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The article presents several case studies related to orthodontics, which were submitted for the award Orthologic/'A' Company Prize in 1977. In first case a 13 year girl having saggital discrepancy and crowding of teeth in labial segment was treated by removal of four teeth and use of modified Twin Block functional appliance. In another case a 14 year old female was treated for transverse discrepancy and crowding of teeth by rapid maxillary expansion (RME) and teeth extraction.

Context: Limited computed tomography with rectal contrast (CTRC) has been shown to be 98% accurate in the diagnosis of appendicitis in the adult population, but data are lacking regarding the accuracy and effectiveness of this technique in diagnosing pediatric appendicitis.Objective: To determine the diagnostic value of a protocol involving ultrasonography and CTRC in the diagnosis and management of appendicitis in children and adolescents.Design, Setting, and Participants: Prospective cohort study of 139 children and adolescents aged 3 to 21 years (2 patients were older than 18 years) who had equivocal clinical findings for acute appendicitis and who presented to the emergency department of a large, urban, pediatric teaching hospital between July and December 1998. Interventions Children were first evaluated with pelvic ultrasonography. If the result was definitive for appendicitis, laparotomy was performed; if ultrasonography was negative or inconclusive, CTRC was obtained. Patients who did not undergo laparotomy had telephone follow-up at 2 weeks and medical records of all patients were reviewed 4 to 6 months after study completion.Main Outcome Measures: Specificity, sensitivity, positive predictive value, negative predictive value, and accuracy of tests based on final diagnoses; surgeons' estimated likelihood of appendicitis on a scale of 1 to 10 for each case and their case management plans before imaging, after ultrasonography, and after CTRC.Results: A total of 108 patients underwent both ultrasonography and CTRC examinations. The protocol had a sensitivity of 94%, specificity of 94%, positive predictive value of 90%, negative predictive value of 97%, and accuracy of 94%. A normal appendix was identified by ultrasonography in 2 (2.4%) of 83 patients without appendicitis and by CTRC in 62 (84%) of 74 patients. A negative ultrasonography result did not change the surgeons' clinical confidence level in excluding appendicitis (P= .06), while a negative CTRC result did have a significant effect (P<.001). Positive results obtained for either ultrasonography or CTRC significantly affected surgeons' estimated likelihood of appendicitis (P=.001 and P<.001, respectively). Ultrasonography resulted in a beneficial change in patient management in 26 (18.7%) of 139 children while CTRC correctly changed management in 79 (73.1%) of 108.Conclusions: These data show that CTRC following a negative or indeterminate ultrasonography result is highly accurate in the diagnosis of appendicitis in children. [ABSTRACT FROM AUTHOR]

The authors review their experience with transrectal drainage of pelvic abscesses in seven children and adolescents (6-16 years old). Initial access was guided by transrectal ultrasound with an endovaginal transducer followed by fluoroscopy for placement of self-retaining catheters (8.5-10 F) using the Seldinger technique. All abscesses were successfully drained without complications. A transrectal catheter was well tolerated by most patients without spontaneous dislodgment. Catheters were removed after an average of 4 days (range 3-7 days). Transrectal drainage of pelvic abscess with transrectal sonographic and fluoroscopic guidance seems to be a safe and effective procedure in children. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this report is to detail the nutritional sequelae seen in survivors of congenital diaphragmatic hernia (CDH) followed in a multidisciplinary clinic., Methods: Data on 121 surviving CDH patients seen between 1990 and 2000 were collected. Regression analysis was used to determine the impact of factors such as Apgar score, birth weight, extracorporeal membrane oxygenation (ECMO), and patch repair on outcomes associated with nutritional morbidity., Results: There were 100 left and 21 right CDH defects. Mean birth weight and 5-minute Apgar score were 3.1 kg (+/-0.8) and 6.8(+/-2), respectively. Extracorporeal membrane oxygenation was required in 43 (36%) patients and patch repair in 39 (32%). A gastrostomy was required in 39 (32%) patients and a fundoplication in 23 (19%) patients. The side of the defect did not affect the frequency of these procedures. Fifty-six percent of patients were below the 25th percentile for weight during most of their first year. Regression analysis found that duration of ventilation (P <.001) and the presence of a patch repair (P =.03) were independent variables predictive of failure to thrive thereby requiring a gastrostomy tube. Patch repair also was predictive of need for subsequent fundoplication caused by gastroesophageal reflux (P <.001). Twenty-nine patients (24%) had severe oral aversion. Risk factors were prolonged ventilation (P =.001) and oxygen requirement at discharge (P =.015). Two thirds of these patients subsequently improved., Conclusions: Nutritional problems continue to be a source of morbidity for survivors of CDH, particularly in the first year of life. Not surprisingly, patients who had prolonged intubation and prosthetic material at the gastroesophageal junction fared worse. Despite aggressive nutritional management, 56% of the population remained below the twenty-fifth percentile for weight. These data show the need for careful nutritional assessment in all CDH patients, especially those at high risk for malnutrition., (Copyright 2001 by W.B. Saunders Company.)

