Arnold G. Coran

Extracorporeal membrane oxygenation (ECMO) in neonatal respiratory failure. 100 cases.

Extracorporeal membrane oxygenation (ECMO) was used in the treatment of 100 newborn infants with respiratory failure in three phases: Phase I (50 moribund patients to determine safety, efficacy, and risks); Phase II (30 high risk patients to compare ECMO to conventional ventilation); and Phase III (20 moderate to high risk patients, the current protocol). Seventy-two patients survived including 54% in Phase I, 90% in Phase II, and 90% in Phase III. The major complication was intracranial bleeding, which occurred in 89% of premature infants (less than 35 weeks) and 15% of full-term infants. Best survival results were in persistent fetal circulation (10, 10 survived), followed by congenital diaphragmatic hernia (9, 7 survived), meconium aspiration (44, 37 survived), respiratory distress syndrome (26, 13 survived), and sepsis (8, 3 survived). There were seven late deaths; in follow-up, 63% are normal or near normal, 17% had moderate to severe central nervous system dysfunction, and 8% had severe pulmonary dysfunction. ECMO is now used in several neonatal centers as the treatment of choice for full-term infants with respiratory failure that is unresponsive to conventional management. The success of this technique establishes prolonged extracorporeal circulation as a definitive means of treatment in reversible vital organ failure.

Surgical Treatment of Gastroesophageal Reflux in Children: A Combined Hospital Study of 7467 Patients

Objective. To review retrospectively the combined clinical experience with the surgical treatment of persistently symptomatic gastroesophageal reflux (SGER) in childhood from seven large childrens surgery centers in the United States. Design. During the past 20 years, 7467 children <18 years of age underwent antireflux operations for SGER at the seven participating hospitals. Fifty-six percent were neurologically normal (NN) and 44% were neurologically impaired (NI). The most frequent diagnostic studies were upper gastrointestinal series (68%), esophageal pH monitoring (54%), gastric emptying study (32%), and esophagoscopy (25%). The age at operation was under 12 months in 40% and 1 to 10 years in 48%. The type of fundoplication was Nissen (64%), Thal (34%), and Toupet (1.5%). A gastric emptying procedure was performed on 11.5% of NN patients and 40% of NI patients. Laparoscopic fundoplication was performed on 2.6% of patients. Results. Good to excellent results were achieved in 95% of NN and 84.6% of NI patients. Major complications occurred in 4.2% of NN and 12.8% of NI patients. The most frequent complications were recurrent reflux attributable to wrap disruption (7.1%), respiratory (4.4%), gas bloat (3.6%), and intestinal obstruction (2.6%). Postoperative death occurred in 0.07% of NN and 0.8% of NI patients. Reoperation was performed in 3.6% of NN and 11.8% of NI patients. The results and complications were similar among the participating hospitals and did not seem related to the type of fundoplication used. Conclusion. The excellent results (94% cure) and low morbidity with gastroesophageal fundoplication with or without a gastric emptying procedure from a large combined hospital study indicate that operation should be used early for SGER in NN children and to facilitate enteral feedings and care in NI children.

Prenatal diagnosis of congenital cystic adenomatoid malformation and its postnatal presentation, surgical indications, and natural history

BACKGROUND/PURPOSE Regression of a cystic adenomatoid malformation (CAM) in a fetus is well described. Little, however, is known about the postnatal course of these infants. This study attempts to correlate the prenatal course of CAMs with postnatal symptoms, radiological manifestations, and need for surgery. METHODS The clinical course of patients with a CAM diagnosed prenatally were retrospectively reviewed. Inclusion in the study required a prenatal ultrasound scan documenting a CAM. RESULTS Over 10 years, 14 patients with a CAM were diagnosed prenatally. Six (43%) showed a partial in utero regression. Four patients were symptomatic at birth and underwent a resection as newborns. Ten patients were asymptomatic at birth, and eight of these had normal chest x-rays. Elective resection has been performed in 3 of these 10, and two additional children are scheduled to undergo an excision near 1 year of age. The remaining five patients have undergone follow-up nonoperatively for a mean of 36 +/- 15 months. Of the seven asymptomatic patients not undergoing immediate surgery, only one has shown a slight postnatal regression, despite five of these showing regression in utero. None have become symptomatic. CONCLUSIONS The results suggest that regression of a CAM on prenatal ultrasound scan is common, but this process does not continue after birth. A normal chest x-ray does not indicate complete regression of a CAM; a computed tomography (CT) scan is required to evaluate such patients, and will generally demonstrate a CAM. Asymptomatic patients with a CAM may be followed up nonoperatively with no apparent adverse effects. The decision and timing of an excision in an asymptomatic patient remains controversial among pediatric surgeons.

