negative CNBx results in this group; 15 of these lesions were correctly diagnosed by excisional biopsy within 4 months of CNBx. In two patients (0.9%), delayed diagnoses of ductal carcinoma in situ were made at 15 and 19 months after CNBx. Malignant results were obtained in 240 lesions (23%), 220 of which were surgically excised from 202 patients at our institution. Two lesions diagnosed as ductal carcinoma in situ were reclassified as atypical ductal hyperplasia and considered false-positive results (0.4%). For malignant lesions, the sensitivity and specificity of CNBx for the detection of invasion were 89% and 96%, respectively. During the first surgical procedure, 115 of 199 patients (58%) diagnosed by CNBx underwent local excision; 194 of 199 patients (97%) evaluated by SNLBx underwent local excision. For patients whose initial surgery was local excision, those diagnosed before surgery by CNBx had larger excision specimens and were more likely to have negative surgical margins than were patients initially evaluated by SNLBx. Overall, patients diagnosed by CNBx required fewer surgical procedures for definitive treatment than did patients diagnosed by SNLBx. CONCLUSIONS: Diagnosis by CNBx spares most patients with benign mammographic abnormalities from unnecessary surgery. With the selective use of SNLBx to confirm discordant results, missed diagnoses are rare. When compared with SNLBx, preoperative diagnosis of breast cancer by CNBx facilitates wider initial margins of excision, fewer positive margins, and fewer surgical procedures to accomplish definitive treatment than diagnosis by SNLBx.     

Delayed surgical resection of central nervous system germ cell tumors

OBJECTIVE: To determine the value of delayed surgical resection in patients with central nervous system germ cell tumors who exhibit less than complete radiographic response despite declining serum and cerebrospinal fluid (CSF) tumor markers after initial chemotherapy. METHODS: We retrospectively analyzed 126 patients enrolled on two international multicenter clinical trials (the First and Second International Central Nervous System Germ Cell Tumor Studies) for patients with newly diagnosed central nervous system germ cell tumors. After at least three cycles of chemotherapy, 10 of these patients underwent delayed surgical resection owing to evidence of residual radiographic abnormalities despite declining or completely normalized serum and CSF levels of alpha-fetoprotein and human chorionic gonadotropin. RESULTS: Eight of these patients demonstrated nongerminomatous germ cell tumor elements at the time of initial diagnosis. In these patients, either serum or CSF tumor markers were elevated initially. Two patients demonstrated pure germinomas with normal levels of serum and CSF tumor markers. After chemotherapy, radiographic evaluation revealed a partial response in seven patients, a minor response in one patient, and stable disease in two patients. All 10 patients had either normal or decreasing levels of serum and CSF tumor markers before second-look surgery. At delayed surgical resection, 7 of the 10 patients underwent gross total resection, and 3 patients underwent subtotal resection of residual lesions. Pathological findings at second-look surgery demonstrated three patients to have mature teratomas, two with immature teratomas, and five with necrotic or scar tissue alone. To date, 7 of the 10 patients have had no recurrence during an average follow-up time of 36.9 months (range, 3-96 mo). Three of four patients with nongerminomatous germ cell tumors who had tumor markers that were decreased, but not normalized, before second-look surgery eventually developed tumor dissemination/progression, and they required subsequent radiation therapy despite having teratoma or necrosis/scar tissue at delayed surgery. In contrast, three of four patients with nongerminomatous germ cell tumors and completely normalized markers did not progress and did not require radiation therapy. CONCLUSION: Delayed surgical resection should be considered in patients with central nervous system germ cell tumors who have residual radiographic abnormalities and normalized tumor markers, because these lesions are likely to be teratoma or necrosis/scar tissue. However, second-look surgery should be avoided in patients whose tumor markers have not normalized completely.     

七氟烷对发育期中枢神经系统毒性的研究进展

背景 七氟烷作为临床上常用的吸入麻醉药,广泛用于儿科手术中.近年来,关于七氟烷发育期中枢神经系统(central nervous system,CNS)毒性的研究成为人们关注的焦点.目的 文章就近年发表的有关七氟烷对发育期CNS毒性相关研究进展作一综述.内容 七氟烷神经毒性与神经保护的双重性;七氟烷与异氟烷对发育期CNS毒性作用的比较;七氟烷对发育期CNS毒性作用的机制研究及预防手段探讨. 趋向 今后的研究重点是开展可靠的临床试验分析、比较各种吸入麻醉药在小儿麻醉中的优劣、明确七氟烷麻醉对幼儿神经发育的影响及可能的治疗手段.     

Potentially toxic effects of anaesthetics on the developing central nervous system

A growing body of experimental evidence suggests that anaesthetics, by influencing GABAergic and glutaminergic neural signalling, can have adverse effects on the developing central nervous system. The biological foundation for this is that gamma-aminobutyric acid and glutamate could act non-synaptically, in addition to their role in neurotransmission in the adult brain, in the regulation of neuronal development in the central nervous system. These neurotransmitters and their receptors are expressed from very early stages of central nervous system development and appear to influence neural progenitor proliferation, cell migration and neuronal differentiation. During the synaptogenetic period, pharmacological blockade of N-methyl-d-aspartate (NMDA)-type glutamate receptors as well as stimulation of GABAA receptors has been reported to be associated with increased apoptosis in the developing brain. Importantly, recent data suggest that even low, non-apoptogenic concentrations of anaesthetics can perturb neuronal dendritic development and thus could potentially lead to impairment of developing neuronal networks. The extrapolation of these experimental observations to clinical practice is of course very difficult and requires extreme caution as differences in drug concentrations and exposure times as well as interspecies variations are all important confounding variables. While clinicians should clearly not withhold anaesthesia based on current animal studies, these observations should urge more laboratory and clinical research to further elucidate this issue.     

Entry, dispersion and differentiation of microglia in the developing central nervous system

Microglial cells within the developing central nervous system (CNS) originate from mesodermic precursors of hematopoietic lineage that enter the nervous parenchyma from the meninges, ventricular space and/or blood stream. Once in the nervous parenchyma, microglial cells increase in number and disperse throughout the CNS; these cells finally differentiate to become fully ramified microglial cells. In this article we review present knowledge on these phases of microglial development and the factors that probably influence them.     

Morphine in tetanus--the management of sympathetic nervous system overactivity

Morphine was administered intravenously in bolus doses 6-hourly to 10 patients; 9 developed signs of sympathetic overactivity and required increased morphine dosage. The mean daily morphine dosage was 103 +/- 36 mg and the maximum daily dosage was 170 +/- 65 mg. In all cases morphine decreased the mean arterial blood pressure (mean 18%; P less than 0.01) and heart rate (mean 7%; P less than 0.01). In 7 cases the cardiac output fell minimally (mean 7%; P = 0.07), while the systemic vascular resistance decreased (mean 12%; P less than 0.01) in 8 cases. Nine patients survived, 1 died from renal failure and septicaemia. There were no apparent problems with either opiate withdrawal or addiction. No patient required either alpha- or beta-adrenergic blockers and the consequent simplified management constitutes a significant improvement in control of these patients.