Objective: To determine correlates of clinical outcomes in patients with short bowel syndrome (SBS)., Methods: Retrospective medical record review of neonates treated between 1986 and 1998 who met our criteria for SBS: dependence on parenteral nutrition (PN) for at least 90 days after surgical therapy for congenital or acquired intestinal diseases., Results: Thirty subjects with complete data were identified; 13 (43%) had necrotizing enterocolitis, and 17 (57%)had intestinal malformations. Mean (SD) residual small bowel length was 83 (67) cm. Enteral feeding with breastmilk (r = -0.821) or an amino acid-based formula (r = -0.793) was associated with a shorter duration of PN, as were longer residual small bowel length (r = -0.475) and percentage of calories received enterally at 6 weeks after surgery(r = -0.527). Shorter time without diverting ileostomy or colostomy (r = 0.400), enteral feeding with a protein hydrolysate formula (r = -0.476), and percentage of calories received enterally at 6 weeks after surgery (r = -0.504) were associated with a lower peak direct bilirubin concentration. Presence of an intact ileocecal valve and frequency of catheter-related infections were not significantly correlated with duration of PN. In multivariate analysis, only residual small bowel length was a significant independent predictor of duration of PN, and only less time with a diverting ostomy was an independent predictor of peak direct bilirubin concentration., Conclusions: Although residual small bowel length remains an important predictor of duration of PN use in infants with SBS, other factors, such as use of breast milk or amino acid-based formula, may also play a role in intestinal adaptation. In addition, prompt restoration of intestinal continuity is associated with lowered risk of cholestatic liver disease. Early enteral feeding after surgery is associated both with reduced duration of PN and less cholestasis.

Background/purpose: In 1990, the authors began a multidisciplinary follow-up clinic for congenital diaphragmatic hernia (CDH) patients. Although the nonpulmonary complications associated with CDH have been reported previously from this clinic, the purpose of this report is to detail the pulmonary outcome in survivors of CDH with severe pulmonary hypoplasia., Methods: Between 1990 and 1999, one hundred patients were seen in the clinic. Before hospital discharge, all patients had baseline tests performed, which were repeated per protocol at clinic during follow-up. The data were analyzed by regression analysis to identify and determine the impact of factors on outcomes associated with the long-term pulmonary morbidity., Results: The average birth weight was 3.16 kg (+/-0.7) with a mean Apgar score of 7 (+/- 2) at 5 minutes. Forty-one patients had an antenatal diagnosis performed. Extracorporeal membrane oxygenation (ECMO) was utilized in 29 patients, and a patch repair was required in 32, whereas 16 patients received both. Average time to extubation was 20.7 (+/- 20) days and mean time to discharge was 59.7 (+/- 61) days. Regression analysis showed that both the need for ECMO and a patch repair were independent predictors of delay in extubation (P <. 001, R(2) = 36%), and delay in discharge from the hospital (P =.001, R(2) = 29%). ECMO also was significantly correlated with the need for diuretics at discharge (P <.001, R(2) = 18%), and with the presence of left-right mismatch (P =.009, R(2) = 9%) and V/Q mismatch (P =.005, R(2) = 11%) on subsequent pulmonary ventilation-perfusion examinations. Sixteen patients required O(2) at discharge, and diuretics were necessary in 43 patients. Seventeen patients at discharge required bronchodilators, and during the first year an additional 36 required at least transient therapy. Similarly, 6 patients at discharge required steroids, and an additional 35 patients required at least transient therapy during the first year. Chest x-rays, although frequently abnormal, had little correlation with clinical outcome, but did influence medical therapy. V/Q scans had limited utility in patient management, and the presence of V/Q mismatch was not highly specific for future obstructive airway disease. Nevertheless, V/Q mismatch was sensitive for obstructive airway disease assessed by spirometry. Twenty-five patients over 5 years of age performed pulmonary function tests (PFTs), which showed 72% normal PFT results and 28% with evidence of obstructive airway disease. Before January 1997, 2 of 8 patients who required urgent treatment in the emergency department (ED) were admitted to the intensive care unit (ICU) secondary to acute respiratory distress. After the implementation of respiratory syncytial viral prophylaxis in January 1997, 8 patients were treated in the ED for acute respiratory distress, but none required admission to the ICU., Conclusions: Pulmonary problems continue to be a source of morbidity for survivors of CDH long after discharge. The need for ECMO and the presence of a patch repair are both predictive of more significant morbidity, but the data clearly show that non-ECMO CDH survivors also require frequent attention to pulmonary issues beyond the neonatal period. These data show the need for long-term follow-up of CDH patients preferably with a multidisciplinary team approach.

