Dennis P. Lund

Congenital diaphragmatic hernia--a tale of two cities: the Boston experience.

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.

Hepatic vascular anomalies in infancy: A twenty-seven-year experience

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.

Congenital diaphragmatic hernia: the hidden morbidity.

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)

Airway foreign bodies (FB): a 10-year review

A retrospective chart review of children who had airway foreign body removed via direct laryngoscopy and bronchoscopy (DLB) from 1987-1997 was conducted in Childrens 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.

Blue Rubber Bleb Nevus Syndrome: Surgical Eradication of Gastrointestinal Bleeding

Objective:We report the largest clinical experience to date of surgically treated patients with blue rubber bleb nevus syndrome (BRBNS). Summary Background Data:BRBNS is a rare congenital disorder presenting with multifocal venous malformations of the skin, soft tissues, and gastrointestinal (GI) tract. Patients with BRBNS develop anemia from chronic GI bleeding, and require lifelong treatment with iron and blood transfusions. An aggressive surgical approach to treat the GI venous malformations of BRBNS has been considered unlikely to be successful because of the large number of lesions, their position throughout the GI tract, and the likelihood of recurrence. Based on our belief that eradicated lesions would not recur, we undertook the removal of all GI tract lesions in an effort to eliminate bleeding. Methods:Ten patients with BRBNS were treated from 1993 to 2002. Lesions were identified using complete GI endoscopy. The multiple venous malformations were removed by a combination of wedge resection, polypectomy, suture-ligation, segmental bowel resection, and band ligation. Results:Patient ages ranged from 2 to 36 years, and patients received an average of 53 prior blood transfusions. A mean of 137 focal GI venous malformations per patient were resected at operation (range 4–557), with a mean operative duration of 14 hours (range 7–23 hours). Only 1 patient who had a less extensive procedure developed recurrent GI bleeding. The mean follow-up period was 5.0 years (range 2.9–10.3 years). Conclusions:We believe that an aggressive excisional approach is indicated for the venous anomalies that cause GI bleeding in BRBNS.

Effect of Computed Tomography on Patient Management and Costs in Children With Suspected Appendicitis

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/mm3 (10 × 109/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

Delayed repair and preoperative ECMO does not improve survival in high-risk congenital diaphragmatic hernia

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

Congenital fibrosarcoma masquerading as congenital hemangioma: Report of two cases

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.

Congenital diaphragmatic hernia: Predictors of severity in the ECMO era

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)

Cloacal exstrophy: Experience with 20 cases

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.