CONGENITAL CARDIAC DEFECTS—Indications for Surgical Repair

The optimum ages for the operative repair of the various congenital defects have not as yet been definitely established. With certain exceptions, it has generally been the authors'' preference to delay elective operations until after the age of 18 months. In the usual case, the optimum age for the closure of septal defects is believed to be three to five years. Operative relief of stenosis of the aortic or pulmonic valves, however, is related entirely to the extent of the obstruction, age not being a factor. Although the surgical treatment of coarctation of the aorta is probably best delayed beyond the period of infancy, it is the authors'' belief that in the presence of extreme hypertension, cardiomegaly or cardiac failure, early resection of the aortic block should be accomplished. As to the treatment of tetralogy of Fallot, the authors'' policy is to delay operation until such time as definitive repair becomes less hazardous. Patients with severe symptoms, however, are given the benefit of some form of operative relief as soon as possible. Whether this is palliative or corrective depends upon the characteristics of the individual case.Definitive diagnostic studies are of great aid in assessing the urgency of operative intervention. The fact that approximately one-third of the patients dealt with required operation at less than 18 months of age suggests that, in selected cases, these studies should be performed early in infancy.     

Ruptured aneurysms of sinuses of Valsalva

At least one additional cardiac lesion was present in 18 consecutive patients with ruptured aneurysms of the sinuses of Valsalva who were investigated between 1956 and 1973 at the University of Alberta Hospital. Clinical diagnosis was made in 78% (14/18) of the patients. Confirmation at cardiac catheterization, operation or autopsy was obtained in all but one. The main sites of rupture were the right ventricle (seven cases), right atrium (five) and left ventricle (five). Fifty percent (9/18) are alive and well following prompt operative repair, an average of 8.2 years later (range, six months to 15 years). Replacement of the aortic valve was associated with a high mortality (50% early, 13% late, total 63%) which could be explained by the higher operative risk in this group of very ill patients. Eight patients (44.4%) had had bacterial endocarditis prior to presentation and this may have played a significant role in the rupture of the sinus of Valsalva aneurysm.     

Early repair and breast-feeding for infants with cleft lip

This study attempts to define the effect of early repair and breast-feeding on the outcome of cleft lip surgery. The first part deals with 100 consecutive cleft lip repairs categorized retrospectively by age at operation. Forty-nine patients were operated on during the first 3 weeks of life; 51 at an older age. There were no statistically significant differences in complication rate between the groups (14 and 18 percent, respectively). A subgroup of 26 infants was operated on at a week or less of age; these sustained significantly fewer complications (8 percent). There was no apparent difference in the operative results as defined by whether or not the child needed a subsequent revision. A second group of 60 mothers was offered the choice of breast-feeding their babies immediately following operation. Sixteen breast-fed for a minimum of 6 weeks, 22 were fed by means of a cup or syringe, and 22 started breast-feeding but converted to a bottle within 6 weeks. No complications attributable to breastfeeding were observed, and the rate of weight gain was definitely enhanced in the breast-feeding group. Hospital stay was shortened by an average of over a day (33 percent) as compared with those fed by cup. This effect was related to the easier transition from IV administration to oral intake when breast-fed. We are currently encouraging early repair and breast-feeding in the full-term baby as the optimum method of management of newborns with cleft lip.     

The Operative Approach to Congenital Aortic Stenosis in Childhood

Forty-seven infants and children, aged from one month to 15 years, were operated upon during the past six years at the Hospital for Sick Children, Toronto, using extracorporeal circulation. Retrograde cardiac catheterization of the left ventricle with a pull-through tracing across the valve is important in selection of candidates for operation. Patients with a pressure gradient of over 45 mm. Hg require operation. In this series, valvular stenosis was the condition most commonly encountered; subvalvular membranous stenosis was present in eight patients, and muscular subvalvular stenosis in four. There was one operative death in an infant with multiple anomalies, and one late death from subacute bacterial endocarditis two years after operation. It is suggested that early operative treatment in cases of aortic stenosis in infancy and childhood might lessen the incidence of calcific aortic stenosis in adult life.     

