Kevin P. Lally

Current surgical management of congenital diaphragmatic hernia: A report from the congenital diaphragmatic hernia study group

BACKGROUND Repair of congenital diaphragmatic hernia (CDH) has changed from an emergent procedure to a delayed procedure in the last decade. Many other aspects of management have also evolved since the first successful repair. However, most reports are from single institutions. The lack of a large multicenter database has hampered progress in the management of congenital diaphragmatic hernia (CDH) and makes determination of the current standard difficult. METHODS The CDH study group was formed in 1995 to collect data from multiple institutions in North America, Europe, and Australia. Participating centers completed a registry form on all live-born infants with CDH during 1995 and 1996. Demographic information, data about surgical management, and outcome were collected for all patients. RESULTS Sixty-two centers participated, with 461 patients entered. Overall survival was 280 of 442 patients (63%) where survival was recorded. The defect was left-sided in 78%, right-sided in 21%, and bilateral in 1%. A subcostal approach was used in 91% of patients, with pleural drainage used in 76%. A patch of some kind was used in just over half (51%) of the patients, with polytetrafluoroethylene being the most commonly used material (81%) in those patients with a patch. The mean surgical time was 102 minutes, with an average blood loss of 14 mL (range, 0 to 500 mL). The overwhelming majority of patients underwent repair between 6:00 AM and 6:00 PM (289 of 329, 88%). Nineteen percent of patients had surgical repair on extracorporeal membrane oxygenation (ECMO) at a mean time of 170 hours into the ECMO course (range, 10 to 593 hours). The mean age at surgery in patients not treated with ECMO was 73 hours (range, 1 to 445 hours). CONCLUSIONS The multicenter nature of this report makes it a snapshot of current management. The data would indicate that prosthetic patching of the defect has become common, that after-hours repair is infrequent, and that delayed surgical repair has become the preferred approach in many centers. Furthermore, the mean survival rate of 63% indicates that despite decades of individual effort, the CDH problem is far from solved. This highlights the need for a centralized database and cooperative multicenter studies in the future.

Defect size determines survival in infants with congenital diaphragmatic hernia

OBJECTIVES. Congenital diaphragmatic hernia is a significant cause of neonatal mortality. The objective of this study was to evaluate the clinical factors associated with death in infants with congenital diaphragmatic hernia by using a large multicenter data set. METHODS. This was a prospective cohort study of all liveborn infants with congenital diaphragmatic hernia who were cared for at tertiary referral centers belonging to the Congenital Diaphragmatic Hernia Study Group between 1995 and 2004. Factors thought to influence death included birth weight, Apgar scores, size of defect, and associated anomalies. Survival to hospital discharge, duration of mechanical ventilation, and length of hospital stay were evaluated as end points. RESULTS. A total of 51 centers in 8 countries contributed data on 3062 liveborn infants. The overall survival rate was 69%. Five hundred thirty-eight (18%) patients did not undergo an operation and died. The defect size was the most significant factor that affected outcome; infants with a near absence of the diaphragm had a survival rate of 57% compared with infants having a primary repair with a survival rate of 95%. Infants without agenesis but who required a patch for repair had a survival rate of 79% compared with primary repair. CONCLUSIONS. The size of the diaphragmatic defect seems to be the major factor influencing outcome in infants with congenital diaphragmatic hernia. It is likely that the defect size is a surrogate marker for the degree of pulmonary hypoplasia. Future research efforts should be directed to accurately quantitate the degree of pulmonary hypoplasia or defect size antenatally. Experimental therapies can then be targeted to prospectively identify high-risk patients who are more likely to benefit.

Complications after surgical intervention for necrotizing enterocolitis: A multicenter review

Necrotizing enterocolitis (NEC) is a serious condition affecting predominantly the premature infant. The purpose of this study is to report a multicenter experience of complications in 252 infants requiring surgical therapy for NEC. Data from eight institutions for the years 1980 through 1990 were collected and analyzed for infants undergoing surgical therapy for NEC. Records were reviewed for gestational age, birth weight, age at operation, indications for operation, degree of intestinal involvement, operation(s) performed, complications, and 30-day mortality rates. A total of 264 infants underwent surgical intervention for NEC during the study period. Complete information was available for 252 patients. The mean gestational age was 31 +/- 5 weeks and the mean birth weight was 1,552 +/- 823 g. The mean age at operation was 18 +/- 35 days. Pneumoperitoneum was the most common indication for operation (42%). The 30-day survival rate was 72%. Eighty-one percent of patients underwent primary laparotomy, whereas peritoneal drainage was performed in 48 (19%) patients. Postoperative complications were identified in 119 (47%) patients. The most common postoperative complications were sepsis (9%), intestinal strictures (9%), and short gut (9%). Wound infections occurred in 6%, and the incidence of intraabdominal abscess formation was only 2.3%. Gestational age < 27 weeks (P < .005) and birth weight < 1,000 g (P < .005) were associated with significantly increased mortality but no increase in postoperative morbidity. The incidence of complications was similar in the very low birth weight (< 1,000 g) infants (51%) compared with infants > or = 1,000 g (46%).(ABSTRACT TRUNCATED AT 250 WORDS)

