Is surgical ligation of a patent ductus arteriosus the preferred initial approach for the neonate with extremely low birth weight?

The optimal approach to a patent ductus arteriosus (PDA) in an extremely low birth weight (ELBW) neonate, whether initial surgical ligation or a trial of indomethacin, has not been established. The authors reviewed the records of 82 ELBW premature infants who had surgical ligation of a PDA during a 2-year period. Thirty-one received indomethacin before ligation. Bronchopulmonary dysplasia (BPD) occurred in 33% of the infants. Predictors of BPD were prolonged positive pressure ventilation, severe intraventricular hemorrhage (IVH) and lower birth weight (BW). Seventy-seven percent of the infants survived. Predictors of mortality were severe IVH, lower BW, and the occurrence of necrotizing enterocolitis (NEC). The indomethacin-treated infants had a lower incidence of NEC and IVH. Overall, 16% of the patients had perioperative morbidity, and 10% of the patients died. The study shows that a trial of indomethacin therapy is not associated with increased complications in ELBW infants with PDA.

Inflammatory pseudocyst associated with trisomy 21 and Hirschsprung's disease.

A 15-month-old black male with trisomy 21 and Hirschsprung's disease developed an inflammatory abdominal pseudocyst 1 month after Swenson pull-through repair. Although abdominal pseudocyst associated with ventriculoperitoneal shunts, peritoneal dialysis catheters, meconium peritonitis, and pancreatitis have been reported, this is the first report of a pseudocyst developing 1 month after a surgical repair of Hirschsprung's disease.

Atypical cytogenic aberrations in two childhood peripheral primitive neuroectodermal tumors.

Atypical cytogenetic abnormalities were detected in peripheral primitive neuroectodermal tumors (PPNET) of the extremity in two children. One had an osseous tumor with a balanced reciprocal translocation, t(5;9)(q22;q32), and had a complete response to therapy. The other had a non-osseous tumor with an interstitial deletion, del(18)(q12.2q21.2), was resistant to combination therapy, and at autopsy had evidence of possible clonal evolution with the karyotype 46,XX der(8)t(8;8)(p11.2;q13), inv(16)(p13.2q12),del(18)(q12.2q21.2). Neither tumor demonstrated the t(11;22)(q24;q12) typically found in Ewing's sarcoma and PPNET, suggesting heterogeneity of the cytogenetic aberrations seen in this rare childhood malignancy.

Mediastinal tumors--airway obstruction and management.

Large mediastinal massess can cause compression of surrounding mediastinal structures. Patients may have symptoms of airway obstruction or cardiovascular compromise. The additive effects of anesthetics, paralysis, and positioning during biopsy can lead to acute airway obstruction and death. In some cases, tissue diagnosis can be achieved and treatment initiated without general anesthesia. When general anesthesia is necessary, specific measures should be taken to avoid disaster or immediately alleviate obstruction should it occur. Some patients at greatest risk will require pretreatment of the mass before tissue diagnosis. This article reviews these issues and provides a useful algorithm for managing patients with mediastinal masses.

Use of argon beam ablation and sclerotherapy in the treatment of a case of life-threatening total abdominal lymphangiomatosis.

Congenital peritoneal and retroperitoneal lymphangiomas are rare lesions, which are often difficult to treat. This is the case of a 15 year old who had life-threatening complications from diffuse abdominal lymphagiomatosis after failure of accepted medical and surgical therapies. The authors describe the use of the argon beam coagulator in the treatment of this disease.

Emergency center arteriography in the evaluation of suspected peripheral vascular injuries in children.

During the 6-year period from 1983 to 1989, 109 children aged 3 to 18 years (mean, 16) with suspected peripheral vascular injuries underwent 113 emergency center arteriograms (ECA) performed by hand injection of contrast material using a single roentgenographic film. The most common indication for ECA was the proximity of the injury in 93 (82.3%) of the cases as penetrating injury accounted for 106 (94%) of the cases. There were 89 true-negative, 14 true-positive, 1 false-negative, and no false-positive arteriograms. The remaining 9 arteriograms were either equivocal or technically inadequate. The sensitivity, specificity, and diagnostic accuracy of this procedure were 98.9%, 100%, and 91.2%, respectively. Operative intervention was required for 9 (64.2%) injuries detected by emergency arteriography. The remaining 5 injuries were considered minor and were observed with no complications during a period of 21 months. Sixty-eight children (76.4%) with negative ECA were followed for a mean of 12 months with no vascular complications or growth abnormalities noted. ECA is a rapid, accurate, and cost-effective technique. It is of particular value in detecting the presence of occult arterial injuries that might have deleterious effects in the growing child.

