Frederick J. Rescorla

Increased risk of necrotizing enterocolitis in premature infants with patent ductus arteriosus treated with indomethacin

OBJECTIVE The authors evaluated the risk of necrotizing enterocolitis (NEC) in very low birth weight infants receiving indomethacin (INDO) to close patent ductus arteriosus (PDA). BACKGROUND DATA Controversy exists regarding the best method of managing very low birth weight infants with PDA and whether to employ medical management using INDO or surgical ligation of the ductus. METHODS Two hundred fifty-two premature infants with symptomatic PDA were given intravenously INDO 0.2 mg/kg every 12 hours x 3 in an attempt to close the ductus. Patients were evaluated for sex, birth weight, gestational age, ductus closure, occurrence of NEC, bowel perforation, and mortality. RESULTS There were 135 boys and 117 girls. The PDA closed or became asymptomatic in 224 cases (89%), whereas 28 (11%) required surgical ligation. Ninety infants (35%) developed evidence of NEC after INDO therapy. Fifty-six were managed medically; surgical intervention was required in 34 of 90 cases (37.8%) or 13% of the entire PDA/INDO study group. Bowel perforation was noted in 27 cases (30%). Factors associated with the onset of NEC included gestational age < 28 weeks, birth weight < 1 kg, and prolonged ventilator support. The overall mortality rate was 25.5%, but was higher in infants with NEC versus those without. The highest mortality was noted in perforated NEC cases. The PDA/INDO patients were compared with a control group of 764 infants with similar sex distribution, birth weights, and gestational ages without PDA who did not receive INDO. Necrotizing enterocolitis occurred in 105 of 764 control patients (13.7%), including 13 (12.3%) with perforation. The overall mortality rate of controls was 25%, which was similar to the overall 25.5% mortality rate in the PDA/INDO study group. CONCLUSION These data indicate that there is increased risk of NEC and bowel perforation in premature infants with PDA receiving INDO. Mortality was higher in the PDA/INDO group with NEC than those PDA/INDO infants without NEC.

Long-Term Outcome for Infants and Children With Sacrococcygeal Teratoma: A Report From the Childrens Cancer Group

BACKGROUND/PURPOSE Sacrococcygeal teratomas (SCT) are a relatively uncommon tumor affecting neonates, infants, and children. This study was designed to determine the effect of therapy on the long-term outcome of neonates and children with sacrococcygeal teratomas (SCT). METHODS The authors conducted a retrospective review of children with SCT treated at 15 Childrens Cancer Group institutions from 1972 to 1994. RESULTS One hundred twenty-six children presented with SCT diagnosed prenatally (n = 32), at birth (n = 79), or later in infancy (n = 15). For neonates, complete resection was performed except in two babies with lethal associated defects. All others (n = 15) underwent resection at the age of diagnosis. Six had a sacral mass identified at birth but had delayed surgery (1.5 to 11 months) and of these, two were malignant. Resection was via sacral (n = 96) or abdominosacral (n = 28) approach. Histology showed mature teratoma (MT, 69%), immature teratoma (IT, 20%), or endodermal sinus tumor (EST, 11%) at presentation. Seven neonates (5.6%) died of perioperative complications, whereas the remaining 117 were available for long-term follow-up. Between 6 and 34 months postresection, recurrent disease developed in 9 of 80 MT patients (11%) followed-up for a mean of 5 years. Recurrent disease was MT (n = 2) and EST (n = 7). The recurrent EST patients were treated with adjuvant chemotherapy. Six are alive with mean follow-up of 114 months, whereas one with metastatic disease was lost to follow-up. Recurrence (MT) developed in only 1 of 24 IT patients, and all are alive and well at mean follow-up of 39 months. Patients presenting with EST (n = 13) underwent excision, with two dying from non-EST causes. Six EST patients received no chemotherapy, with two of the six (33%) experiencing recurrence within 11 months and both disease free after salvage chemotherapy. The remaining five EST patients received adjuvant chemotherapy; four are alive and one died of metastatic disease. Of the 18 EST patients followed-up after resection (presentation, 11, recurrent teratoma, 7), 16 (89%) are free of disease with a mean follow-up of 91 months. CONCLUSIONS (1) Benign teratomas have a significant recurrence rate mandating close follow-up for more than 3 years. (2) Surgical resection alone is adequate therapy for nonmetastatic malignant tumors. (3) Survival for malignant lesions with metastases is excellent with modern chemotherapy.