Hyperglycemia and morbidity and mortality in extremely low birth weight infants

Objective:The purpose of this study was to determine the association between hyperglycemia and mortality and late-onset infections (>72 h) in extremely low birth weight (ELBW) infants.Study design:Retrospective analysis of a prospective cohort study of 201 ELBW infants who survived greater than 3 days after birth. Mean morning glucose levels were categorized as normoglycemia (<120 mg/dl), mild-moderate hyperglycemia (120 to 179 mg/dl) and severe hyperglycemia (⩾180 mg/dl). Hyperglycemia was further divided into early (first 3 days of age) and persistent (first week of age). Logistic regression was performed to assess whether hyperglycemia was associated with either mortality or late-onset culture-proven infection, measured after 3 and 7 days of age.Results:Adjusting for age, the odds ratio (OR) for either dying or developing a late infection was 5.07 (95% confidence interval (CI): 1.06 to 24.3) for infants with early severe hyperglycemia and 6.26 (95% CI: 0.73 to 54.0) for infants with persistent severe hyperglycemia. Adjusting for age, both severe early and persistent hyperglycemia were associated with increased mortality. Among survivors, there was no significant association between hyperglycemia and length of mechanical ventilation or length of hospital stay. Persistent severe hyperglycemia was associated with the development of Stage II/III necrotizing enterocolitis, after adjusting for age and male gender (OR: 9.49, 95% CI: 1.52 to 59.3).Conclusion:Severe hyperglycemia in the first few days after birth is associated with increased odds of death and sepsis in ELBW infants.

Congenital diaphragmatic hernia. Stabilization and repair on ECMO.

Availability of extracorporeal membrane oxygenation (ECMO) support and the potential advantages of delayed repair of congenital diaphragmatic hernia (CDH) have led several centers to delay CDH repair, using ECMO support if necessary. This study reviews the combined experience of five ECMO centers with infants who underwent stabilization with ECMO and repair of CDH while still on ECMO. All infants were symptomatic at birth, with a mean arterial oxygen pressure (PaO2) of 34 mmHg on institution of bypass despite maximal ventilatory support. A total of 42 infants were repaired on ECMO, with 18 (43%) surviving. Seven infants had total absence of the diaphragm, and 28 required a prosthetic patch to close the defect. Only five infants ever achieved a best postductal PaO2 over 100 mmHg before institution of ECMO. Prematurity was a significant risk factor, with no infants younger than 37 weeks of age surviving. Significant hemorrhage on bypass was also a hallmark of a poor outcome, with 10 of the 24 nonsurvivors requiring five thoracotomies and six laparotomies to control bleeding, whereas only one survivor required a thoracotomy to control bleeding. In follow-up, nine of the 18 survivors (50%) have developed recurrent herniation and seven (43%) have significant gastroesophageal reflux. Importantly, five of the 18 survivors were in the extremely high-risk group who never achieved a PaO2 over 100 mmHg or an arterial carbon dioxide pressure (PaCO2) less than 40 mmHg before the institution of ECMO. In conclusion, preoperative stabilization with ECMO and repair on bypass may allow some high-risk infants to survive. Surviving infants will require long-term follow-up because many will require secondary operations.

Congenital diaphragmatic hernia.

Congenital diaphragmatic hernia occurs in approximately 1 in every 2500 live births and is associated with a reported mortality of almost 35% in live-born patients and a higher mortality when in utero deaths are counted. Ventilator-induced lung injury, pulmonary hypoplasia, and other associated anomalies account for the high death rate. Numerous adjunctive measures have been used to treat these patients. Inhaled vasodilators (nitric oxide), intravenous vasodilators, and fetal therapy have no proven benefit. While animal models of congenital diaphragmatic hernia are surfactant deficient, controversy remains over the use of surfactant in infants. There has been no clinical trial showing any clear benefit with the use of exogenous surfactant in these patients. Similarly, prenatal corticosteroids show some improvements in animal models, but again, there is a complete absence of supportive data to show benefit in humans. Mechanical ventilator strategies that limit ventilator-induced lung injury by avoiding hyperventilation and lung over inflation are the strategies currently in use that have been associated with improved survival. Long-term follow-up of these patients is quite important since gastroesophageal reflux, developmental delay, chronic lung disease, and chest wall deformity are all seen with increased frequency in these children.

