Early complications of stenting in patients with congenital heart disease: a multicentre study.

AIMS: Stenting has become an established interventional cardiology procedure for congenital heart disease. Although most stent procedures are completed successfully, complications may occur. This multicentre study evaluated early complications after stenting in patients with congenital heart disease, including potential risk factors. METHODS AND RESULTS: In this combined Dutch-Belgian retrospective study, 309 consecutive patients had undergone 366 catheterizations and received 464 stents in 13 different anatomical positions (418 sites). Seventy-two stenting-related complications (19%) occurred, of which 24 (5.7%) were major. Seven procedure-related deaths were documented (2.3%). Stent malpositioning and embolization were most common (7.7%). The use of non-premounted stents tended to be associated with higher complication rates. Centre inexperience with stenting and stenting of native vs. post-surgical stenosis tended to be associated with increased major complication rates. CONCLUSION: After stenting, complications are common for congenital heart disease. The vast diversity of stenotic sites combined with relatively small patient populations makes these procedures sensitive to complications. Combining operator experience may reduce the risks of stenting in congenital heart disease. The availability of premounted stents for greater vessel diameters will likely reduce incidences of stent migration and embolization.     

Histological changes in the midbrain around the aqueduct in congenital hydrocephalic rat LEW/Jms

Primary aqueductal stenosis is one of the main causes of congenital hydrocephalus in humans and experimental models. The congenitally hydrocephalic rat strain LEW/Jms is one such model. In this report, we describe further detailed histological features of periaqueductal structure, including the posterior commissure, subcommissural organ (SCO), and ependyma, and discuss the changes in these structures in relation to the cause of hydrocephalus. Coronal sections of the aqueduct in normal rats showed that the usual ependyma was absent in the center of the base facing the dorsal side, which was replaced by tall columnar cells. On the other hand, in hydrocephalic rats the ependyma encircled the aqueductal cavity. In midline sagittal sections, normal and hydrocephalic rats showed the SCO, although the SCO in hydrocephalic rats was shorter than in normal rats. There was also a marked difference between normal and hydrocephalic rats in the dorsoventral dimension of the rostral midbrain. In hydrocephalus, this dimension was large in comparison with normal rats. The superior collicular commissure located caudal to the posterior commissure ran along the ventral side of the midbrain in rats with hydrocephalus, and there was a cell-depleted area just dorsal to the superior collicular commissure. The same findings were observed from the 17th day of gestation until the postnatal period. Although the role of the SCO has been widely discussed from the viewpoint of secretory function, the present study indicated that this organ might be involved in the formation of the shape of the aqueduct.     

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。     

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.     

腹腔镜下幽门环肌切开术治疗小儿先天性肥厚性幽门狭窄50例报告

目的总结腹腔镜下幽门环肌切开术治疗小儿先天性肥厚性幽门狭窄的成功经验.方法2001年4月～2004年4月,应用腹腔镜下幽门环肌切开术治疗小儿先天性肥厚性幽门狭窄50例,年龄12～90 d,平均35 d.分别在左、右上腹各置入3 mm trocar,左侧trocar置入无损伤抓钳夹近幽门处胃壁,右侧trocar先后置入伸缩式幽门肌切开刀、剥离器和幽门分离钳,完成幽门环肌切开术.结果腹腔镜下完成手术48例,中转开腹2例,其中1例为术中发现幽门前瓣膜症,1例为幽门黏膜损伤,经开腹修补痊愈.手术时间15～45 min,平均25 min.术后6 h拔胃管,开始喂奶.3～5 d出院.42例术后随访3～6个月,平均4.5月,生长发育均恢复正常.结论丰富的开腹手术经验、熟练的腹腔镜操作技术、术中良好的麻醉和合适的手术器械是完成腹腔镜下幽门环肌切开术的保障.