Pancreatic anatomy in children: emphasis on its importance to pancreatectomy.

The literature is riddled with misconceptions concerning the landmarks and the actual amount of pancreatic tissue resected in "subtotal" 60% to 90% pancreatectomies. In order to clarify the surgical anatomy of the pancreas, 13 autopsy specimens from children aged 2 days to 15 years were carefully examined. Pancreata were serially removed from the in situ position in four sections and weighed. Section 1 included pancreatic tissue from the tail to the left of the superior mesenteric vessels (SMV); section 2 included all tissue from the tail to the right of the SMV; section 3 contained tissue up to the left of the pancreaticoduodenal vessels in the head of the pancreas; and section 4 included the remainder of the pancreatic head within the duodenal sweep and the uncinate process. Results showed that section 1 weighed 47.5% +/- 9.7% (mean +/- SD) of the total pancreatic weight with a wide range of 32.3% to 67.3%; section 2 weighed 53.5% +/- 9.0% (range, 38.7% to 72.2%); section 3 weighed 69.1% +/- 15.6% (range, 43.5% to 95.8%); and section 4 weighed 31.6% +/- 14.6% (range, 4.2% to 56.5%). As noted, pancreatic anatomy was extremely variable in relation to the percent of tissue to either side of the SMV and the proportion of tissue contained within the uncinate process. Resections historically reported to result in a "75%" pancreatectomy (section 2) were demonstrated to contain a mean of only 53.5% +/- 9.0% of the pancreas. Less tissue than expected would be resected by using the SMV as landmarks for a 75% resection. The wide variation in anatomy also has important clinical implications in performing "subtotal" pancreatectomies in infants with nesidioblastosis in whom the amount of tissue resected is critical to the patient's postoperative clinical course. This study demonstrates that a subtotal pancreatectomy should not be based upon the presumed relation of the pancreatic substance to the SMV. The entire gland including the uncinate process should be visualized at operation in order that a proper pancreatectomy is performed.

Thoracic trauma in children.

Thirty-seven children were admitted for the treatment of thoracic injuries during a 15-year period. Two thirds suffered blunt trauma. Of these children with blunt injuries, three quarters were male, with a median age of 6 years. Half of all victims of blunt trauma were between the ages of 4 and 6 years, and most were involved in motor vehicle crashes. Thirteen children (35%) were victims of penetrating trauma. Sixty-nine percent were male. The median age was 13 years, with 9 of 13 patients in their teenage years. Injuries were evenly distributed between stab and gunshot wounds. Eight of the 37 patients (22%) required thoracotomy. Fourteen had associated intra-abdominal injuries. Twelve of these patients had injuries that required exploratory laparotomy and two were managed expectantly. There were 27 patients with lung injuries, three quarters of whom were managed with tube thoracostomy alone. Seven children suffered injuries that resulted in their deaths. Six had been struck by a motor vehicle. Four of these six died of closed head injuries. The overall mortality of patients with blunt trauma was 25%.

Experience with a total muscle-sparing approach for thoracotomies in neonates, infants, and children.

We have adopted a total muscle-sparing technique for thoracotomies in infants and children. The technique preserves the latissimus dorsi and serratus anterior muscles and provides excellent exposure for most thoracic and mediastinal operations. Thirty-two such procedures have been performed with only one complication, a small wound seroma. Use of this technique may not only decrease postoperative pain and splinting, but may decrease the incidence of scoliosis and muscle dysfunction found in children having undergone thoracotomies as infants.

Acute genital injury in the prepubertal girl.

In an effort to develop guidelines for the management of acute genital injuries in prepubertal girls, we categorized 32 cases by the object that allegedly caused the injury: straddle injuries, nonpenetrating injuries, penetrating injuries, and torque injuries. Using these categories and the anatomic features of symmetry and/or hymenal transection, we determined that the most dangerous injuries were the penetrating injuries that were symmetric and transected the hymen; in this series these were all the result of sexual assault. Future studies are needed to determine if these unique injuries can be managed with less physical and psychosocial trauma to the young patient.

Isoperistaltic intestinal lengthening for short bowel syndrome.