Purpose: The aim of this study was to evaluate the outcomes of reconstruction of all cloacal exstrophy cases seen by the authors from 1974 to 1999., Methods: A retrospective chart review and personal follow-up was conducted on all 50 patients, who ranged in age from newborn to 35 years. Thirty-eight were secondary cases, 12 were primary (no previous surgery). Twenty-eight (56%) were genetic boys; 6 were raised as boys and 22 as girls. Forty patients underwent extensive reconstructive surgery; 6 await reconstruction, and 4 were seen only in consultation. For urinary continence, 21 had bladder neck narrowing, 7 received a bowel nipple, and 12 had a Mitrofanoff catheterizable conduit on the abdominal wall, using tapered intestine in 8, ureter in 3 and the appendix in 1. Four patients had a urostomy. Bladder augmentation was performed in 35 patients (18 with stomach, 11 small bowel, and 6 with both). Twenty-five patients had pull-through of their colon to the perineum. Thirty-two have had a vagina constructed. Forty-seven of the 50 patients had spinal cord tethering, and most underwent neurosurgical release., Results: Overall survival rate was 98%; 1 patient died preoperatively at another institution. Acceptable bowel continence was achieved with enema washouts in 19 of 25 pullthroughs; 4 failed and were reversed. One case is too recent to judge. Of 40 reconstructed cases, 31 were dry, 3 had slight leakage, and 1 is too early to evaluate. Five had enough leakage to require further surgery., Conclusions: The once hopeless anomaly of cloacal exstrophy is treatable with extensive reconstructive surgery. Continence of urine (mainly by catheterization) and stool (mainly by enema washouts) is achievable in most. The hindgut, including the cecum and the usually rudimentary distal colon, should be saved and used as colon, not for urinary or vaginal reconstruction. In genetic boys, the authors continue to believe that gender assignment should depend on the likelihood for reconstructing an adequate phallus.

A retrospective chart review of children who had airway foreign body removed via direct laryngoscopy and bronchoscopy (DLB) from 1987-1997 was conducted in Children's Hospital, Boston. Patient characteristics noted included age, sex, and clinical presentation. Pre-operative radiographic findings, reason for delay in evaluation, DLB findings, length of procedure, reason for repeat DLB, and types of foreign body etc. were recorded. Serious complications from aspirated foreign bodies such as severe airway obstruction and death tend to occur in infants and younger children because of their small airway size. A history compatible with foreign body aspiration dictates diagnostic endoscopy with or without radiologic confirmation. Chest and airway radiographs supplemented by fluoroscopy can increase the ratio of correct and early diagnosis. Fluoroscopy should be universally accepted as an initial diagnostic technique in airway foreign body evaluation. Fluoroscopy is not a worthwhile investigation if a preceeding chest radiograph suggests the presence of a foreign body. Long-standing airway foreign bodies are associated with considerable morbidity, and early diagnosis remains the key to successful and uncomplicated management of foreign body aspiration. Education aimed at increasing diagnostic acumen of the physicians and heightening of public awareness are the most important steps needed to reduce the morbidity and mortality. Parents should be instructed to abstain from feeding nuts and seeds to young children and to keep small, potentially ingestible objects out of their reach.

Context: Limited computed tomography with rectal contrast (CTRC) has been shown to be 98% accurate in the diagnosis of appendicitis in the adult population, but data are lacking regarding the accuracy and effectiveness of this technique in diagnosing pediatric appendicitis., Objective: To determine the diagnostic value of a protocol involving ultrasonography and CTRC in the diagnosis and management of appendicitis in children and adolescents., Design, Setting, and Participants: Prospective cohort study of 139 children and adolescents aged 3 to 21 years (2 patients were older than 18 years) who had equivocal clinical findings for acute appendicitis and who presented to the emergency department of a large, urban, pediatric teaching hospital between July and December 1998. Interventions Children were first evaluated with pelvic ultrasonography. If the result was definitive for appendicitis, laparotomy was performed; if ultrasonography was negative or inconclusive, CTRC was obtained. Patients who did not undergo laparotomy had telephone follow-up at 2 weeks and medical records of all patients were reviewed 4 to 6 months after study completion., Main Outcome Measures: Specificity, sensitivity, positive predictive value, negative predictive value, and accuracy of tests based on final diagnoses; surgeons' estimated likelihood of appendicitis on a scale of 1 to 10 for each case and their case management plans before imaging, after ultrasonography, and after CTRC., Results: A total of 108 patients underwent both ultrasonography and CTRC examinations. The protocol had a sensitivity of 94%, specificity of 94%, positive predictive value of 90%, negative predictive value of 97%, and accuracy of 94%. A normal appendix was identified by ultrasonography in 2 (2.4%) of 83 patients without appendicitis and by CTRC in 62 (84%) of 74 patients. A negative ultrasonography result did not change the surgeons' clinical confidence level in excluding appendicitis (P= .06), while a negative CTRC result did have a significant effect (P<.001). Positive results obtained for either ultrasonography or CTRC significantly affected surgeons' estimated likelihood of appendicitis (P=.001 and P<.001, respectively). Ultrasonography resulted in a beneficial change in patient management in 26 (18.7%) of 139 children while CTRC correctly changed management in 79 (73.1%) of 108., Conclusions: These data show that CTRC following a negative or indeterminate ultrasonography result is highly accurate in the diagnosis of appendicitis in children.

