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.

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)

Enteric thoracoabdominal duplications in children.

The clinical and pathological findings of two children with thoracoabdominal enteric duplications are described in detail. In addition, 23 case reports are reviewed. The majority of duplications communicated with the gastrointestinal tract below the diaphragm, but in one case, reported here, the duplication communicated with the cervical esophagus. In infants these duplications most often present with respiratory symptoms, whereas older children more typically have pain and melena. If possible the entire duplication should be removed during one operation.

Congenital duodenal obstruction. A review of 65 cases.

We report our experience with 65 patients with congenital duodenal obstruction, 36 with intrinsic and 29 with extrinsic lesions. Seventeen patients had trisomy 21 syndrome. Eight pregnancies were complicated by polyhydramnios. The diagnostic features encountered, the operative procedures used, and the postoperative management regimes used are presented. Thirty-two of the 36 patients with intrinsic lesions and 28 of the 29 patients with extrinsic lesions survived. The data on the five patients who died emphasize the effect of multiple congenital anomalies and prematurity on survival. This review suggests that the surgical procedures available for treating patients with congenital duodenal obstruction are well established and yield predictably good results.

Acute appendicitis in preschool age children.

Appendicitis in preschool children is not uncommon. A duration of greater than 40 hours usually implied perforation. The most common findings were abdominal pain, tenderness, temperature elevation and vomiting. In the presence of perforation or abscess formation, appendectomy followed by copious wound irrigation, abscess drainage and delayed secondary wound closure is the procedure of choice.

Cholelithiasis and cholecystitis in childhood.

In this review of 50 children with gallbladder disease in Houston, Texas, there were an equal number of patients with and without hemolytic disease and a slight predominance of males in both groups. Nearly all had been symptomatic for a prolonged period before diagnosis and cholecystectomy. Common early diagnoses were hemolytic crisis and appendicitis. Ultrasonography has become a very accurate tool for the preoperative evaluation of these children. Once the diagnosis of cholelithiasis is established, elective cholecystectomy is the treatment of choice.

Late presentations of midgut malrotation in children.

Malrotation of the intestine may become symptomatic in the older child and may manifest itself in atypical presentations. Older children may present with symptoms of less than 72 hours duration which are typical of acute duodenal obstruction. More frequently, however, the older child with malrotation will present with chronic abdominal pain with or without vomiting or chronic diarrhea. The diagnosis of malrotation should be considered in any child with intermittent abdominal pain, vomiting, diarrhea, or malabsorption. Surgical intervention is curative and should be implemented as soon as possible after the diagnosis is made.

Gastroschisis and omphalocele.

The experience of 73 consecutive infants with gastroschisis and omphalocele is reported. The overall survival rate was 80 percent; however, since 1973 the survival rate for ruptured and intact omphaloceles has been 87 percent and 93 percent for gastroschisis. This remarkable reduction in mortality has been attributed primarily to the advent of total parenteral hyperalimentation, but mortality has also decreased due to the use of pediatric respirators which overcome the effects of increased intraabdominal pressure, and the creation of the neonatal intensive care unit where monitoring of these often fragile infants and the presence of specialty personnel assist in their care.

Intestinal stenosis resulting from necrotizing enterocolitis.

Fourteen infants with intestinal stenosis as a late sequela of necrotizing enterocolitis were treated at Texas Children's and Ben Taub General Hospitals from 1972 to 1979. Barium enema studies are the keystone in making the diagnosis and should be performed in any infant with abdominal distention or poor feeding after recovery from acute necrotizing enterocolitis. Spontaneous resolution of stenosis is a definite clinical entity, and nonobstructed infants should be given a trial for resolution. This trial should be for a limited time. The operative therapy for the infant with obstruction or the infant who fails to have spontaneous resolution of stenosis must be individualized. Resection with primary anastomosis best serves those infants with limited stenotic lesions, minimal dilatation of the intestinal lumen and no other complicating medical problems. Intestinal diversion with later resection and enterostomy closure should be reserved for infants with intestinal obstruction and complicating medical problems.

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.

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.

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.

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.

Morgagni hernias during infancy: presentation and associated anomalies.

Morgagni hernias during infancy are associated with significant respiratory symptoms as well as other congenital anomalies, particularly congenital heart disease. During the past 6 years we have had the opportunity to treat five infants less than 1 year of age with Morgagni hernias. A review of the literature revealed an additional seventeen detailed case reports of infants with Morgagni hernias. Each of our patients was symptomatic as were all but two of those previously reported. These patients presented with tachypnea, dyspnea, and cyanosis which was very similar to the presentation of Bochdalek hernias seen during infancy. Thirteen of 16 patients in whom detailed descriptions of associated anomalies were given including four of our own patients, had significant congenital anomalies. Nine involved the heart, including three infants with dextracardia, three with ventricular septal defects, and two with anomalous pulmonary venous return. Five infants were retarded, including three with Trisomy 21. Two of the previously reported patients had large omphaloceles and other stigmata of Cantrell's syndrome. All but one of the patients reported, including ours, had a hernia sac. The liver, colon, and small bowel were most commonly found in the hernia sac; however, the presence of the stomach and spleen have been reported. While both the transabdominal and transthoracic approaches to Morgagni hernias have been advocated and, indeed, repair of the defect can be accomplished through both approaches, the transthoracic approach allows better exposure of incarcerated viscera, particularly the frequently found anomalous left lobe of the liver.