Inguinal hernia repair in the perinatal period and early infancy: Clinical considerations

Contemporary neonatal intensive care has resulted in survival of many seriously ill preterm and older infants that frequently present with symptomatic inguinal hernia. Controversy exists concerning timing and safety of early repair in prematures or other neonates, especially those hospitalized with concurrent illness. This study examines this topic by evaluating predisposing factors, presentation, and postoperative complications in 100 recent consecutive hernia repairs in previously hospitalized infants less than 2 months of age. There were 85 boys and 15 girls. Thirty percent were premature (less than 36 wks gestation). Forty-two infants were hospitalized for RDS with assisted ventilation in 16 infants, hydrocephalus and ventriculoperitoneal (VP) shunt in 7 infants, and congenital heart disease (CHD) in 19 infants. Clinical presentation was on the right side in 44 infants, bilateral in 42, and on the left side in 14. Incarceration occurred in 31 cases with nine babies having overt intestinal obstruction. The incidence of cryptorchidism was 12.9%. All (VP) shunt, CHD patients, and incarcerated cases were treated with preoperative antibiotics. Following discharge, 49 preterm or previously ill infants developed a symptomatic hernia at home and were readmitted. Nine full-term infants were treated as outpatients. Bilateral inguinal exploration was performed in 92 cases with second hernia or patent processus found in 81. Seven of eight with unilateral exploration had acute incarceration with obstruction at the time of the procedure. Three subsequently required a second hernia repair. Two infants with incarceration and cryptorchid testis or ovarian slider had gonadal infarction. There were eight postoperative complications.(ABSTRACT TRUNCATED AT 250 WORDS)

Surgical resection alone is effective treatment for ovarian immature teratoma in children and adolescents: A report of the Pediatric Oncology Group and the Children’s Cancer Group☆☆☆★★★

OBJECTIVE In both adult women and children the potential for malignant recurrence from ovarian immature teratoma has prompted the standard use of chemotherapy after complete resection of the primary tumor. The efficacy of postoperative chemotherapy in children and adolescents with ovarian immature teratoma, however, has not been established. A pediatric intergroup trial (INT 0106) was designed to determine the need for postoperative chemotherapy in patients with ovarian immature teratoma after management with surgical resection only. STUDY DESIGN Between 1990 and 1995, 44 patients with completely resected ovarian immature tumor and without postoperative chemotherapy, who were able to undergo assessment, were accrued. Tumor tissue was evaluated by central pathology review to confirm diagnosis and determine tumor grading of immature neural elements. Patients were followed carefully for recurrence of disease with appropriate diagnostic imaging and serum marker studies. RESULTS Thirty-one patients had pure ovarian immature teratoma with a tumor grade of 1 (n = 17), 2 (n = 12), or 3 (n = 2). Age at diagnosis ranged between 1.5 and 15 years (median, 10). Of the 29 patients studied, the serum alpha-fetoprotein level was elevated in 10 (34%); the median level was 25 ng/ml. Thirteen patients had ovarian immature teratoma plus microscopic foci of yolk sac tumor. Tumor grade was 1, 2, or 3 in 1, 6, and 6 patients, respectively. Age ranged between 6 and 20 years (median, 12). In the 12 patients evaluated for serum alpha-fetoprotein, 10 (83%) had elevated levels; the median level was 262 ng/ml. The 4-year event-free and overall survival for the ovarian immature teratoma group and for the ovarian immature teratoma plus yolk sac tumor group was 97.7% (95% confidence interval, 84.9%-99.7%) and 100%, respectively. The only yolk sac tumor relapse occurred in a child with ovarian immature teratoma and yolk sac tumor who was then treated with chemotherapy and is alive and free of disease 57 months after recurrence. CONCLUSION The results of this study suggest that surgery alone is curative for most children and adolescents with resected ovarian immature teratoma of any grade, even when elevated levels of serum alpha-fetoprotein or microscopic foci of yolk sac tumor are present. This experience strongly supports avoiding the long-term effects of chemotherapy in most children with ovarian immature teratoma by reserving postoperative therapy for cases with relapse.

Incidence, risk factors, and treatment patterns for deep venous thrombosis in hospitalized children: An increasing population at risk