Objective: Children evaluated in the emergency department for possible appendicitis are often admitted for observation, despite the widespread availability of accurate diagnostic studies, particularly computed tomography (CT). We sought to establish effective and efficient strategies for using CT to diagnose and manage children with possible appendicitis., Design: Retrospective chart review and decision analysis. Setting. Emergency department of a large, urban tertiary care pediatric teaching hospital., Patients: All patients admitted from January 1996 to August 1997 for suspected appendicitis. METHOD OF ANALYSIS: Three modeled strategies were empirically applied to the retrospective cohort of patients admitted for observation. Outcomes and costs under the modeled strategies were compared with those under current practice. The three strategies were: 1) to obtain CT scans on all patients and discharge those with normal findings; 2) to obtain CT scans and admit all patients; 3) to selectively obtain CT scans on those patients with a peripheral white blood cell count >10 000/mm(3) (10 x 10(9)/L) and admit all. The sensitivity and specificity of CT for diagnosing appendicitis were determined empirically from the data. A sensitivity analysis was performed., Main Outcome Measures: The number of preoperative inpatient observation days, total hospital costs, and the rates of both missed appendicitis and negative laparotomies., Results: Of 609 patients hospitalized for possible appendicitis, 287 went directly to the operating room and 14 patients had known perforation and abscess. Three hundred eight children were observed and comprised the study cohort. Of the cohort, 112 (36.4%) underwent appendectomy and 26 (23.2%) of these had a normal appendix at pathology. Three patients were discharged from the hospital after observation and were subsequently readmitted with appendicitis (missed appendicitis). Among the 75 patients who had CT performed, the sensitivity and specificity of CT were both 97%. Under the current practice strategy, the cohort collectively accumulated 487 inpatient observation days and incurred a per patient cost of $5831. All three CT strategies would have reduced the total number of inpatient observation days, operations, negative laparotomies, as well as the per patient cost. The strategy of obtaining CT scans on all patients and then admitting them had the lowest rate of missed appendicitis. The additional cost of preventing each case of missed appendicitis under this strategy compared with the strategy of obtaining CT scans and sending home those with negative findings was $150,304. Even at the lowest reported sensitivity and specificity of CT in the literature, the ordering of the three strategies remained constant and continued to reduce total cost per patient., Conclusion: Compared with current practice, diagnostic strategies using CT could reduce costs and improve diagnosis, management, and outcomes for children with appendicitis.

Purpose: To evaluate the effect of computed tomographic (CT) information on diagnostic confidence and initial clinical treatment in children with abdominal trauma., Materials and Methods: Senior surgical staff completed questionnaires before and after abdominal CT was performed in 138 consecutive children with acute abdominal trauma seen between April 1996 and April 1997. Physicians were asked to estimate the probability of underlying abdominal injury, which organ was injured, their level of confidence in the CT findings, and initial clinical management plans. The gain in percentage diagnostic confidence and the proportion of children in whom CT information changed diagnoses and initial management plans were evaluated., Results: The CT findings changed the surgeons' initial diagnoses in 116 (84%) patients (95% confidence interval [CI] = 75%, 86%). The mean gain in diagnostic certainty with CT was 36% (95% CI = 31%, 40%). Initial management plans changed in 61 (44%) patients after CT information was available (95% CI = 35%, 50%). This resulted in decreased intensity of care in 52 (38%) patients and increased intensity of care in nine (6.5%)., Conclusion: Abdominal CT had a strong effect on surgeons' clinical diagnoses and initial treatment plans in children with blunt trauma. CT information enabled surgeons to safely reduce the intensity of care provided to injured children.

Caustic injury to the upper aerodigestive system with scarring of the pharynx, hypopharynx, and esophagus is a challenging reconstructive problem. The authors report on seven patients who required total esophageal replacement from the pharynx to the stomach. Injury occurred from alkali in six and acid in one. Age at injury ranged from 14 months to 14 years (mean, 4.5 years.) in five boys and two girls. Time from injury to esophageal replacement was 6 months to 10 years (mean, 3.5 years). Two required pharyngeal reconstruction before and one after esophageal replacement. Six patients had an isoperistaltic right or transverse colon interposition. One who had gastric necrosis had an ileo-right colonic substernal interposition with creation of a jejunal reservoir. Results of barium swallows showed intact anastomoses in all patients. There were no leaks. Most had some degree of mild to moderate aspiration, and one who had left vocal cord paralysis had initially massive aspiration. Three patients currently eat regular diets; four eat but still require supplemental tube feeds. The authors conclude that children who have hypopharyngeal scarring and obliterated esophageal inlet can undergo a successful colonic esophageal replacement with high proximal pharyngocolic anastomosis.

