Scolioses idiopathiques et congénitales

Résumé D'étiologie toujours inconnue, la scoliose idiopathique de l'adolescent ne peut être traitée efficacement que si le dépistage est très précoce, entre 8 et 10 ans. La preuve de l'évolutivité angulaire doit être faite avant d'entreprendre un traitement orthopédique, dont l'objectif sera de contrôler l'aggravation liée à la poussée de croissance pubertaire. De nouveaux protocoles à temps partiel sont actuellement à l'étude et s'adresseront à des courbures peu sévères et bien réductibles. Les indications opératoires sont posées en cas d'échec ou d'intolérance au traitement orthopédique. Les limites angulaires pour de telles interventions semblent diminuer progressivement, les techniques devenant plus fiables. On accorde par ailleurs de plus en plus d'importance au plan sagittal, compte tenu de la mauvaise tolérance à long terme des lordoses thoraciques. Malgré les progrès techniques, la réalisation d'une arthrodèse solide avec une greffe abondante reste le seul garant de la stabilité angulaire. L'évolution et l'histoire naturelle des courbures congénitales du rachis sont actuellement bien connues, ainsi que les malformations viscérales habituellement associées. La surveillance radiographique dès le plus jeune âge permet d'évaluer l'asymétrie de croissance vertébrale dans le plan frontal et sagittal. Ceci permet de proposer en cas d'évolutivité un traitement à visée étiologique précoce, sous forme d'une hémi arthrodèse et épiphysiodèse convexe, antérieure et postérieure pour freiner l'hypercroissance du côté de la malformation. Pour les formes dépistées plus tardivement, une greffe postérieure avec ou sans matériel est l'indication classique, en évitant absolument toute manoeuvre de distraction qui comporte un risque de complication neurologique, en particulier en cas de composante cyphotique associée.

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Idiopathic and congenital scoliosis

Summary The exact etiology of idiopathic scoliosis is still unknown and therefore the only way to improve the prognosis is to recommend an early screening program, between 8 and 10 years of age. Angular progression must be proven for every curve before any brace application. The goal of such a treatment is to control curve progression during the adolescent growth spur. Recent part-time bracing programs have been proposed for low angle and flexible curves. Surgery is indicated in case of failure or non compliance of the conservative treatment. Currently the angular limits for surgery seem to decrease, as a result of improved surgical techniques. Sagittal unbalance and loss of thoracic lordosis are of major importance for the treatment choice, since their poor outcome in the adult population. Despite technical improvements, the quality of a solid fusion is the most important factor for long lasting results. The natural history and evolution of congenital spine deformities have been precisely documented, as well as the frequently associated visceral problems. Repeated curve measurements in young children will evaluate the growth potential asymetry in the coronal and sagittal planes. In case of rapidly progressive curves, an early surgical treatment can be proposed, consisting in convex anterior and posterior hemiarthrodesis, producing an epiphysiodesis effect of the overgrowing convex side. For cases diagnosed in older ages, the classic indication is a posterior fusion, with or without instrumentation. It is important to avoid any excessive distraction, because of a high risk of neurological complications, especially in case of an associated kyphotic deformity.

Travail présenté aux Actualités. en Orthopédie Pédiatrique organisées par le Dr Cl. Karger, le 19 au 21 mars 1992 à Aubure     

Kongenitale Duodenalstenose im Erwachsenenalter

Zusammenfassung Eine hochgradige Duodenalstenose im Erwachsenenalter kann in seltenen Fällen angeboren sein und findet ihre Ursache in einer intraduodenal gelegenen Membran. Die Anamnese zeigt Wachstumsstörungen mit Erbrechen und Meteorismus und abdominelle Beschwerden. Eine Perforationsöffnung in dieser Membran ist die Ursache fur ein Überleben bis ins Erwachsenenalter. Die Röntgendarstellung und die tiefe Duodenoskopie lassen auf einfache Weise die Diagnose stellen. Differentialdiagnostisch müssen der Volvulus bei Malrotation, Ladd-Bänder und das Pancreas anulare, sowie die Kompression des Duodenums durch Mesenterialgefäße erwogen werden. Das operative Verfahren der Wahl ist die Resektion der intraduodenalen Membran. Dabei ist v. a. die Papilla Vateri darzustellen, da diese nicht selten im Bereich des Septums mündet. Das längs eröffnete Duodenum wird quer verschlossen. Bei der Wind-Sock-Web-Anomalie sollte das Duodenum an der Ansatzstelle des Diaphragmas eröffnet werden. Die Anlage einer Gastrojejunostomie ist inadäquat und zu vermeiden.