Importance of diarrhea as a presenting symptom of appendicitis in very young children

BACKGROUND Appendicitis is an uncommon diagnosis in very young children. It is frequently complicated by delays in diagnosis, perforation, and lengthy hospital stays. OBJECTIVES To review our recent experience with appendicitis among children younger than 3 years old, and to identify the independent predictors of a prolonged hospital stay. METHODS A retrospective case series review was performed on all children under age 3 who had an appendectomy for appendicitis between January 1983 and February 1994. Multiple regression analysis was used to identify the independent predictors of a prolonged hospital stay. RESULTS Sixty-three children were identified. Mean age was 2.2 years (range 11 to 35 months). The mean delay from onset of symptoms to presentation was 4.3 days. Fifty-seven percent were initially misdiagnosed. Diarrhea was reported in 33%. Perforation and/or gangrene were found in 84%. Perforation and/or gangrene at laparotomy and a history of diarrhea at presentation were independent predictors of a prolonged hospital stay. CONCLUSIONS Appendicitis in children under 3 years old is characterized by delays in diagnosis and perforation. A history of diarrhea is an important factor that confuses the diagnosis, prolongs the observation period, and delays appropriate therapy.

Management of pediatric trauma

Injury is the number 1 killer of children in the United States. In 2004, injury accounted for 59.5% of all deaths in children younger than 18 years. The financial burden to society of children who survive childhood injury with disability continues to be enormous. The entire process of managing childhood injury is complex and varies by region. Only the comprehensive cooperation of a broadly diverse group of people will have a significant effect on improving the care and outcome of injured children. This statement has been endorsed by the American Association of Critical-Care Nurses, American College of Emergency Physicians, American College of Surgeons, American Pediatric Surgical Association, National Association of Childrens Hospitals and Related Institutions, National Association of State EMS Officials, and Society of Critical Care Medicine.

Risk factors for chronic lung disease and mortality in newborns with congenital diaphragmatic hernia.

Background: Congenital diaphragmatic hernia (CDH) is associated with a mortality rate of 10–35% in live-born infants. Moreover, CDH survivors have a substantial risk of developing long-term pulmonary sequelae, such as bronchopulmonary dysplasia (BPD). Objectives: This study aims to evaluate risk factors associated with BPD and mortality in neonates with CDH, with particular focus on the initial ventilation mode. Methods: Eligible for inclusion were live-born infants with CDH born from 2001 through 2006 at the centers participating in the CDH Study Group. BPD (defined as oxygen dependency at day 30) and/or mortality by day 30 served as the primary endpoint. Results: A total of 2,078 neonates were included in the analysis. At day 30, 56% of the patients had either died or met the criteria for BPD. In infants who survived until day 30, the prevalence of BPD was 41%. The overall mortality rate was 31%. High-frequency oscillatory ventilation as initial ventilation mode, a right-sided defect, a prenatal diagnosis, a lower Apgar score at 5 min, a cardiac anomaly, a chromosomal anomaly and a lower gestational age were all associated with BPD and/or mortality by day 30. Conclusions: Despite improvements in neonatal care, the rates of BPD and early mortality in newborns with CDH are still considerable. Several important risk factors for a worse outcome are reported in this nonrandomized prospective observational study.

Surgical implications of Henoch-Schölein purpura

Intestinal intussusception, perforation, necrosis, and massive gastrointestinal bleeding are the surgical conditions most often seen in Henoch-Schönlein purpura (HSP). We reviewed the charts of 183 patients who were diagnosed with HSP at two childrens hospitals over a 17-year period. Ten patients underwent laparotomy. Intussusception was the most common surgical lesion. Sequential barium enema and upper gastrointestinal series established the diagnosis of intussusception preoperatively in five of six patients. Two patients developed jejunal perforation and segmental ileal ischemia. Although gastrointestinal bleeding occurred in 52% of patients, the bleeding was self-limiting and no blood transfusion was required. Laboratory data were not helpful in differentiating patients with and without surgical problems.