Infants with congenital diaphragmatic hernia (CDH) show a wide range of anatomic and physiological abnormalities, making it difficult to compare the efficacy of management protocols between institutions. The purpose of this study was twofold: (1) to analyze the results of treatment of CDH in a large tertiary care pediatric center using conventional mechanical ventilation (CMV) with extracorporeal membrane oxygenation (ECMO) as rescue therapy, and (2) to compare these results with those of a parallel study by a similar large urban center that used high-frequency oscillating ventilation (HFOV) as rescue therapy without ECMO. All patients who had CDH diagnosed within the first 12 hours of life and were referred for treatment before repair (between 1981 and 1994) were included in the analysis (n = 196). CMV was used initially in all patients, with conversion to ECMO for refractory hypoxemia or hypercapnea. Between 1981 and 1984, ECMO was not available. Between 1984 and 1987, ECMO was offered postoperatively. Between 1987 and 1991, ECMO was offered preoperatively. In all three groups, aggressive hyperventilation and alkalosis was the norm. Since 1991, permissive hypercapnia has been used. HFOV was used in three patients as stand-alone therapy with one survivor. Twenty patients died without repair: Ten had other lethal anomalies, eight died before ECMO could be instituted, and two died of ECMO-related complications. Overall, 104 patients (53%) survived and 92 (47%) died. Ninety-eight patients (50%) received ECMO, and 43 (44%) survived. Survivors had significantly higher 1- and 5-minute Apgar scores and higher postductal Po2s than did nonsurvivors. Associated anomalies were present in 39%, who had a significantly lower survival than those with isolated CDH. Antenatal diagnosis and side of the defect had no impact on outcome. Survival was not improved with the institution of ECMO or delayed repair but rose significantly to 69% (84% with isolated CDH, P = .007) with the introduction of permissive hypercapnea. Autopsy results from nonsurvivors showed other lethal anomalies and significant barotrauma as the primary causes of death. Comparisons between the Boston and Toronto series showed similar patient demographics and no significant differences in survival in any time period. The two series differed in the number of associated anomalies, their impact on survival, and in the prognosis of right-sided CDH. From the individual and combined analyses the authors concluded: (1) CMV with ECMO as rescue produced an overall survival in CDH patients equivalent to CMV with HFOV in a parallel series, (2) neither HFOV nor ECMO has significantly improved outcome in CDH patients, (3) institution of permissive hypercapnia has resulted in a significant increase in survival, and (4) the leading causes of death in CDH patients appear to be associated anomalies and pulmonary hypoplasia, which are currently untreatable. Barotrauma, which may contribute in up to 25% of deaths in CDH patients is avoidable.

CDH patients are critically ill but can expect excellent survival if other serious anomalies are not present; however, significant long-term morbidities exist in the survivors. The most notable of these are developmental delay, poor growth, gastroesophageal reflux disease, hearing loss, and musculoskeletal abnormalities. Patients require careful long-term follow-up for these problems, and caregivers must continue to critically evaluate their medical interventions in the neonatal period to try to minimize these morbidities.

Objective: Infantile hemangioma and arteriovenous malformation (AVM) of the liver have a similar presentation but a different natural history, and therefore require different treatment. This study was undertaken to clarify differential diagnosis and management of these two biologically distinct vascular disorders., Study Design: We retrospectively analyzed the records of 43 children with hepatic vascular anomalies treated during the past 27 years., Results: Ninety percent were hemangiomas (n = 39); 10% were AVM (n = 4). Infants with AVM or large solitary hemangioma had hepatomegaly, congestive heart failure, and anemia as presenting symptoms at birth. Multiple hepatic hemangiomas manifested at 1 to 16 weeks of age with the same clinical triad, plus multiple cutaneous lesions (19/23). The mortality rate after treatment of hepatic AVM was 50% (2/4). The mortality rates after treatment of liver hemangiomas were as follows: resection of solitary lesions, 20% (2/10); embolization, 43% (3/7); corticosteroids, 30% (3/10); and interferon alfa-2a, 15% (2/13)., Conclusion: Solitary hepatic hemangioma cannot always be distinguished from hepatic AVM without radiologic studies. Multiple hepatic hemangiomas are differentiated from hepatic AVM by coexistence of multiple cutaneous hemangioma and by radiologic imaging. We recommend combined embolization and surgical resection for hepatic AVM and for solitary symptomatic hemangioma, if drug therapy fails. Pharmacologic treatment is used for symptomatic multiple liver hemangiomas. Embolization allows interim control of heart failure. A decreased mortality rate after interferon alfa-2a therapy is encouraging.