FUNNEL CHEST (PECTUS EXCAVATUM) IN INFANCY AND ADULT LIFE

Funnel chest, unless corrected, may lead to cardiorespiratory distress or psychic problems or both. Since operation to correct the deformity is relatively simple in infancy and much more extensive if done later, early surgical intervention is indicated. If the condition is not noted until the patient is beyond infancy, cardiorespiratory studies will aid in determining the advisability of operation.     

Mechano-biology in the thoracic aortic aneurysm: a review and case study

An aortic aneurysm is a permanent and localized dilatation of the aorta resulting from an irreversible loss of structural integrity of the aortic wall. The infrarenal segment of the abdominal aorta is the most common site of aneurysms; however, they are also common in the ascending and descending thoracic aorta. Many cases remain undetected because thoracic aortic aneurysms (TAAs) are usually asymptomatic until complications such as aortic dissection or rupture occurs. Clinical estimates of rupture potential and dissection risk, and thus interventional planning for TAAs, are currently based primarily on the maximum diameter and growth rate. The growth rate is calculated from maximum diameter measurements at two subsequent time points; however, this measure cannot reflect the complex changes of vessel wall morphology and local areas of weakening that underline the strong regional heterogeneity of TAA. Due to the high risks associated with both open and endovascular repair, an intervention is only justified if the risk for aortic rupture or dissection exceeds the interventional risks. Consequently, TAAs clinical management remains a challenge, and new methods are needed to better identify patients for elective repair. We reviewed the pathophysiology of TAAs and the role of mechanical stresses and mathematical growth models in TAA management; as a proof of concept, we applied a multiscale biomechanical analysis to a case study of TAA.     

Coarctation of the aorta in adults.

Thirty-six patients, 19 men and 17 women, presented at age 18 or older between 1952 and 1974 with coarctation of the aorta. Of the 14 (39%) who had associated cardiovascular disease, 12 had aortic stenosis or insufficiency or both. Three patients had infections-two, endocarditis (aortic valve) and one, endarteritis. Three of the seven patients who did not undergo an operation are alive, two at more than 50 years of age. Five patients had myocardial infarctions, two at 35 years of age. Twenty-nine (80%) had operations; in eight instances the patient was over age 40. All 18 patients undergoing repair of isolated coarctation survived, while only 7 of the 11 patients with associated cardiovascular lesions who underwent repair recovered. Aortic valvular disease and myocardial infarction are serious complicating factors in coarctation of the aorta.     

Ten-year follow-up after endovascular repair of traumatic abdominal aortic rupture

Blunt abdominal aortic injury is often associated with bowel injury that precludes operative repair because of the risk of graft infection. Endovascular repair has been reported but with limited follow-up. We present a case of a 15-year-old boy who underwent endovascular repair of blunt abdominal aortic rupture and whom we were able to follow up over a decade. Our experience with this case and three others, as well as the experience reported in the literature, suggests that endovascular repair is a reasonable option in the setting of concomitant bowel injury. The risk of over sizing, collapse, and migration may be less than that described for thoracic aortic injuries because there is no need to deploy the endograft across an angle.     

Cleft palate repair at 3 to 7 months of age

We report the speech outcome in 90 children with complete unilateral cleft lip and palate who underwent soft palate repair either between 3 and 7 months of age (n = 40) or later than 7 months of age (n = 50). In all patients, palatoplasty was performed by one of two experienced surgeons using a modification of the Furlow technique, and speech evaluations were conducted using the Pittsburgh Weighted Values for Speech Symptoms Associated with Velopharyngeal Incompetence by two speech pathologists with high inter-rater reliability. There were no differences between the groups with respect to resonance, nasal air emission, and articulation. Velopharyngeal function, as measured by the total speech score, was similar between the two groups of patients, as were the rates of secondary pharyngoplasty. These results suggest that very early closure of the soft palate may not offer significant benefit over repair later in infancy with respect to speech outcome.     