Pulmonary embolism in children

The diagnosis of pulmonary embolism (PE) is seldom considered in the pediatric patient; however, pulmonary embolic phenomenon occur with a greater frequency than is generally recognized. Reports of all autopsies performed at the University of Michigan during the 25-yr period 1955 through 1979 were reviewed. All cases of pulmonary thromboembolism in infants and children ranging in age from 0 to 19 yr were studied; patients with emboli from fat or tumors and patients with primary pulmonary artery thrombosis were excluded. The records of 116 children with PE were evaluated. The sex distribution was equal and the average age was 8.0 yr. Age itself was not an independent risk factor for the development of PE. The primary disease processes of shunted hydrocephalus (15.2%), accidental trauma (8.4%), heart disease (4.5%), infection (4.4%), neoplasia (4.0%), and general medical illness (1.8%) were identified as significant risk factors for the development of pulmonary emboli. In the group with neoplasia, children with solid tumors (6.7%) were at an increased risk over the lymphomaleukemia group (2.6%) for the development of emboli. Children who had operation exclusive of the risk factors noted above (7.2%) were at an increased risk when compared to the nonoperative medical group (1.7%). Other risk factors including immobility, central venous catheterization, pre-existing hematologic disorders, and secondary infection were frequently seen. These factors, however, were not able to be studied epidemiologically. Venous thromboses were found in 40% of those children who had pulmonary emboli at the time of autopsy. Iliofemoral venous thrombosis was infrequently seen. The PE was deemed to be clinically important in 30% of the total series. In patients with clinically significant PE, only 50% had documented signs and symptoms of embolization and an antemortem diagnosis was considered in only one-third of these symptomatic patients. The total incidence of PE did not change over the 25-yr period, nor were there any significant trends in the associated risk factors. This study supports the concept that PE occurs in children with a greater frequency than is commonly recognized. Signs and symptoms of clinically significant pulmonary emboli occur in only 50% of the patients and even when the event presents clinically, it is an underdiagnosed entity. A heightened index of suspicion for PE in children is warranted.

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.

Enterocolitis associated with Hirschsprung's disease: A clinical-radiological characterization based on 168 patients

The enterocolitis associated with Hirschsprungs disease (HD) has not been clearly characterized. This study was undertaken to analyze the clinical and radiological findings of Hirschsprungs enterocolitis (HEC) in 168 patients treated from July 1974 through October 1992. HEC occurred in 57 patients (33.9%), either preoperatively (13; 7.7%) or postoperatively (36; 21.4%). In eight patients (4.8%), it occurred pre- and postoperatively. The number of bouts of HEC per patient ranged from one to six (mean, 2.2). The major presenting features were abdominal distension (83%), explosive diarrhea (69%), vomiting (51%), fever (34%), lethargy (27%), rectal bleeding (5%), and colonic perforation (2.5%). There were no deaths directly related to HEC. The analysis of 150 plain x-rays of the abdomen, taken at the onset of HEC or in between bouts, showed that colonic dilatation was the most sensitive radiological finding (90% sensitivity), but it had poor specificity (24%). However, an intestinal cutoff sign (gaseous intestinal distension with abrupt cutoff at the level of the pelvic brim) was both sensitive (74%) and specific (86%) for HEC. Barium enema was of limited value in the diagnosis of HEC bouts because most of the radiographic findings persisted for prolonged periods after cessation of such bouts. The authors conclude that (1) HEC can be characterized as abdominal distension and explosive diarrhea associated with the intestinal cutoff sign and (2) the occurrence of explosive diarrhea in any patient with HD is suggestive of HEC, even in the absence of systemic symptoms, and should be treated to avoid the morbidity and potential mortality of HEC.

An analysis of factors contributing to the development of total parenteral nutrition-induced cholestasis

The risk of developmental of total parenteral nutrition (TPN)-associated cholestatic jaundice in neonates receiving intravenous hyperalimentation is high. Numerous factors have been cited as contributing to TPN cholestasis; however, the exact etiology remains obscure. This retrospective study was undertaken in order to identify any factors which might contribute to this syndrome. The hospital records of 172 neonates requiring TPN for a minimum of 1 week were reviewed. In addition, a subgroup of 32 infants requiring TPN for a minimum of 7 weeks was also examined. Cholestasis was defined as a direct serum bilirubin greater than 2.0 mg/dl during the course of TPN therapy. Significant factors for the development of cholestasis in both groups (n = 172, n = 32) included: number of operations (2.56 vs. 1.08, p = 0.0000), the number of days the patients received antibiotics (40.3 vs 12.9, p = 0.0000), and delayed start of enteral feedings (33.8 vs 14.1, p = 0.0000). Fifteen of the 32 patients who received TPN for at least 7 weeks did not develop cholestasis. In this subgroup (n = 32), there were no differences in birth weight, gestational age, days from birth to the start of TPN, or respiratory distress between those who developed cholestasis and those who remained anicteric. In contrast, there were significant differences between the cholestasis and noncholestasis groups in number of operations (13 vs 6, p = 0.0407), and days until enteral feedings were started (33.1 vs 18.9, p = 0.0289). This study suggests that the factor(s) contributing to the development of TPN-associated cholestasis are likely multifactorial. There appears to be a direct correlation between increasing severity of cholestatic jaundice and duration of TPN. This review does add a new parameter to the various causative factors suggested, namely the number of operative procedures. This new variable could be related to the stress of surgery itself or to the repeated administration of anesthetic agents.

