The current state of congenital tracheal stenosis

Congenital tracheal stenosis (CTS) is an uncommon condition that has challenged pediatric surgeons for decades. Patients with CTS can present with a wide spectrum of symptoms and varying degrees of severity. In addition, a variety of techniques have been devised to repair this malformation. A review of these procedures and our suggestions for clinical standards and practice guidelines will be presented in this paper. A retrospective review of the literature on CTS from 1964 to 31 March, 2006. There is not one standard technique for the repair of CTS, as individualized approach to each patient and airway lesion is necessary to optimize patient management; nevertheless there is a consensus about segmental resection and anastomosis being best for short segment stenosis while slide tracheoplasty is most effective for the long-segment ones. Conservative management is also an option for select group of patients with careful and close follow up. Survival following surgery over the years has improved, but mortality remained high, particularly in a specific subset of patients presenting at the age less than 1 month with associated cardiac malformations. In conclusion, CTS remains a significant challenge for pediatric surgeons. Additional research is required to improve our understanding of the pathogenesis of CTS, and to develop evidence-based treatment protocols for the entire spectrum of presentation including conservative management.     

Aortic stenosis

Valvular aortic stenosis in pediatric age group is mostly congenital in origin. The aortic valve may be unicuspid, bicuspid, tricuspid or rarely quadricuspid. Left ventricle undergoes concentric hypertrophy secondary to obstruction to its outflow tract. In neonatal aortic stenosis, left ventricle may be hypoplastic. The pathophysiology and clinical presentation vary with the age of onset and severity of obstruction. Echocardiography and Doppler are indispensible for the diagnosis of aortic stenosis and its severity. Cardiac catheterization is not necessary for the diagnosis, however it is performed as a part of balloon aortic valvuloplasty in severe cases. Balloon valvuloplasty is an effective alternative to surgery in pédiatrie age group. Some of these patients require surgical valve repair or replacement on follow-up. Neonates and young infants with critical aortic stenosis present with cardiogenic shock and need aggressive treatment with prostaglandin E1 infusion along with inotropic support. In experienced hands, balloon valvuloplasty is a safe procedure in neonates and infants with critical stenosis. Patients with mild and moderate aortic stenosis may be left on medical follow-up.     

Congenital esophageal stenosis.

We report 6 new cases of congenital esophageal stenosis (CES) that presented to us with special diagnostic and management problems and review the literature on this subject. Gastroesophageal reflux and achalasia are important differential diagnoses of esophageal stenosis, they may be associated with CES making appropriate management even more difficult. Treatment is also controversial, ranging from simple dilatation to segmental resection. CES should be present in every pediatric surgeon's mind should dysphagia occur on the introduction of semisolid food or esophageal food impaction, with a segmental stenosis demonstrated at esophagogram even when more obvious diagnoses are evoked.     

Pediatric catheter interventions: a year in review 2004-2005

PURPOSE OF THE REVIEW: The fields of pediatric and congenital cardiac interventions continue to expand at a rapid pace. The last few years have witnessed a tremendous explosion in procedures such as the transcatheter closure of secundum atrial septal defects, patent foramen ovale, patent ductus arteriosus, stent placement for pulmonary artery stenosis, and coarctation of the aorta. The purpose of this review is to highlight important publications in the last year and the direction the field is taking. RECENT FINDINGS: Research data over the last year has concentrated on six main themes: (a) mid-to-long-term data for device closure of atrial septal defects, the incidence of late cardiac erosions, thrombus formation and heart block; (b) the transcatheter closure of muscular ventricular septal defects; (c) the transcatheter closure of perimembranous ventricular septal defects; (d) the placement of bioprosthetic valves in the pulmonary and aortic positions; (e) the 'hybrid' approach that combines surgical and interventional techniques in the management of various congenital cardiac defects; and (f) fetal interventions. SUMMARY: This review outlines the key data presented in the literature involving interventional cardiology for pediatric and congenital cardiac defects over the last year. It illustrates that in close collaboration with industry, together with bioengineers and cardiothoracic surgeons, adult cardiac interventionists and perinatologists should continue to expand the role of pediatric catheter interventions. Fetal interventions have the potential to alter the natural history of abnormal cardiac development.     