Relationship between bronchial hyperresponsiveness and impaired lung function after infantile asthma

Wheezing during infancy has been linked to early loss of pulmonary function. We prospectively investigated the relation between bronchial hyperresponsiveness (BHR) and progressive impairment of pulmonary function in a cohort of asthmatic infants followed until age 9 years. We studied 129 infants who had had at least three episodes of wheezing. Physical examinations, baseline lung function tests and methacholine challenge tests were scheduled at ages 16 months and 5, 7 and 9 years. Eighty-three children completed follow-up. Twenty-four (29%) infants had wheezing that persisted at 9 years of age. Clinical outcome at age 9 years was significantly predicted by symptoms at 5 years of age and by parental atopy. Specific airway resistance (sRaw) was altered in persistent wheezers as early as 5 years of age, and did not change thereafter. Ninety-five per cent of the children still responded to methacholine at the end of follow-up. The degree of BHR at 9 years was significantly related to current clinical status, baseline lung function, and parental atopy. BHR at 16 months and 5 years of age did not predict persistent wheezing between 5 and 9 years of age, or the final degree of BHR, but it did predict altered lung function. Wheezing that persists from infancy to 9 years of age is associated with BHR and to impaired lung function. BHR itself is predictive of impaired lung function in children, strongly pointing to early airway remodeling in infantile asthma.     

Endoscopically assisted "components separation" for closure of abdominal wall defects

The repair of ventral hernia defects of the abdominal wall challenges both general and plastic surgeons. Ventral herniation is a postoperative complication in 10 percent of abdominal surgeries; the repair of such defects has a recurrence rate as high as 50 percent. The "components separation" technique has successfully decreased the recurrence rates of ventral abdominal hernias. However, this technique has been associated with midline dehiscence and a prolonged postoperative stay at the authors' institutions. The purpose of this study was to determine whether endoscopically assisted components separation could minimize operative damage to the vasculature of the abdominal wall and decrease postoperative wound dehiscence. The study group consisted of seven patients who underwent endoscopically assisted components separation; the control group consisted of 30 patients who underwent open components separation. The two groups were similar regarding demographic data and defect size. The endoscopic group had a higher initial success rate than the open group (100 versus 77 percent). Recurrence rates were not significantly different between the two groups. However, the endoscopically assisted components separation patients had fewer postoperative and long-term complications. In the authors' experience, endoscopically assisted components separation has proved to be a safe and effective method for the repair of complicated and recurrent midline ventral hernias.     

改良带蒂胸大肌肌皮瓣修复口腔颌面肿瘤术后缺损的临床分析

目的:探讨只带血管蒂的改良胸大肌皮瓣转移修复口腔颌面部组织缺损的临床效果。方法:取胸大肌皮瓣时蒂部只保留血管,利用改良只带血管蒂的胸大肌皮瓣对6例不同口腔颌面部组织缺损进行修复,包括舌癌3例、舌咽癌1例、牙龈癌1例、颊癌1例。结果:术后皮瓣血供良好,完整成活,成活率100%;所有患者获得3~18个月随访,在随访期内均存活;重建的舌外形良好,虽然味觉功能无法恢复,运动功能随切除范围增加而降低,但均能满足发音、吞咽和咀嚼功能需要。结论:只带血管蒂的改良胸大肌皮瓣是修复口腔颌面部组织缺损有效而可靠的方法,为恶性肿瘤根治术后造成的缺损提供了有力修复保障。     

Extrinsic Mortality Effects on Reproductive Strategies in a Caribbean Community

Extrinsic mortality is a key influence on organisms’ life history strategies, especially on age at maturity. This historical longitudinal study of 125 women in rural Domenica examines effects of extrinsic mortality on human age at maturity and pace of reproduction. Extrinsic mortality is indicated by local population infant mortality rates during infancy and at maturity between the years 1925 and 2000. Extrinsic mortality shows effects on age at first birth and pace of reproduction among these women. Parish death records show huge historical variation in age-specific mortality rates. The infant mortality rate (IMR) in the early 1920s was low, increased dramatically beginning in 1929, and reached a maximum in the 1950s, at which point IMR declined steadily to its present low rate. The mortality rate early in life showed a quadratic association with age at first birth. Women who experienced conditions of low IMR early in life reproduced relatively late in life. Those born into moderately high levels of infant mortality tended to reproduce earlier than those born at low levels. At very high infant mortality levels early in life, women went on to delay reproduction until relatively late, possibly as a result of somatic depletion and energetic stress associated with the conditions that lead to high IMR. Population mortality rates at age of maturity also showed a quadratic association with age at first birth. The pace of reproduction, estimated as number of surviving offspring controlled for maternal age, showed a similar quadratic effect. There were complex interactions between population mortality rates in infancy and at maturity. When extrinsic mortality was high during infancy, extrinsic mortality later in life had little effect on timing of first birth. When extrinsic mortality was low to moderate in infancy, extrinsic mortality later in life had significant effects on adult reproduction. I speculate that these effects are mediated through development of personality facets associated with reproduction.     