Because of improvements in supportive care, many infants now survive massive intestinal loss and have short bowel syndrome. Unfortunately, some survivors are left with an insufficient amount of intestine and cannot be weaned from total parenteral nutrition. An isoperistaltic intestinal lengthening procedure was used to treat surgically two such infants with 25 centimeters of remaining small intestine and absent ileocecal valves. This surgical technique longitudinally divides the short, dilated small intestine into two smaller, parallel lumens that are anastomosed end to end. This procedure preserves all mucosa, prolongs transit time by doubling intestinal length and corrects the ineffective peristalsis by tapering the dilated intestine. The lengthening technique can be performed because of the anatomic division of the intestinal vasculature within two leaves of the mesentery. Longitudinal division between the two leaves maintains vasculature to each side of the intestine. The isoperistaltic intestinal lengthening procedure, as it was successfully applied to two infants, is described in detail.

A preliminary report of ticarcillin and clavulanate versus triple antibiotic therapy in children with ruptured appendicitis.

Standard therapy for childhood ruptured appendicitis includes combination antibiotic therapy with ampicillin, gentamicin and clindamycin. Complicated dosing schedules and the possibility of aminoglycoside toxicity make alternatives desirable. One such alternative is Timentin (a combination agent of ticarcillin disodium and clavulanate potassium). This agent has a more convenient dose schedule than standard therapy and eliminates the possibility of aminoglycoside ototoxicity and nephrotoxicity. It is active in vitro against most pathogens associated with ruptured appendicitis in children. The preliminary results of an ongoing prospective, open label, randomized trial comparing ticarcillin and clavulanate with ampicillin, gentamicin and clindamycin in childhood ruptured appendicitis are reported herein. While further evaluation is necessary, we have found single agent therapy with ticarcillin and clavulanate to be equivalent in safety and efficacy to standard combination therapy. Also discussed are the relative merits of immediate versus delayed primary closure of the abdominal wound after appendectomy.

Hepatocellular transplantation in acute hepatic failure and targeting genetic markers to hepatic cells.

Orthotopic liver transplantation (OLT) represents the only therapeutic option for many patients with end-stage liver disease as well as many inborn genetic errors of hepatic metabolism. Despite dramatic progress in methods for OLT, the utilization of this procedure is limited by its considerable morbidity and mortality, by a chronic shortage of organs for transplant, and by difficulty arranging funding for many patients. Many children with fulminant hepatic failure do not receive OLT because this technology is unavailable or unaffordable. Hepatocellular transplantation (HCT), in which isolated, heterologous hepatocytes from a donor liver would be infused into the diseased organ in order to provide essential hepatic functions, could provide a much needed therapeutic alternative to OLT in the treatment of some causes of hepatic insufficiency. Experiments in animals have demonstrated that several genetic deficiencies of hepatic metabolism as well as experimental induced hepatic failure in animals can be reversed by HCT. Despite this experience, HCT has never been attempted in human subjects. This protocol represents the first proposed clinical trial of HCT. We are proposing a clinical trial in which HCT would be attempted as a therapeutic intervention in children with acute hepatic failure who have no other medical or surgical options. This proposal is intended to establish surgical methods for HCT and to evaluate the feasibility of this procedure for treating hepatic disease in humans. It is our expectation that HCT may provide short-term support for patients awaiting organ availability, a "bridge to recovery" allowing patients with fulminant hepatic failure to recover, or a long-term repopulation of the patient's liver with healthy donor cells. One of the major limitations of many animal studies in HCT is that, since the donor hepatocytes are often indistinguishable from those of the host, it has often been difficult to demonstrate a clear correlation between engraftment and the therapeutic effect. In order to verify engraftment independent of the therapeutic response, we propose to "mark" the donor hepatocytes by transducing these cells with a recombinant retroviral vector (LNL6) carrying a marker gene (NEO-R, neomycin phosphoribosyl transferase). The presence of this marker will enhance the ability to identify transplanted cells in the host using assays for the NEO-R gene or transcribed NEO-R mRNA. The LNL6 vector has been approved for human use and has been used as a marker gene for transplanted cells in human subjects without any reported adverse effects. We would like to emphasize that this is a proposal with therapeutic intent.(ABSTRACT TRUNCATED AT 400 WORDS)

Current management of laryngeal and laryngotracheoesophageal clefts.