A 33-week-gestation infant with a massive sacrococcygeal teratoma weighted 4,000 g, but the actual weight of the infant was approximately 1,500 g. With the potential for massive blood loss and impaired lung compliance during resection, some type of cardiopulmonary support was necessary. Resection was undertaken with the assistance of venoarterial extracorporeal membrane oxygenation (ECMO) and hypothermic hypoperfusion. Immediately after removal of the tumor, which weighted 2,420 g, the infant was decannulated from ECMO, and the carotid artery was primarily reconstructed end-to-end. The amount of intraoperative blood loss was 550 mL Postoperatively, the child weighted 1,580 g. Follow-up head ultrasound results were normal, and the patient has done well. This is the first reported case in which ECMO with hypothermic hypoperfusion was used for resection of a massive tumor. This experience shows that ECMO is both useful and safe as a means of temporary cardiopulmonary support for resection of massive tumors in infants.

The authors report on two infants who had large congenital fibrosarcomas that initially were believed to be hemangiomas. Although hemangioma and congenital fibrosarcoma can have a similar presentation, their treatment is dissimilar. The authors review the anatomic findings, hematologic differences, and radiological clues that can help to differentiate congenital fibrosarcoma from congenital hemangioma.

The purpose of this clinical report is to discuss the anaesthetic management of a patient with end-stage cardiomyopathy. An eight-year-old boy sustained electro-mechanical dissociation following a "standard anaesthetic induction" but was subsequently successfully anaesthetized using a combination of etomidate, midazolam, lidocaine, vecuronium and glycopyrrolate. Anaesthetic techniques and their relationship to the specific category of cardiomyopathy (dilated, hypertrophic or restrictive) are discussed. The potential detrimental effects of anaesthetic sympatholysis, myocardial depression and negative chronotropy on cardiac output are explained.

Background: Resection is the accepted management of a choledochal cyst. However, the debate continues regarding the optimal method of biliary reconstruction. The Roux-en-Y limb is used most frequently, but concerns have been raised about this method due to associated peptic ulcer disease, cholangitis, and poor growth. A method of reconstruction using an interposed segment of jejunum with a nipple valve placed between the common bile duct and the duodenum has been proposed., Study Design: We have reviewed a series of 12 children requiring biliary reconstruction for choledochal cyst (11 children) and biliary stricture (one child). All had reconstruction with a nipple valve, and ten had an interposed segment of jejunum., Results: All of the children are alive and have had follow-up evaluation from six months to 8.5 years (median of three years). Sequential examinations with ultrasound and biliary excretion scans have shown no evidence of obstruction, and liver function tests have remained normal. Three children have had cholangitis. One child had a brief episode in the perioperative period. The second child had cholangitis 16 months postoperatively, and the third child had multiple episodes of cholangitis. These latter two children were unique. One had Alonso-Lej type IV choledochal cyst with intrahepatic dilatation, which persisted after reconstruction. The other had a prior diversion with a Roux-en-Y limb from the gallbladder after resection of a choledochal cyst and had multiple episodes of cholangitis before reconstruction. These episodes are now controlled with chronic antibiotic suppression. Postoperative complications were limited to two episodes of obstruction of the small bowel requiring lysis of adhesions. No child has had peptic ulcer disease. These children have grown well after reconstruction, except for three with multiple anomalies or chronic pancreatitis., Conclusions: Biliary reconstruction with a jejunal interposition containing a nipple valve can be performed safely with a low incidence of complications. It offers a more physiologic method of reconstruction and a low incidence of postoperative cholangitis.

Perforated appendicitis in children continues to be associated with significant morbidity. In 1976, a treatment algorithm was begun at the authors' institution, which included immediate appendectomy, antibiotic irrigation of the peritoneal cavity, transperitoneal drainage through the wound, and 10-day treatment with intravenous ampicillin, clindamycin, and gentamicin. Initial results with this scheme in 143 patients demonstrated a 7.7% incidence of major complications and no deaths. From 1981 through 1991, the authors continued to use this treatment plan in all patients with perforated appendicitis. Three hundred seventy-three patients with perforated appendicitis were treated, and the rate of major complications was 6.4%. Infectious complications occurred in 18 patients (4.8%) and included intraabdominal abscesses (5 patients, 1.3%), phlegmon treated with an extended course of antibiotics (6 patients, 1.6%), wound infections (5 patients, 1.3%), and enterocutaneous fistula requiring further operations (2 patients, 0.5%). There were six cases of small bowel obstruction (1.6%), which required operative intervention. There were no deaths. The average length of stay for all patients was 11.4 days (range, 8 to 66 days). Utilization of transperitoneal drainage and choice of antibiotic therapy continue to be sources of controversy in the surgical literature. However, the treatment plan used in the present study resulted in the lowest complication rate reported to date, and the authors conclude that this scheme is truly the "gold standard" for treatment of perforated appendicitis. New treatment plans using laparoscopic appendectomy, different or shorter courses of antibiotics, or not using drains should have complication rates that are as low as, or lower than this one to be considered as useful alternatives.