Successful stenting of congenital bronchial stenosis in infancy

Recently, stent implantation has become the treatment of choice for patients with tracheobronchial stenosis due to malignant tumours, tuberculosis and recurrent stenosis following lung transplant. However, reports on this procedure in infants with congenital bronchial stenosis are extremely rare. We report successful stent implantation in an infant with congenital left bronchial stenosis followed by rapid improvement in his respiratory condition. Conclusion The use of a stent in infants is still controversial because size mismatch will take place with growth. However, we believe that implantation of a metallic stent can be the preferred treatment of congenital bronchial stenosis even in small infants. Received: 4 November 1997 / Accepted in revised form: 10 June 1998     

Long-term psychosocial consequences of surgical congenital malformations

Surgical congenital malformations often represent years of treatment, large number of hospital stays, treatment procedures, and long-term functional sequels affecting patients’ psychosocial functioning. Both functional defects and psychosocial difficulties that occur commonly in childhood may pass through adolescence on to adulthood. This overview presents reports published over the past 3 decades to elucidate the long-term psychosocial consequences of surgical congenital malformations.Literature searches conducted on PubMed database revealed that less than 1% of all the records of surgical congenital malformations described long-term psychosocial consequences, but with diverse findings. This inconsistency may be due to methodological differences or deficiencies; especially in study design, patient sampling, and methods. Most of the studies revealed that the functional deficits may have great impact on patients’ mental health, psychosocial functioning, and QoL; both short- and long-term negative consequences. Factors other than functional problems, e.g., repeated anesthesia, multiple hospitalization, traumatic treatment procedures, and parental dysfunctioning, may also predict long-term mental health and psychosocial functioning. Through multidisciplinary approach, pediatric surgeons should also be aware of deficits in emotional and psychosocial functioning. To achieve overall optimal psychosocial functioning, the challenge is to find a compromise between physically optimal treatment procedures and procedures that are not psychologically detrimental.     

Perioperative Care of the Adult with Congenital Heart Disease in a Free-Standing Tertiary Pediatric Facility

Adult patients with congenital heart disease are presenting more frequently for cardiac surgery. Frequently, pediatric congenital heart surgeons perform these procedures at pediatric hospitals. Between July 1995 and June 2000, a retrospective review of adult patients (> or = 18 years old) who had undergone cardiothoracic operations was performed. A total of 112 operations were performed and divided into two groups--81 cardiac operations in 79 patients and 31 noncardiac operations in 23 patients. One patient had a cardiac and noncardiac operation performed. The overall early operative mortality was 6% (6/101). There were 3 late deaths. New-onset cardiac arrhythmias requiring treatment were diagnosed after 5/81 (6%) cardiac operations. Six of 79 (7%) patients were diagnosed with postoperative clinical depression. An acceptable mortality can be achieved when adult patients undergo cardiothoracic operations at a pediatric facility. New-onset arrhythmias necessitating treatment are relatively common, and postoperative clinical depression should be anticipated.     

Current therapeutic indications in laryngeal stenoses in children]

Among pediatric patients with laryngeal stenosis, acquired forms are now more common than congenital forms. External surgery is not always warranted except in complete or tight obstructions. After establishing the diagnosis by endoscopy, consequences on respiration and phonation should be assessed before deciding on the most appropriate treatment: abstention, medical therapy, endoscopic treatment, or surgical treatment by the cervical route. Surgical laryngoplasty techniques have changed radically over the last twenty years. The Cotton procedure is the most widely used. Another available method is the cricoid-split technique. These laryngoplasty procedures can be performed from birth and tracheostomy is now warranted only after failure of initial surgery.     