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Congenital duodenal stenosis in adulthood

A high-grade duodenal stenosis in adults can, in rare cases, be congenital, and its cause is found in an intraduodenally sited membrane. The anamnesis reveals growth disorders with vomiting and meteorism and abdominal complaints. A perforation opening in this membrane is the reason for survival into adulthood. The X-ray appearance and deep duodenoscopy make the diagnosis easy. Volvulus in cases of malrotation, Ladd's ligaments, anular pancreas, and compression of the duodenum by mesenteric vessels must be considered in the differential diagnosis. When the intraduodenal membrane is resected it is most important to expose the papilla Vateri, since this not uncommonly ends in the area of the septum. If necessary, a duodenoduodenostomy is performed. If the windsock web abnormality is present the duodenum should be opened at the point of attachment of the diaphragm. The construction of a gastrojejunostomy should be avoided.

     

成人先天性胆管扩张症手术治疗

目的 探讨成人先天性胆管扩张症手术治疗的经验体会。方法 回顾分析1985年1月至2004年12月中国医科大学附属第一医院普外科收治的成人先天胆管扩张症68例手术治疗资料。依Flanigan分型：Ⅰ型59例，Ⅱ型3例，Ⅳ型6例。所有病例均行囊肿切除，结果 术后发生胆汁漏6例。胰漏3例，急性胰腺炎2例。死亡2例，56例经1-20年随访，随访率为82．3％。5例表现轻度胆管炎，2例吻合口狭窄。余下49例均痊愈，无胆管炎及胆管狭窄表现。结论 囊肿切除、胆管空肠Roux-en-Y吻合是成人先天性胆管扩张症的首选治疗。切除囊肿时，囊肿近端在尽量切净囊肿基础上行大口吻合，防止术后吻合口狭窄；囊肿远端在保证睫液引流通畅、不损伤胰管开口的基础上，尽量切净囊肿内膜。     

Prevalence and estimated incidence of Toxoplasma infection among pregnant women in Poland: a decreasing trend in the younger population

This study investigated the prevalence of specific Toxoplasma gondii IgG in pregnancy, the incidence of congenital toxoplasmosis and the prevalence trend of T. gondii infection among pregnant Polish women between 1998 and 2003. The study population comprised 4916 women who were admitted to the Polish Mother's Memorial Hospital Research Institute in Łódź. Their sera were tested for specific IgG and IgM antibodies to T. gondii, and the incidence of T. gondii infection was calculated from the increase in prevalence rates of IgG antibodies in various age groups. Specific IgG antibody was found in 41.3% (95% CI 39.9–42.7) of pregnant women, and the prevalence of IgG increased with age. The linear trend was significant (p <0.001), with an annual seroconversion rate of 0.7% (95% CI 0.004–0.010). The risk of primary infection was estimated to be 0.5% for 9 months, i.e., an incidence of 5/1000 pregnancies. Assuming a 30% maternofetal transmission rate, 1.5/1000 neonates were infected in utero. Seroprevalence during the 6-year study period decreased from 45.4% in 1998 to 39.4% in 2003, with a yearly decline in prevalence of 1.0% (p 0.02). The most important contributory factor to this decline was the group of women aged 19–29 years, among whom seroprevalence decreased significantly (p 0.007). Specific IgM was found in 244 (4.9%) women.     

冠状动脉起源于肺动脉的影像学研究

目的 探讨冠状动脉(简称冠脉)起源于肺动脉(ACAPA)影像学诊断方法的价值。方法 回顾性分析11例ACAPA的影像学表现。11例均行X线胸片、超声心动图(Echo)和心血管造影检查，其中1例行电子束CT(EBCT)检查。结果 10例为左冠脉起源于肺动脉，1例为右冠脉起源于肺动脉。11例胸片均未确诊，Echo诊断3例，EBCT诊断1例。心血管造影全部诊断正确，其中左冠脉异常起源者左冠脉均发自主肺动脉后窦或后壁，通过扩张的右冠脉藉侧支逆行充盈；右冠脉异常起源者右冠脉从主肺动脉右窦发出。手术与造影所见相同。3例前乳头肌缺血性纤维化，二尖瓣环扩大，前叶脱垂致二尖瓣关闭不全。结论 X线胸片诊断受限，Echo简便、无创，但操作技术及认知水平有待提高。心血管造影仍是术前确诊的“金标准”。     

The relations among putative biorisk markers in schizotypal adolescents: minor physical anomalies, movement abnormalities, and salivary cortisol.