With the increase in popularity of delayed repair of congenital diaphragmatic hernia (CDH), many institutions are using extracorporeal membrane oxygenation (ECMO) to stabilize patients preoperatively. This practice has led to controversy regarding whether the repair should be performed while the patient is on ECMO or after decannulation. This report details the authors' experience with repair of CDH on ECMO. Of the 154 high-risk CDH patients treated at Children's Hospital, Boston, MA, since ECMO became available (1984), 97 received ECMO, including 31 who had repair performed while on ECMO. In group I (nine patients), repair was carried out only if the patients were unweanable from ECMO after 7 days. Activated clotting times (ACT) were maintained at 200 to 220 seconds. In group II (22 patients), repair was performed on ECMO electively, before decannulation. ACT were maintained at 180 to 200 seconds. Additionally, all patients in group II received aminocaproic acid before surgery. This was administered continuously for 72 hours postoperatively or until decannulation. Patients in group II had significantly less overall blood loss (P = .02), and lower transfusion requirement (P = .0003) than those in group I. Additionally, four of the nine patients in group I required reexploration because of hemorrhage; this was not required for any patient in group II (P = .005). Although the survival rates differed, this may have been because of a bias in patient selection between the two groups. From these preliminary data, the authors conclude that repair of congenital diaphragmatic hernia on ECMO can be performed safely, with a minimum of hemorrhagic complications.(ABSTRACT TRUNCATED AT 250 WORDS)

It has been reported previously that infants diagnosed with congenital diaphragmatic hernia (CDH) antenatally have a much poorer prognosis than those diagnosed postnatally. The authors identified 173 high-risk (symptomatic within the first 6 hours of life) infants with CDH treated in the past decade. Seventy-seven cases were diagnosed antenatally and 96 were diagnosed postnatally. The survival rate was slightly worse for the antenatal group (34% v 48% for the postnatal group; P = .04). However, 59 of the 173 patients (34%) had other life-threatening congenital anomalies. Among the 114 patients with isolated CDH, the survival rate increased to 59% in the antenatal group and 63% in the postnatal group--a difference that was not significant. The timing of antenatal diagnosis (> or < 25 weeks) had no impact on the statistics (P = .08). The only parameter consistently different between all groups and subgroups was the Apgar score at 5 minutes, which was always better in the antenatal group (P < .02), possibly reflecting more efficient resuscitation. Of the 59 patients with other life-threatening anomalies (42 cardiac), there was one survivor among the 34 in the antenatal group and only two among the 25 in the postnatal group, despite the fact that 39 patients had undergone resuscitation, which included ECMO in 25.(ABSTRACT TRUNCATED AT 250 WORDS)

It is often thought that survivors of congenital diaphragmatic hernia (CDH) have an isolated problem related to lung hypoplasia, and little data exist regarding the extrapulmonary problems of high-risk CDH patients who do survive. In 1990, the authors began a multidisciplinary follow-up clinic for CDH patients. Members of the program include representatives from the departments of surgery, pulmonary medicine, development, nursing, and nutrition. Since this program began, the authors have followed up on 33 infants who survived after treatment of high-risk CDH, ie, those who were symptomatic within 6 hours of birth. Twenty patients were treated with extracorporeal membrane oxygenation (ECMO). Neurological problems were common in these patients: seven children (21%) required hearing aids, and seven others had abnormal results with brain-stem auditory evoked response (BAER) testing. Extraaxial fluid collections or enlarged ventricles were present on head computed tomography scans of 10 children, and four children had clinical seizure activity. Fifteen patients had developmental delays, which improved rapidly once the children began to thrive. Six patients required eyeglasses or had strabismus, and one patient is congenitally blind. There were a variety of problems related to growth and nutrition, with six patients needing fundoplications, and 13 patients below the fifth percentile for weight. Of 10 patients with patch repairs, two had recurrent hernias. Six others required surgery for bowel obstruction. Eleven patients had pectus excavatum, usually mild, and four had mild to moderate degrees of scoliosis. There were undescended testicles in five boys, vesicoureteral reflux in two patients, and kidney stones in two patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Exstrophy of the cloaca, a rare anomaly, is among the most complex malformations of infancy. Features include omphalocele, imperforate anus, and exstrophy of two hemibladders, between which lies the everted cecum. A small colon ends blindly in the pelvis, and the terminal ileum often prolapses out of the exposed cecum. Formerly, most of these infants died. From 1974 to 1992, 20 of these patients were treated, the majority in the past decade. Six were managed primarily; 14 were referred after prior surgery elsewhere. Current treatment of a newborn with cloacal exstrophy includes closure of the omphalocele, separation of the gastro-intestinal tract from the hemibladders, and closure of the two hemibladders as a single viscus. The colon can be pulled through in some cases, either immediately or later (6 cases). The bladder must be augmented in all cases to provide adequate volume and compliance; stomach is ideal for this (10 cases). A bladder continence mechanism must be constructed by narrowing the outlet (9 cases), or inserting a reversed small bowel nipple (7 cases). Intermittent catheterization is needed for the patient to empty the reconstructed bladder. Although two thirds of these patients are genetic males, they should be raised as females because they lack adequate tissue to construct a phallus. Magnetic resonance imaging of the spine has shown tethering of the cord in all patients; most were released neurosurgically. Lessons provided by these cases demonstrate that a satisfactory surgical and social outcome can be achieved today in most children with cloacal exstrophy.