Intrahepatic cholangiojejunostomy for complex biliary stenosis after pediatric living‐donor liver transplantation

The treatment of biliary stenosis after pediatric LDLT is challenging. We describe an innovative technique of peripheral IHCJ for the treatment of patients with complex biliary stenosis after pediatric LDLT in whom percutaneous treatment failed. During surgery, the percutaneous biliary drainage is removed and a flexible metal stylet is introduced trough the tract. Subsequently, the most superficial aspect of the biliary tree is recognized by palpation of the stylet's round tip in the liver surface. The liver parenchyma is then transected until the bile duct is reached. A side‐to‐side anastomosis to the previous Roux‐en‐Y limb is performed over a silicone stent. Among 328 pediatric liver transplants performed between 1988 and 2015, 26 patients developed biliary stenosis. From nine patients requiring surgery, three patients who had received left lateral grafts from living‐related donors due to biliary atresia were successfully treated with IHCJ. After a mean of 45.6 months, all patients are alive with normal liver morphological and function tests. The presented technique was a feasible and safe surgical option to treat selected pediatric recipients with complex biliary stenosis in whom percutaneous procedures or rehepaticojejunostomy were not possible, allowing complete resolution of cholestasis and thus avoiding liver retransplantation.     

Three-dimensional imaging of the pediatric trachea in congenital tracheal stenosis

Technological advances in three-dimensional computer software packages have resulted in fairly widespread acceptance of 3D imaging for musculoskeletal studies. We have recently applied this technology to axial CT images of the pediatric trachea for children with congenital tracheal stenosis, in an effort to replace tracheo-bronchography with a safer, less invasive modality to image the pediatric trachea. This method has provided our surgical colleagues with images which they find easier to interpret because they are more anatomically oriented with respect to regional mediatinal structures, which can be included or removed from the image at will. The images can also be manipulated and rotated through various projections, thus facilitating surgical planning.     

影像学成像技术评价血管狭窄的研究进展

血管狭窄主要分为先天性血管狭窄及后天获得性血管狭窄两大类.先天性血管狭窄病因未明,多见于先天性心血管及脑血管狭窄等疾病;后天获得性血管狭窄多见于动脉粥样硬化斑块、血管内皮支架植入术及球囊成形术后.影像学方法有助于准确、及时地帮助临床诊断、评估各种先天性或后天性血管狭窄.目前影像学成像技术快速发展,影像学新技术层出不穷.该文简要综述影像学新技术用于评价血管狭窄的进展.     

Isolated congenital tracheal stenosis in a preterm newborn

Severe tracheal stenosis, resulting in functional atresia of the trachea is a rare congenital malformation with an estimated occurrence of two in 100,000 newborns. If no esophagotracheal fistula is present to allow for spontaneous breathing, this condition is usually fatal. We report on a male infant born at 32 weeks of gestation. The patient presented with respiratory distress immediately after delivery due to severe congenital tracheal stenosis resulting in functional atresia of the trachea. Endotracheal intubation failed and even emergency tracheotomy did not allow ventilation of the patient lungs. The patient finally succumbed to prolonged hypoxia due to functional tracheal atresia. The etiology of tracheal atresia and tracheal stenosis is still unclear, but both conditions are frequently combined with other anomalies of the VACTERL (vertebral anomalies, anal atresia, cardiovascular anomalies, tracheoesophageal fistula, esophageal atresia, renal/radial anomalies and limb defects) and TACRD (tracheal agenesis, cardiac, renal and duodenal malformations) association. Conclusion Successful treatment of severe congenital tracheal stenosis and tracheal atresia depends on either prenatal diagnosis or recognition of this condition immediately after birth to perform tracheotomy without delay. Nevertheless, despite any efforts, the therapeutical results of severe tracheal stenosis and tracheal atresia are still unsatisfactory.     