Diagnosis, management, and outcome of cervicofacial teratomas in neonates: A Childrens Cancer Group study

The management of cervicofacial teratomas in neonates is often complicated and may result in significant morbidity and death. A Childrens Cancer Group (CCG) retrospective study was conducted to evaluate a multiinstitutional experience with the treatment of these extremely rare neoplasms. Twenty neonates with cervicofacial teratomas, presenting from 1971 to 1994, were identified from nine CCG institutions. Fourteen neonates had cervical teratomas, and six had orofacial teratomas. There were 12 males and eight females. A diagnostic prenatal ultrasound examination was performed in six cases. Life-threatening airway obstruction occurred in seven infants (35%) in the early postnatal period. Two neonates died in the delivery room without ever having their airway secured. Two other infants with a prenatal diagnosis survived only because tracheostomies were performed by pediatric surgeons who were in the delivery room. Three other patients were orally intubated, one after sustaining hypoxic cardiac arrest. Eighteen infants had their primary tumor excised. Three patients required tracheostomy. After resection, two patients had evidence of unilateral recurrent laryngeal nerve injury, and two required prolonged thyroid hormone replacement. Histological examination showed eight mature and seven immature teratomas. Four infants (20%) clearly had malignant lesions. Pulmonary metastases occurred in two patients and contributed to one late death at 6 months of age. The overall survival rate was 85%, and the mean follow-up period was 5 years (range, 2 months to 16 years). Twelve of 17 surviving patients (70%) have had an excellent functional and cosmetic outcome. Four children have varying degrees of developmental delay and mental retardation. Hypoxia at birth was believed to have contributed to these problems in two cases.(ABSTRACT TRUNCATED AT 250 WORDS)

A decade of experience with the primary pull-through for hirschsprung disease in the newborn period: a multicenter analysis of outcomes.

ObjectiveTo determine whether use of a primary pull-through would result in equivalent perioperative and long-term complications compared with the two-stage approach. Summary Background DataDuring the past decade, the authors have advanced the use of a primary pull-through for Hirschsprung disease in the newborn, and preliminary results have suggested excellent outcomes. MethodsFrom May 1989 through September 1999, 78 infants underwent a primary endorectal pull-through (ERPT) procedure at four pediatric surgical sites. Data were collected from medical records and a parental telephone interview (if the child was older than 3 years) to assess stooling patterns. A similar group of patients treated in a two-stage fashion served as a historical control. ResultsMean age at the time of ERPT was 17.8 days of life. Comparing primary ERPT with a two-stage approach showed a trend toward a higher incidence of enterocolitis in the primary ERPT group compared with those with a two-stage approach (42.0% vs. 22.0%). Other complications were either lower in the primary ERPT group or similar, including rate of soiling and development of a bowel obstruction. Median number of stools per day was two at a mean follow-up of 4.1 ± 2.5 years, with 83% having three or fewer stools per day. ConclusionsPerformance of a primary ERPT for Hirschsprung disease in the newborn is an excellent option. Results were comparable to those of the two-stage procedure. The greater incidence of enterocolitis appears to be due to a lower threshold in diagnosing enterocolitis in more recent years.

Efficacy of the Nissen fundoplication in the management of gastroesophageal reflux following esophageal atresia repair

From January 1974 to December 1988, 80 patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) were treated at the C.S. Mott Childrens Hospital with division of their TEF and esophagoesophagostomy. Thirty-four in this group developed gastroesophageal reflux (GER). After an unsuccessful trial of medical management, 21 underwent Nissen fundoplication, and an additional child with refractory GER died intraoperatively before completion of her fundoplication. Following fundoplication, only eight patients had an uncomplicated course with elimination of reflux and no postoperative dysphagia. Wrap disruption and recurrent reflux occurred in 7 of the 21 (33%), a markedly higher incidence than the 10% figure seen in 220 children without EA who have undergone fundoplication at this institution. Upward tension on the wrap due to the presence of a shortened esophagus probably predisposes to an increased frequency of fundoplication failure in the EA child. In addition, postoperative dysphagia requiring prolonged gastrostomy feedings complicated eight otherwise successful initial or redo-fundoplications. Prolonged dysphagia in this group is likely related to the inability of the dyskinetic esophagus, common in EA, to overcome the increased resistance caused by the Nissen fundoplication. Three deaths (14%) from complications related to antireflux surgery occurred in the series. Although 15 of the 21 children (71%) eventually had excellent long-term results following initial or redo-fundoplication with elimination of reflux and normalization of oral intake, morbidity and mortality were clearly significant. Due to the high incidence of postoperative dysphagia and recurrent reflux, the transabdominal Nissen fundoplication may not be appropriate in EA patients.