自固化磷酸钙人工骨修复小儿局部骨缺损的临床应用

目的：研讨自固化磷酸钙人工骨（Calcium Phosphate Cement,CPC)填充修复小儿局部骨缺损的临床意义,方法：选用CPC修复小儿骨缺损18例,年龄最小8个月,最大12岁,平均8岁,骨缺损部位：肱骨9例,胫骨6例,胫骨3例,病因,单纯性骨囊肿8例,骨纤维异常增生症5例,动脉瘤样骨囊肿4例,嗜酸性肉芽肿1例,骨缺损大小,最大7cm,最小2cm,平均5cm,CPC填充方式：单纯粉末7例,粉末＋松质骨粒6例,粉末＋条形骨块5例。CPC初步固化时间,最短15分钟,最长30分钟,平均20分钟,随访时间：13－27个月,平均18．5个月。结果：全组18例应用CPC后未见明显局部和全身不良反应。手术前后血PH值钙磷代谢无异常改变。X线片显示：CPC与宿主骨接触紧密,无脱落,术后3个月出现降解,新生骨形成。结论：CPC安全无毒,使用方便,易塑形,生物相容性好,能在体内降解,可以替代自体骨材料在小儿局部骨缺损应用。     

Testosterone measured in infancy predicts subsequent sex-typed behavior in boys and in girls

The testes are active during gestation, as well as during early infancy. Testosterone elevation during fetal development has been shown to play a role in human neurobehavioral sexual differentiation. The role of early postnatal gonadal activation in human psychosexual development is largely unknown, however. We measured testosterone in 48 full term infants (22 boys, 26 girls) by monthly urinary sampling from day 7 postnatal to age 6 months, and related the area under the curve (AUC) for testosterone during the first 6 months postnatal to subsequent sex-typed behavior, at the age of 14 months, using the Pre-School Activities Inventory (PSAI), and playroom observation of toy choices. In boys, testosterone AUC correlated significantly with PSAI scores (Spearman's rho = 0.54, p = 0.04). In addition, play with a train and with a baby doll showed the anticipated sex differences, and play with the train correlated significantly and positively with testosterone AUC in girls (Spearman's rho = 0.43, p = 0.05), while play with the doll correlated significantly and negatively with testosterone AUC in boys (Spearman's rho = -0.48, p < 0.03). These results may support a role for testosterone during early infancy in human neurobehavioral sexual differentiation.     

Surgical management of traumatic disruption of the descending aorta.

During an 11 1/2-year period, 20 consecutive patients presenting with a traumatic disruption of the proximal descending aorta underwent an emergency operative repair. The mean age was 26 years (range 15 to 62), and 13 (65%) were male. Associated injuries were frequent and required additional major operative procedures in half of the cases. Two patients died as a result of associated intracranial injuries, for a hospital survival of 90%. The operative repair was accomplished by graft replacement of the involved segment of the aorta in all but one patient who underwent a primary repair. Simple aortic crossclamping was used in 8 patients (40%) and heparinless femoral-femoral venoarterial bypass in 12 patients (60%). Neither renal failure nor paraplegia in any of the patients. Four patients required thoracic reoperations. These results indicate that an aggressive multidisciplinary surgical approach can produce favorable results in patients with traumatic descending aortic injuries.     

Delay in motor development of twins in Africa: a prospective cohort study.