It has been suggested that delayed repair with preoperative stabilization might improve survival in high-risk (symptomatic within 6 hours of birth) congenital diaphragmatic hernia (CDH). This study compares the results of immediate operation versus delayed repair using extracorporeal membrane oxygenation (ECMO) when necessary. Since we first used ECMO in 1984, 101 high-risk CDH infants have been treated. Prior to 1987, we used immediate repair and postoperative ECMO if necessary. Between 1987 and 1990 we combined delayed operation (24 to 36 hours) with preoperative ECMO as necessary. No infant in this series was excluded from ECMO therapy unless absolute contraindications existed (prematurity, intracranial hemorrhage, or other major anomalies). Fifty-five patients received immediate operation and 46 had delayed repair. The two groups were comparable populations based on gestational age, birth weight, age at onset of symptoms, Apgar scores, best postductal PO2 (BPDPO2), and frequency of antenatal diagnosis. There was no statistically significant difference in overall survival between the two groups. Differences in survival among subpopulations (BPDPO2 greater than 100 or less than 100, antenatal diagnosis, inborn v outborn) also are not significant. The requirement for ECMO was similar in both groups. Survivors in the delayed repair group were ventilated longer and on ECMO longer, but had fewer late deaths (greater than 21 days) and fewer pulmonary sequelae (O2 dependency at discharge) than infants in the immediate repair group (P less than .05).(ABSTRACT TRUNCATED AT 250 WORDS)

Infants with congenital diaphragmatic hernia (CDH) demonstrate a wide range of anatomic and physiologic abnormalities, making it difficult to compare the efficacy of new forms of therapy such as extracorporeal membrane oxygenation (ECMO) among institutions. This study was undertaken to determine whether any predictors of severity could be identified in the ECMO era. The charts of all patients with CDH treated at this institution since 1984, when ECMO became available. (n = 110), were reviewed. Infants were considered high risk and included in this study if they presented with respiratory distress within the first 6 hours of life (n = 94). In order to focus on predictors of pulmonary insufficiency, patients who died of nonpulmonary causes or had other significant congenital anomalies were excluded from this review, leaving 59 patients for analysis. All the infants during this period had intensive pharmacological and ventilatory support. When needed, ECMO was offered postoperatively from 1984 to 1987, and preoperatively from 1987 to the present. Forty-five of 59 had a best postductal PO2 (BPDPO2) greater than 100 mm Hg, and 41 of these responders survived (91%). Fourteen patients had a BPDPO2 less than 100 mm Hg and only one survived (7%) (P = .0001). Mean BPDPO2 between survivors with or without ECMO, and nonsurvivors were also significantly different (P = .001). To incorporate ventilatory information, an oxygenation/ventilation index was devised: [OVI = PO2/(mean airway pressure x respiratory rate) x 100]. Differences in OVI between these three groups were also significant. When analyzing the data by the method proposed by Bohn (PCO2 v VI), no correlation between ventilatory parameters and outcome was found.(ABSTRACT TRUNCATED AT 250 WORDS)

We have previously shown that angiogenesis inhibition and tumor regression can be accomplished by combinations of heparin or heparin fragments with cortisone [Folkman, J., Langer, R., Linhardt, R. J., Haudenschild, C. & Taylor, S. (1983) Science 221, 719-725]. Oral heparin was also effective in combination with cortisone. We now show that a single oral dose of [35S]heparin or [3H]heparin (15,000 units/kg) results in continuous release of radioactive material into the bloodstream for at least 12 hr. This is associated with the presence of anti-factor Xa activity at a level of approximately equal to 0.1 unit/ml. The radioactive material is identified as oligo-, di-, and monosaccharides by its behavior in chromatographic systems, its possession of anti-factor Xa activity, and the effect of treatment with bacterial heparinase. The heparin fragments are extensively metabolized to fragments without anti-factor Xa activity that are readily subject to urinary excretion.

Adipose tissue has been used to promote wound healing and to revascularize ischemic myocardium. We explored whether fat from various sources was angiogenic in the cornea. Rabbit subcutaneous and omental fat induced grossly visible neovascularization of all rabbit corneas studied, and at a similar rate and intensity. Neovascularization was not observed in any cornea following control implantation of liver or muscle. Neovascularization was blocked in all rabbits in which indomethacin was administered orally 3 days before implantation of fat and continued following implantation, suggesting that prostaglandins are associated with fat induced angiogenesis.