Changing concepts in management of pelvic rhabdomyosarcoma in children.

Survival with embryonal rhabdomyosarcoma of all sites has improved dramatically in recent years with the increased use of long-term, cyclic, multidrug chemotherapy. Protocols have been established and are currently being evaluated by the Intergroup Rhabdomyosarcoma Study. The management of embryonal rhabdomyosarcoma of the pelvic viscera, though, remains troublesome. Limited surgical excision is rarely possible and high-dose radiotherapy to the bony pelvis may cause severe and disabling growth disorders. Yet, survival with these lesions is increasing as with rhabdomyosarcoma from all sites and is directly related to a well-planned and aggressive multidisciplinary program. We have seen 12 cases of pelvic rhabdomyosarcoma within the last seventeen years at this institution. These cases will be reviewed in regard to varying modes of therapy and survival. Our current therapeutic approach, based on national and local experience, will be presented.

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.

Congenital anomalies of the diaphragm. Personal experience with thirty-five consecutive cases.

A personal experience of thirty-five consecutive patients with congenital anomalies of the diaphragm is reported. The anomalies included twenty-five Bochdalek hernias, seven diaphragmatic eventrations, and three esophageal hiatal hernias. Except for the patients with esophageal hiatal hernias, virtually all patients presented with respiratory embarrassment, twenty-seven of the thirty-five within 24 hours of birth. An especially high mortality is associated with this early onset of symptoms. The treatment was surgical with a thoracic approach used for right Bochdalek defects and eventrations, and an abdominal approach used for left Bochdalek hernias. The importance of proper preoperative and postoperative management in a well equipped neonatal intensive care unit is emphasized.

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.

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.

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.

Congenital lung cysts.

Congenital cystic adenomatoid malformation (CCAM), pulmonary sequestration, congenital lobar emphysema (CLE), and bronchogenic cysts are all congenital malformations of the lung that present in imaging studies as abnormal air, air/fluid, or fluid-filled cysts. The embryology, histology, clinical presentation, and treatment of these lesions are discussed based on world literature and our experience with 22 operative resections of congenital lung cysts over the past 10 years. The roles of prenatal diagnosis and fetal surgery in the management of certain lung cysts are considered. Computed tomography has emerged as an extremely useful tool in the differential diagnosis of these lesions. Surgical excision by segmentectomy or lobectomy for intrapulmonary lesions and simple excision for extralobar sequestrations and bronchogenic cysts are safe and have a low incidence of complications. Asymptomatic patients with CLE may not require resection. Overall, with accurate diagnosis and preoperative planning these rare but fascinating anomalies can be treated safely and effectively, with excellent results.

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.

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.

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.

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.

Major duodenal injuries in children: diagnosis, operative management, and outcome.

The diagnosis, treatment, and outcome of ten children less than 13 years old operated on for major duodenal injuries is reviewed. Three had gun shot wounds with perforation and seven had blunt trauma resulting in duodenal disruption. Of those with blunt trauma, three had massive injuries requiring immediate operation, and four had what appeared to be lesser injuries. The symptoms of those children with lesser injuries were initially vague and delayed in onset, resulting in a delay of 24 hours to 7 days from time of injury to admission. At the time of admission, all four had tenderness localized to the right lower quadrant and an unclear preoperative diagnosis. Eight had associated injuries, most commonly to the pancreas. The single death occurred within 48 hours of injury and was the result of an associated head injury. The surviving nine patients left the hospital between the 11th and 22nd postoperative day doing well. One patient with a transected pancreas and extensive duodenal injury underwent a pancreaticoduodenectomy with a Whipple type reconstruction. Two patients with an extensive blowout injury to the duodenum and one with a crush injury to the pancreas underwent a pyloric exclusion and gastrojejunostomy. These patients did well with no fistula formation. The remaining seven children underwent debridement and primary closure of the duodenal injury. Four developed fistulae; however, two were pancreatic, one was jejunal, and only one was duodenal. All drained less than 250 mL per 24 hours and all except the jejunal fistula were closed by the 17th postoperative day. All were supported nutritionally either intravenously or more recently with a jejunal catheter placed at operation.(ABSTRACT TRUNCATED AT 250 WORDS)

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.

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.

Limy bile syndrome.

Limy bile syndrome (LBS) is a rare condition in which a radiopaque gallbladder and/or bile ducts are noted on plain roentgenograms. LBS is caused by calcium carbonate precipitation in the bile and is usually associated with distal biliary tract obstruction. The etiology of limy bile syndrome is unclear; however, it may be a long-term complication of total parenteral nutrition.

Bilateral Wilms' tumor.

Recent studies indicate a favorable outcome with bilateral Wilms' tumor. From 1971 to 1985, ten children between the ages of 6 months and 5 years were treated for this disease. During the early part of the series, five patients had nephrectomy on one side and partial nephrectomy on the other side, and one patient had bilateral partial nephrectomies at the same operation. More recently four patients initially had a biopsy of both tumors and lymph node sampling followed by chemotherapy. At the second-look procedure two patients had multiple biopsies because there was no obvious tumor. Histologically there was no tumor on the third-look procedure in these two patients. Nine patients had a favorable histology of the Wilms' tumor. One patient had a favorable histology on one side and an unfavorable type of histology on the other side. Eight patients are surviving between 6 months and 13 years. Two died of extensive disease within 16 months of diagnosis. One patient had an unfavorable histology. The good results following partial nephrectomies have led us to attempt to conserve additional tissue, as has been done in the last four patients. Our early results suggest biopsy of the tumor followed by chemotherapy, then a second look, and if necessary, third-look procedures may result in preservation of functioning renal tissue.

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.

Resection with primary anastomosis for necrotizing enterocolitis.

Primary anastomosis following the resection of bowel for necrotizing enterocolitis (NEC) can be done with acceptable morbidity and mortality rates. Twenty-seven patients are reported with three deaths. Primary anastomosis will avoid problems seen with small-bowel stomas. The concomitant ligation of a significant patent ductus arteriosus (PDA) should be considered for patients undergoing abdominal procedures for NEC.