OBJECTIVE The optimal prophylactic strategy and treatment regimen for deep venous thrombosis (DVT) in hospitalized pediatric patients is not clearly established. This study assessed the incidence, risk factors, and treatment patterns for DVT among pediatric patients admitted to a hospital ward. METHODS Children (aged <17 years) admitted to a single tertiary-care hospital during a 14-year period who developed or presented with DVT were retrospectively identified. Patient demographic and clinical data were analyzed retrospectively. Patients who developed DVT in the hospital were stratified according to the Wells clinical probability scoring system from criteria noted before the diagnosis. Treatment patterns and outcomes were evaluated between the two time intervals of 1992 to 2001 (group I) and 2002 to 2005 (group II). RESULTS Between 1992 and 2005, 358 children were evaluated for DVT, and 99 (52 boys, 47 girls) were admitted to the hospital and were determined to have DVT by confirmatory imaging. A prior DVT (12 total) was present in eight of the 21 patients admitted for DVT treatment; of the remaining, only seven received DVT prophylaxis on admission. In those developing a DVT, the inpatient clinical probability score was 21% (low), 40% (moderate), and 39% (high). The most common risk factor in those with prehospital DVT was a prior DVT (38%) or thrombophilic condition (33%), whereas inpatients had a central catheter (45%), with nearly 50% in the femoral vein. Children acquiring an inpatient DVT had concomitant severe respiratory (17%), oncologic (14%), and/or infectious (15%) diseases and required a prolonged intensive care unit (12.7 days) stay. Prehospital DVT was lower extremity predominant (90%) and statistically different from inpatient-acquired DVT (62%, P = .01). Treatment patterns between periods I and II revealed a trend to more low-molecular-weight heparin and less unfractionated heparin use (P = .09). Three patients died (one fatal pulmonary embolism). The number of recognized cases per 10,000 admissions increased from 0.3 to 28.8 from 1992 to 2005. CONCLUSION The incidence of DVT in hospitalized children is increasing. Those presenting with DVT typically have prior DVT, thrombophilia, or lower extremity disease. Our study suggests that children admitted with severe medical conditions who require a prolonged intensive care unit stay in addition to central venous access (especially via the femoral vein) should be considered candidates for DVT prophylaxis. A clinical probability scoring system alone cannot stratify patients sufficiently to forgo prophylaxis in hopes of a rapid clinical diagnosis. Childhood-specific level 1 trials aimed at determining guidelines for DVT prophylaxis are urgently required.

Diagnosis and treatment of symptomatic breast masses in the pediatric population

Between 1980 and 1993, 74 children and adolescents were referred for surgical evaluation of palpable breast masses. Thirty-two were managed nonoperatively for unilateral thelarche (26), fibroadenoma (3), gynecomastia (2), or hemorrhagic cyst (1). The other 42 children had surgical intervention for giant or painful fibroadenomas (19), breast abscesses (5), painful gynecomastia (6), metastatic disease (4), or other conditions (8). No instances of primary breast malignancy were noted. Physical examination and minimal (selective) diagnostic testing can conserve health care dollars in cases of pediatric patients with breast masses.

Inguinal hernia in children: Factors affecting recurrence in 62 cases☆

This report analyzes factors associated with 71 recurrent inguinal hernias in 62 children treated between 1976 and 1988. Cases were evaluated for sex, age, type of initial repair, interval to recurrence, the presence of comorbid conditions, and type of reoperation. There were 57 boys and 5 girls. Sixty percent of patients were less than 6 months old and 72% were less than 1 year of age at the time of the initial repair. Recurrence was on the right in 74%, left in 24%, and bilateral in 2%. Recurrence was noted by 6 months in 50%, by 2 years in 76%, and by 5 years in 96%. Comorbid conditions were present in 60% of cases, including increased intraabdominal pressure (ventriculoperitoneal [VP] shunts), growth failure, prematurity, chronic pulmonary disease, bladder exstrophy, connective tissue disorders, cryptorchism, seizure disorder, and malnutrition. Incarceration was a factor in four of the 62 cases. Seven patients had multiple recurrences. Fifty-one recurrences were indirect, whereas 20 were direct inguinal hernias. Inadequate high ligation (three with chromic catgut), wound infection, and groin hematoma were other findings. The direct hernias may be related to injury to the floor of the canal during initial repair. Recurrent repair included high ligation of the sac alone (20), high ligation plus snugging of a large internal ring (11), and high ligation with repair of the iliopubic tract in patients with VP shunts, connective tissue disorder, or weak floor (20). All direct hernias had a Coopers ligament (McVay) repair. Two direct hernias recurred again and were successfully repaired using a preperitoneal approach.(ABSTRACT TRUNCATED AT 250 WORDS)

Delayed surgical repair and ECMO improves survival in congenital diaphragmatic hernia.