Twins are prone to developmental delay due to prematurity and low birthweight. However it is unknown if twinning is an independent risk factor for developmental delay. The objective of this study was to compare the attainment of a set of gross motor milestones in a cohort of twins and singletons in The Gambia. Eighty-four pairs of twins and 72 singletons were enrolled at birth and followed up until 18 months of age. The mean age at achieving milestones was higher in twins for each development outcome and the difference between twins and singletons was significant after adjustment for confounders for maintaining head, sitting without support and walking. In twins, we found a highly significant correlation within pairs for most milestones. When monozygotic and dizygotic twins were compared, a significant heritability was observed for crawling, sitting, standing and walking, with over 90% of population variance observed due to genetic factors rather than environmental factors. There was little evidence for a genetic contribution towards very early milestones. In conclusion, our data suggest that twinning is an independent risk factor for developmental delay in early life in The Gambia, and that genetic factors contribute strongly to certain motor development outcomes.     

Results of Parks operation for faecal incontinence after anal sphincter injury.

Parks operation for faecal incontinence was performed on 97 patients with total loss of anorectal control due to injury. All had sustained complete division of the anal sphincters as a result of trauma, anal surgery, or obstetric tears and either were incontinent or had been given a colostomy. In all patients the divided sphincters were repaired using an overlapping technique; in 93 the repair was protected by a temporary defunctioning stoma. There was no operative mortality. Continence was completely restored in 65 (78%) and improved in a further 11 (13%) of the 83 patients assessed from four to 116 months postoperatively. Minor complications which did not affect the eventual clinical outcome occurred in 23 patients. Factors associated with failure of the operation included breakdown of the repair in the early postoperative period, fistula, and pelvic floor neuropathy. The results show that even after severe injury to the sphincters surgical reconstruction can restore continence in most patients.     

Neonates mount robust and protective adult-like CD8(+)-T-cell responses to DNA vaccines

Neonates are thought to mount less vigorous adaptive immune responses than adults to antigens and infectious agents. This concept has led to a delay in the administration of many currently available vaccines until late infancy or early childhood. It has recently been shown that vaccines composed of plasmid DNA can induce both humoral and cell-mediated antimicrobial immunity when administered within hours of birth. In most of these studies, immune responses were measured weeks or months after the initial vaccination, and it is therefore questionable whether the observed responses were actually the result of priming of splenocytes within the neonatal period. Here we show that DNA vaccination at birth results in the rapid induction of antigen-specific CD8(+) T cells within neonatal life. Analyses of T-cell effector functions critical for the resolution of many viral infections revealed that neonatal and adult CD8(+) T cells produce similar arrays of cytokines. Furthermore, the avidities of neonatal and adult CD8(+) T cells for peptide and the rapidity with which they upregulate cytokine production after recall encounters with antigen are similar. Protective immunity against the arenavirus lymphocytic choriomeningitis virus, which is mediated by CD8(+) cytotoxic T cells, is also rapidly acquired within the neonatal period. Collectively these data imply that, at least in the case of CD8(+) T cells, neonates are not as immunodeficient as previously supposed and that DNA vaccines may be an effective and safe means of providing critical cell-mediated antiviral immunity extremely early in life.     

Hybrid thoracic stent graft repair of a complex type B aortic dissection in a patient who presented three weeks after repair of a type a aortic dissection

Long-term management after repair of a type A aortic dissection includes aggressive medical therapy and routine surveillance with serial imaging to ensure thrombosis of the false lumen. Retained patency of the false lumen can lead to either the development of a false lumen aneurysm with a subsequent rupture or extension of dissection. Typically such events occur late, usually months after repair, and are treated with either a conventional one-stage open thoracoabdominal repair or a two-stage "elephant trunk" procedure. However, most patients who undergo such procedures experience major complications and the procedure-related mortality rate is high. We present a unique case of a 61-year-old woman who presented with a ruptured type B aortic dissection 3 weeks after repair of a type A aortic dissection. She underwent an emergent thoracotomy and primary repair of the ruptured aorta followed by concomitant arch debranching and thoracic stent graft placement. Simultaneous surgical debranching with a median sternotomy and endovascular repair with stent grafts is an attractive hybrid approach in patients who present with an acute rupture of a false lumen aneurysm soon after initial repair of an aortic dissection, a situation in which a conventional repair is not feasible. This report emphasizes that hybrid thoracic stent graft repair should be considered for such high-risk patients in the near future as it offers them relatively lower morbidity and mortality compared with what is seen with conventional repairs.