BACKGROUND: Evidence suggests that prenatal insult may play a role in the etiology of psychotic disorders. Minor physical anomalies (MPA) are an indicator of abnormal fetal development and are elevated in individuals at genetic and behavioral risk for psychosis. Yet, there has been little empirical research on the relationships between MPAs and other neurobiological risk indicators. We hypothesized that the frequency of MPAs (an external marker of prenatal central nervous system [CNS] disruption) would be associated with two other biomarkers suggestive of disruptions in fetal neurodevelopment: movement abnormalities (an indicator of striatal abnormalities) and heightened cortisol secretion (an indicator of hypothalamic-pituitary-adrenal [HPA]/hippocampal function). METHODS: Participants with schizotypal personality disorder (SPD; n = 39) and both normal (n = 47) and other personality disorders (n = 28) control subjects were administered structured diagnostic interviews and assessed for MPAs, movement abnormalities, and salivary cortisol. RESULTS: Schizotypal personality disorder participants showed significantly greater MPAs and movement abnormalities and higher cortisol than both the normal and other personality disorders groups. Hierarchical linear regression analyses revealed that higher rates of MPAs were linked with greater movement abnormalities and salivary cortisol. CONCLUSIONS: The findings suggest that MPAs serve as a marker of neurodevelopmental abnormalities that affect striatal and hippocampal regions.     

先天性心脏病导管介入治疗对小儿生长发育和内分泌功能的影响

目的　了解先天性心脏病 (CHD)导管介入治疗对患儿生长发育和内分泌功能的影响。方法　测定 4 2例CHD患儿导管介入治疗前后的身高、体重和三碘甲状腺原氨酸 (T3)、甲状腺素 (T4 )、促甲状腺素 (TSH)、生长激素(HGH)。结果　治疗前后身高、体重的增长速度明显改善 ,而T3、T4、TSH、HGH无明显改变。结论　导管介入治疗能明显改善先心病患儿生长发育而不会影响患儿的内分泌功能     

Integrated home monitoring predicts lead failure in a pacemaker dependent 4-year-old girl.

A 4-year-old girl with post-surgical complete atrioventricular block received an epicardial dual chamber pacemaker system. During further growth intermittent exit block occurred, first misinterpreted as neurological seizures. The epicardial lead was replaced using a transvenous approach, and a pacemaker with an integrated home monitoring facility was implanted. After her discharge, a rise in the pacing threshold automatically initiated an event message. On the basis of this information, the patient was called in and imminent dislodgement of the ventricular lead was diagnosed by x-ray. The lead was repositioned and was found stable over 1-year follow-up.     

Staged surgical repair of functional single ventricle in infants with unobstructed pulmonary blood flow

Objective: The infant with a functional single ventricle (SV) and unobstructed pulmonary blood flow (UPBF) requires early protection of the pulmonary vascular bed to ensure suitability for a subsequent Fontan procedure. Systemic obstruction by aortic arch obstruction, subaortic stenosis, or combination of both, has been widely recognized as an important risk factor for poor outcome in children with SV–UPBF who are palliated with pulmonary artery banding (PAB). We reviewed our experience with primary PAB in the subset of patients with SV–UPBF to identify risk factors for subsequent palliative procedures and Fontan completion. Methods: Between January 1990 and May 2004, 80 patients (median age, 14 days) with functional SV and UPBF underwent PAB as their primary palliative procedure. Thirty-five neonates had concomitant aortic coarctation or interrupted aortic arch repair (44%). A Damus–Kaye–Stansel procedure was subsequently performed in 19 patients, and subaortic resection or ventricular septal defect or bulboventricular foramen enlargement was performed in five. Results: There were 4 operative deaths, and 15 late deaths. The actuarial overall survival is 84% at 1 year, 76% at 5 and 15 years. Follow-up is complete in all but six children at a mean interval of 4.9±3.7 years (range, 2 months–15 years). Thirty-seven patients (49%; 37 of 76) have undergone the hemi-Fontan procedure (with three hospital deaths) and 40 patients (53%; 40 of 76; 12 children without previous hemi-Fontan) have undergone the completion Fontan procedure without mortality or Fontan takedown. Conclusion: In infants with single ventricle physiology with or without systemic outflow obstruction and unobstructed pulmonary blood flow, a strategy of pulmonary artery banding carries acceptable operative and mid-term mortality in a high-risk group of patients. Pulmonary artery banding does not compromise performance of subsequent Damus–Kaye–Stansel procedure or completion Fontan palliation.