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.

Left testicular and groin pain: an unusual presentation for perforated appendicitis.

A 12 yr old black male presented with a 3 day history of left inguinal and testicular pain associated with nausea, vomiting, and fever. Left groin exploration demonstrated a markedly inflammed patent process vaginalis containing purulent exudate. Concomitant abdominal exploration demonstrated a perforated gangrenous appendix with diffuse peritonitis.

Treatment of uncomplicated meconium ileus via T-tube ileostomy.

There are many treatment modalities for uncomplicated meconium ileus. However, for neonates with intestinal obstruction unrelieved by nonoperative measures we advocate T-tube ileostomy with postoperative irrigation of the intestine utilizing pancreatic enzyme. Our series includes 11 infants with uncomplicated meconium ileus who underwent T-tube ileostomy 24--96 hr after birth. Of the 7 females and 4 males only 2 infants failed to pass meconium. One infant required reexploration for persistent obstruction and 1 infant, the only death encountered, had overwhelming pulmonary infection and intracranial hemorrhage. The remaining 9 infants passed meconium between the 1st and 11th postop days. The T-tube was removed by pulling it out between the 10th and the 14th postop day with the T-tube tract closing spontaneously in all patients. Our experience with T-tube ileostomy suggests that resection of dilated but viable ileum is not necessary for prompt intestinal function in the neonate. With the use of pancreatic enzyme irrigation, the tenacious meconium in these patients can be rapidly liquified and expelled per rectum or T-tube and the necessity of mechanical removal during the operative procedure avoided.

Lateral wall cecal filling defects following successful hydrostatic reduction of cecalcolic intussusceptions.

Two children less than 1 yr of age were found to have a lateral wall filling defect following successful hydrostatic reduction of cecalcolic intussusceptions. Both filling defects were thought to be due to invagination of the lateral wall between the tinea coli. One child was explored due to recurrence of the intussusception despite despite a decrease in size of the filling defect. In the second child the filling defect had disappeared three days after the initial barium enema reduction. When confronted with a typical cecal filling defect in an infant following successful reduction of a cecalcolic intussusception, we believe that one is justified in assuming a cautious "wait and see" attitude. If the child recovers clinically the barium enema may be repeated in three days to access reduction in size or disappearance of the filling defect.

Gastroschisis complicated by intestinal atresia.

Five of 22 infants operated upon in the last 4 yr for gastroschisis had an associated loss of intestinal continuity. Four of the infants had an intestinal atresia and one had a gangrenous segment of ileum. Each had primary closure of the gastroschisis and decompression of the proximal intestine. Four had cutaneous enterostomy and one had long tube intubation. Four infants were returned to the operating room between 2 and 4 wk of age and their intestinal continuity was established. At the second operation the intestine, which had presented as a matted mass at birth, appeared normal with resolution of the serosal edema and few adhesions. The length of the intestine that initially had appeared shortened may be more accurately evaluated and is usually considerably greater than was appreciated at birth. If distal atresias are present, they will become apparent. A primary anastomosis can be carried out using the principles of repair for an isolated intestinal atresia in the newborn. All five of these infants have survived.

Necrotizing enterocolitis: incidence, operative care, and outcome.

At Jefferson Davis Hospital, the incidence of necrotizing enterocolitis (NEC) was three per 1,000 live births, and 30 per 1,000 low birth weight births. The occurrence of NEC was sporadic and no epidemics occurred. NEC occurred most frequently in infants weighing between 750 and 1,500 g, and the smaller infant with NEC was more likely to require surgical intervention. As the survival of small birth weight infants improved over the 4 years of the study, the patient population developing NEC became smaller. The age at operation also increased in the period between 1982 and 1984. Those infants who developed NEC after 30 days of age typically had more extensive disease and a less favorable prognosis. In this series, 31% of infants with acute NEC required surgical intervention. An additional 11% of those infants treated nonoperatively eventually required surgical intervention for late sequelae of NEC. The overall survival of infants with NEC was 75%. While the survival of all infants operated for NEC was 68%, the survival for those with the acute syndrome was 63% and those operated on for late sequelae was 87%. Primary anastomosis in selected patients did not adversely affect mortality and simplified the postoperative care of these infants with severe complications. Indeed, enterostomy closure in an infant who had previously had NEC was an extensive procedure that carried significant risk. Our results indicated that the trained pediatric surgeon could predict at the operating table which infants could safely undergo resection and anastomosis and that, with experience, the percent undergoing primary anastomosis increased to approximately 50%.