Pulmonary development considerations in the surgical management of congenital diaphragmatic hernia

Congenital diaphragmatic hernia remains a clinical challenge for both neonatologists and pediatric surgeons. Advancements in mechanical ventilation strategies and neonatal intensive care have improved survival and transformed treatment of congenital diaphragmatic hernia from emergent surgery to early stabilization of the newborn followed by delayed repair of the diaphragmatic defect. Surgical technique has evolved and minimally invasive surgical approaches to close the diaphragmatic defect in these babies will likely improve with increasing experience. Finally, as more patients are diagnosed prenatally, attempts have been made to close the diaphragmatic defect prenatally. Unfortunately, this approach did not change the outcome of affected babies. Recently, progress has been made with prenatal tracheal plugging to improve prenatal lung development. In the near future experimental studies will start to explore new ways of treating affected babies prior to birth. This article reviews the evolution of the current treatment strategies in congenital diaphragmatic hernia and its future directions.     

Congenital nasal stenosis in newborn infants.

Five newborn infants with evidence of nasal obstruction were shown to have congenital nasal stenosis. Conservative treatment, including temporary nasopharyngeal intubation in three, eventually resulted in symptomatic relief, so that surgery could be avoided.     

Acquired non hypertrophic pyloric stenosis in children]

Pediatric non hypertrophic pyloric stenosis (NHPS) are uncommon. Their causes and treatments are debated.Material and methodRetrospective review of all cases of NHPS from 3 pediatric surgery services during the period 1984–2002.ResultsSix children, aged 17 months to 15 years, underwent surgery for NHPS. Clinical symptoms, food vomiting and loss of weight, were present for several weeks before the diagnosis of NHPS was made. The diagnosis was peptic stenosis in 3 cases and has not been established in 3 cases. Search for Helicobacter pylori was negative in all cases. Failure of specific medical treatment and endoscopic dilatations led to pyloric resection in 3 cases and pyloroplasty in 3 cases. Post operative course was uneventful with normal oral feeding and normalisation of weight status. Histologic data were aspecific. No recurrence was observed.DiscussionWe discuss the origin of the pyloric stenosis, regarding clinical, operative and pathological data: were the stenosis the cause or consequence of peptic ulcer? Peptic disease is always advocated, but difficult to prove and may be excessively incriminated. Late symptomatic congenital and acquired idiopathic pyloric stenosis should be recalled. In all cases of proved pyloric stenosis, after failure of medical and endoscopic treatment, a simple surgical procedure (pyloroplasty) associated with medical treatment seems to be effective.ConclusionThe diagnosis of NHPS should be suspected in a child with food vomiting and loss of weight if his age is not concordant with hypertrophic pyloric stenosis. Upper gastro-intestinal series and endoscopy are diagnostic. The precise cause of the stenosis is more difficult to asses. When the medical treatment fails, a pyloroplasty is usually curative.     

Surgical outcome of congenital valvar aortic stenosis

Valvar aortic stenosis is a common congenital heart defect for which surgical procedures can be done with low risk except in infants whose conditions are seriously compromised. The purpose of this report was to present our experience with the results of surgical aortic valvotomy for congenital valvar aortic stenosis performed at our hospital. The study group consisted of 24 patients, 3 females and 21 males, with ages ranging from 1 to 15.5 years (mean age 8.5 years), who underwent aortic valvotomy for valvar aortic stenosis. The case records of all the patients were retrospectively reviewed. They were followed for 1-10.5 years (mean 5.02+/-2.38 years) after relief of aortic stenosis, and were scheduled for reevaluation. Sixteen of the 24 patients were recatheterized and 15 (93.7%) were found to have aortic regurgitation on angiography. Peak systolic pressure gradients (mean+/-SD) were 65.9+/-19.5 mmHg before and 36.7+/-14.8 mmHg (p<0.05) after the operatilon. Of the 24 patients, 45.8% had a new postoperative diastolic murmur. Twenty patients (83.3%) had residual stenosis and three (12.5%) had recurrent stenosis. Two patients (8.3%) had undergone reoperation six to seven years after the initial aortic valvotomy, and most of the others will require reoperation in the future. No sudden deaths occurred in this series. Timely relief of obstruction prevents sudden death and produces symptomatic improvement in valvar aortic stenosis, but aortic valvotomy is only a palliative measure.     