Laryngeal and laryngotracheoesophageal clefts (L-LTEC) are uncommon anomalies in neonates that cause significant morbidity secondary to aspiration, pneumonia, and respiratory distress. Other anomalies of development, such as esophageal atresia and tracheoesophageal fistula (EA-TEF), are observed in 20% of patients with L-LTEC and often confuse the radiographic and clinical picture. Repair of L-LTEC depends on the length and location of the cleft, associated anomalies, and concurrent systemic illness. For type I L-LTEC, endoscopic repair occasionally is possible with microsurgical instrumentation. With types II to IV L-LTEC, an open approach must be used. Tracheotomy is a universal requirement, often for extended periods of time. Reported here is our experience with four cases of L-LTEC managed over the past 7 years at Texas Children's Hospital.

Ventriculoatrial shunting via the azygos vein.

The treatment of hydrocephalus has evolved through many stages but the "cure" is still elusive. It is not unusual for the neurosurgeon to find that the commonly used routes for catheter placement or sites for drainage of cerebrospinal fluid (CSF) cannot be employed. The azygos vein was used to gain access to the right atrium when the CSF could not be drained into the peritoneal cavity, nor could the neck veins be used to place the catheter into the right atrium. The azygos vein is a convenient and safe route to reach the right atrium in selected patients.

Thyroid nodules in children: current profile of a changing disease.

Pediatric thyroid disease has changed since the decline of widespread cervical irradiation. Our analysis of 32 recently resected childhood thyroid nodules showed that the incidence of malignancy (22%) is decreasing, though the incidence of nodules is stable. Risk factors for development of thyroid disease include female sex, age around puberty, family history of thyroid disease, previous or coexisting thyroid disease, and history of a medical condition that may be steroid- or endocrine-related. Age, sex, location and size of nodule, duration of symptoms, and family or patient history of thyroid disease did not distinguish between malignant and benign disease preoperatively. Of the seven children with thyroid cancer, six (86%) had unrelated concurrent medical conditions, compared with 9/25 (36%) with benign disease and 2/11 (18%) with adenoma. Fine needle biopsies provided correct preoperative diagnosis in 6/7 cases. As pediatric thyroid malignancy becomes rarer and the high-risk nodule becomes better defined, criteria for surgical versus nonsurgical modes of diagnosis and treatment should become more distinct.

Review of bronchopulmonary foregut malformations.

The term bronchopulmonary foregut malformation (BFM) describes rare cases of pulmonary sequestrations that communicate with the upper gastrointestinal tract. An unusual type of BFM is described; it consisted of esophageal atresia, proximal and distal tracheoesophageal fistulae, and bilateral extralobar pulmonary sequestrations communicating with a common bronchial structure that replaced the distal esophagus. This case is related to other reported cases in the literature, and serves as the basis for a proposed theory of the embryonic development of BFM.

Central venous catheters in older children.

This report discusses the safety of 89 cuffed, Silastic (Dow Corning, Midland, MI) catheters placed in children between the ages of 1 and 18, a group that may be considered high risk on the basis of age. The overall complication rate was once every 288 days, three times more often than in published results in adults, but less often than in other pediatric series. The sepsis rate of once each 1236 days was almost twice the adult rate. Thirty (35%) of the catheters were used at home, accounting for 85 per cent of the total catheter use. Among this group, the overall complication rate was only once every 815 days, three times less frequent than in comparable adult series. The complication rate due to sepsis was once each 2444 days, the same as noted in adults. Whereas these catheters may be more hazardous overall in this age group, home usage appears to be safer than expected. These catheters are well accepted by this age group and their use, particularly among outpatients, should not be restricted for fear of an unacceptably high complication rate.

A totally implanted venous access system used in pediatric patients with cancer.

A safe and effective method of venous access is important in the care and treatment of patients with malignancies. A recently available totally implantable venous access system offers advantages over traditional central vein catheters (Broviac and Hickman, Houston). We report our experience with the implanted venous access system used in 31 pediatric patients with malignancies. The mean age of the patients was 7 years (range, 6 months to 17 years), and the mean indwelling time of the catheters was 232 days (range, 14 to 607 days; total patient days, 7,198). The catheters were used to administer chemotherapy, drugs, blood products, and parenteral nutrition, as well as to draw blood. Clotting occurred in the catheters on four occasions, requiring removal of two catheters. Fever occurred in eight patients; one developed a local infection at the site of implantation and four developed bacteremia. Our use of the implanted venous access system in children resulted in a lower rate of infection compared with that when the traditional Broviac and Hickman catheters were used, and simplified patient management.