One hundred ten infants with congenital diaphragmatic hernia (CDH) developed life-threatening respiratory distress in the first 6 hours of life. Associated anomalies were present in 33%. Twenty-eight of 65 infants (43%) treated before 1987 (pre-extracorporeal membrane oxygenation [ECMO] era) survived after immediate CDH repair, and mechanical ventilation with or without pharmacologie support. Only two of 16 (12.5%) infants requiring a prosthetic diaphragmatic patch survived. Since 1987, 31 of 46 (67.4%) infants with birth weight, gestational age, and severity of illness similar to the pre-1987 group survived. All patients were immediately intubated and ventilated. Seven (four with lethal chromosomal anomalies) infants died before treatment, and 30 stabilized (partial pressure of carbon dioxide [Pco2] < 50; partial pressure of oxygen [Po2] > 100; pH > 7.3) and underwent delayed CDH repair at 5 to 72 hours. Fifteen did well on conventional support and survived. Fifteen infants deteriorated after operation: 11 were placed on ECMO with eight survivors, and four infants were not considered ECMO candidates. Nine babies failed to stabilize initially and were placed on ECMO before CDH repair (alveolar-arterial gradient > 600 and oxygenation index > 40), and seven survived. The overall survival rate was 80% at 3 months in this ECMO-treated group. Early mortality was due to inability to wean from ECMO (one), intracranial hemorrhage (one), liver injury (one), and pulmonary hypoplasia (one). Nine of 11 babies requiring a prosthetic patch in the post-1987 ECMO group survived (81.8%). There were three late post-ECMO deaths (3 to 18 months) of right heart failure (two) and sepsis (one). Symptomatic gastroesophageal reflux occurred in nine cases, six requiring a fundoplication in the bypass babies. Recurrent diaphragmatic hernia occurred in nine cases (five ECMO). The overall survival rate was significantly improved in the delayed repair/ECMO group (67% versus 43%; p < 0.05) and was most noticeable in infants requiring a prosthetic diaphragm (81.2% versus 12.5%; p < 0.005). These data indicate that early stabilization, delayed repair, and ECMO improve survival in high-risk CDH. Early deaths are related to pulmonary hypertension and can be reversed by ECMO.

Laparoscopic splenic procedures in children: experience in 231 children.

Objectives:The purpose of this report is to evaluate the efficacy of and complications observed after laparoscopic splenic procedures in children. Methods:Review of a prospective database at a single institution (1995–2006) identified 231 children (129 boys; 102 girls; average age 7.69 years) undergoing laparoscopic splenic procedures. Results:Two hundred twenty-three children underwent laparoscopic splenectomy (211 total; 12 partial) by the lateral approach. Indication for splenectomy was hereditary spherocytosis (111), immune thrombocytopenic purpura (36), sickle cell disease (SCD) (51), and other (25). Four (2%) required conversion to an open procedure. Eight additional laparoscopic splenic procedures were performed: splenic cystectomy for epithelial (4) or traumatic (2) cyst, and splenopexy for wandering spleen (2). Average length of stay was 1.5 days. Complications (11% overall, 22% in SCD patients) included ileus (5), bleeding (4), acute chest syndrome (5), pneumonia (2), portal vein thrombosis (1), priapism (1), hemolytic uremic syndrome (1), diaphragm perforation (2), colonic injury (1), missed accessory spleen (1), trocar site hernia (1), subsequent total splenectomy after an initial partial (1), and recurrent cyst (1). Subsequent operations were open in 3 (colon repair, hernia, and missed accessory spleen) and laparoscopic in 2 (completion splenectomy, and cyst excision). There were no deaths, wound infections, or instances of pancreatitis. Conclusions:Laparoscopic splenic procedures are safe and effective in children and are associated with low morbidity, higher complication rate in SCD, low conversion rate, zero mortality, and short length of stay. Laparoscopic splenectomy has become the procedure of choice for most children requiring a splenic procedure.

The efficacy of early ERCP in pediatric pancreatic trauma

Recognition of pancreatic injuries is frequently delayed, and optimal treatment is often controversial. The use of endoscopic retrograde cholangiopancreatography (ERCP) has allowed accurate delineation of pancreatic ductal injuries; however, the small size of children and the concern with inducing pancreatitis and/or lesser sac contamination have limited its use in children. In 1988, the authors began using ERCP for selected pancreatic injuries. This report describes their experience with this technique and examines the role of ERCP in pediatric pancreatic injuries. Six children with pancreatic transections resulting from blunt trauma were treated between 1988 and 1993. The age range was 2 1/2 to 8 years, and the weight range was 13.6 to 27.9 kg. The average period from injury to referral to the hospital was 14 days (range, 2 to 30 days). All six children presented with chemical evidence of pancreatitis and had an initial computed tomography (CT) scan; five scans were interpreted as being normal. Five of the six patients had subsequent CT scans, which showed lesser-sac fluid collection. Three patients were treated with drainage (2 percutaneous, 1 open [outside hospital]), and when this failed, ERCP was performed, at 13.6 days (average) after presentation. These three patients underwent ERCP relatively early in the course (an average of 3 days after presentation). All six children had major ductal transections documented through ERCP. After ERCP, the serum amylase level remained elevated in three, increased in one, and normal in one. (It was not measured in one of the recent cases taken for immediate operation.)(ABSTRACT TRUNCATED AT 250 WORDS)