Congenital esophageal stenosis: the differential diagnosis and management

Congenital esophageal stenosis (CES) is a rare congenital abnormality that is difficult to diagnose and often masquerades as other types of structural esophageal disease. We report three cases of CES with different presenting symptoms. We advocate for balloon dilation as the preferred first approach to therapeutic intervention. CES is an important clinical entity in the evaluation of pediatric esophageal disorders and should be suspected in young infants with dysphagia.     

Cheatham-Platinum支架置入术治疗儿童先天性心脏病血管狭窄的疗效评价

目的 评价NuMED Cheatham-Platinum(CP)支架置入术治疗儿童先天性心脏病(CHD)血管狭窄的即刻和早中期疗效.方法 2005年8月至2007年5月,采用CP支架置入治疗5例CHD血管狭窄(先天性主动脉缩窄1例,肺动脉狭窄4例).男3例,女2例,年龄4～15岁(中位年龄12岁),体重20～51 kg(中位体重24 kg).根据数字减影血管造影结果选择CP支架和NuMED双球囊,支架准确定位后先后充盈内外球囊扩张支架.结果 5例CHD血管狭窄进行了6次支架置入操作,共置入8只支架,均为8-zig CP支架,支架长度22～39 mm.除1例右肺动脉狭窄支架置入术中移位而于术后11个月重新置入第2只CP支架外,余4例6只支架即刻置入成功.跨狭窄段收缩压差由术前(43.43±25.61)mm Hg(1 mm Hg=0.133 kPa)降至术后(3.29±3.09)mm Hg(t=4.320,P<0.01),最窄处血管直径由术前(6.86±2.04)mm增加至术后(13.44±4.02)mm(t=-4.508,P<0.01).2例单侧肺动脉分支狭窄术后狭窄侧肺血流量占全肺血流量百分比分别由11.0%和13.0%增加至47.5%和52.2%,2例双侧肺动脉分支狭窄的右心室/主动脉收缩压比分别由术前62.3%和72.2%降至术后27.0%和33.3%.1例主动脉缩窄术后上肢血压由206/133 mm Hg降至156/95mm Hg.随访13～34个月(中位时间20个月),2只CP支架于术后6个月发生支架内再狭窄,余结果稳定无并发症发生.结论 CP支架置入术治疗儿童CHD血管狭窄安全可行,即刻和早中期疗效较好,但远期疗效有待进一步随访和更多病例的研究.     

儿童先天性主动脉瓣上狭窄的外科治疗

目的 回顾总结儿童先天性主动脉瓣上狭窄(SVAS)的外科治疗经验,提高治疗水平.方法 1990年至2006年10月25例先天性主动脉瓣上狭窄患儿接受手术治疗.其中男16例,女9例,手术平均年龄(5.4±2.4)岁,平均体重(12.6±3.5)kg.影像学检查发现22例为局限性狭窄,主动脉瓣上0.5～2.0 cm处有狭窄段,3例为弥漫性狭窄.平均压力阶差(81.7±21.5)mmHg,William's综合征12例.所有患儿均在体外循环下行补片扩大主动脉成形术,11例以"泪珠"状补片扩大成形修补,14例以"裤衩"状补片扩大加宽主动脉狭窄部.补片材料选择应用自体心包17例,Dacron涤纶补片2例,Gore-tex人工血管补片6例,其中内衬自体心包片1例.对伴发畸形同时加以纠治.结果 术后检查主动脉瓣上流速、压差均明显下降,平均(26.0±13.2)mmHg,住院死亡1例,存活24例.结论 应用"泪珠"状和"裤衩"状补片进行主动脉扩大成形术矫治儿童主动脉瓣上狭窄有效,尤以"裤衩"状补片为首选,婴幼儿补片材料以自体心包为宜.