Cavitating primary pulmonary fibrosarcoma in a child.

Primary pulmonary sarcomas are rare tumors at all ages. They are usually solid and often remain silent until large. Prognosis is related to size and histologic characteristics. Curative efforts have been directed toward complete surgical removal. Presented in this report is an 11-year-old girl who was thought to have a bronchogenic cyst. At operation a 14 cm cavitating primary pulmonary fibrosarcoma was found, which was incompletely resected. The combined treatment modalities of surgical therapy and chemotherapy have resulted in a disease-free period of 36 months.

Adenocarcinoma of the colon and rectum in the adolescent.

Between 1969 and 1982, seven adolescents (six girls and one boy) with rectal bleeding and other nonspecific intestinal symptoms were diagnosed with biopsy to have colonic or rectal carcinoma. All came from an impoverished urban environment, and two patients were members of a family with a cancer diathesis (Turcot's syndrome). Surgery provided the only successful curative or palliative treatment. Chemotherapy and radiation therapy were unsuccessful in preventing the progression of disease in far-advanced, nonresectable cases. Prompt attention to lower gastrointestinal bleeding and nonspecific abdominal symptoms in adolescents may result in the earlier diagnosis of colorectal malignancy and improved opportunity for definitive surgical cure.

Colonic polyps and coloduodenal fistula: unusual complications in patient with cystic fibrosis.

Abdominal pain, vomiting, and obstipation often occur in children and young adults with cystic fibrosis (CF). The common causes include meconium ileus equivalent, intussusception, and adhesions from previous surgery. One of our patients with CF who had meconium peritonitis as a neonate presented with duodenal obstruction during childhood. This was caused by colonic polyps arising in the hepatic flexure and eroding through the colonic and duodenal walls into the duodenum. She was treated with total parenteral nutrition, right colectomy, gastric diversion, and a controlled duodenal fistula that healed uneventfully. She has remained well 1 year after discharge from the hospital. To our knowledge, a similar case has not been reported previously.

Extracorporeal life support for the treatment of adult respiratory distress syndrome after burn injury.

The pediatric population comprises 38% of hospital admissions for burns in the United States. In the age group of 1- to 14-year-olds, a 62% total body surface area burn represents the median lethal dose and carries a lower mortality rate for burn size than in infants or adults. Adult respiratory distress syndrome (ARDS) is a common accompaniment to severe burn injury. Mortality rates of 50% to 80% are expected once ARDS occurs. This is a report of an 11-month-old boy who had fulminant ARDS after a 32% total body surface area second degree burn. After conventional therapy with maximum mechanical ventilatory support failed, salvage therapy with extracorporeal membrane oxygenation (ECMO) was instituted. ECMO was successfully terminated after 28 days. Open lung biopsy specimens obtained before instituting ECMO and on ECMO day 26 exhibited severe but histologically reversible lung disease and improved alveolar aeration as a result of treatment. This is the first reported survival of a pediatric patient with thermal injury and ARDS by using ECMO for the treatment of respiratory failure.

Paracentesis as an aid to the diagnosis of intestinal gangrene: experience in 50 infants and children.

We performed paracentesis or peritoneal lavage on 50 seriously ill infants and children in whom the diagnosis of intestinal gangrene or perforation was suspected. Thirty-four infants had necrotizing enterocolitis and 16 had other conditions. In infants with suspected intestinal gangrene, the presence of brown peritoneal fluid and/or bacteria on Gram's stain was indicative of intestinal gangrene. In infants with pneumoperitoneum, the presence of cloudy fluid with leukocytosis was indicative of gastrointestinal perforation. Using these two criteria, the accuracy of paracentesis in predicting the need for operation was 90%. When combined with clinical judgment, the accuracy rose to 97.5%. The rate of negative findings from abdominal explorations was 5%. Analysis of the peritoneal fluid may improve the timing and accuracy of the operative decision.

Stunned myocardium during extracorporeal membrane oxygenation.

The "stunned myocardium" is a syndrome of reversible myocardial dysfunction that may be mediated by oxygen-derived free radicals. This phenomenon has been seen in some neonates undergoing extracorporeal membrane oxygenation. We performed echocardiograms and measured creatine phosphokinase isoenzymes and lipid peroxide levels in 16 neonates before, during, and after extracorporeal membrane oxygenation. Infants who developed stunned myocardia by echocardiography did so shortly after initiation of bypass and exhibited concurrent elevations of the MB fraction of creatine phosphokinase. Lipid peroxide levels did not simultaneously rise. These data suggest that oxygen-derived free radicals may not cause the stunned myocardium seen in neonates undergoing extracorporeal membrane oxygenation.

Gastrointestinal malignancies in infancy, childhood, and adolescence.

Gastrointestinal and hepatobiliary malignancies in the pediatric age group are rare tumors and are usually treated with frustratingly poor results because of late diagnosis. Current efforts to improve treatment are directed at earlier diagnosis of these tumors in combination with aggressive surgery and adjuvant therapy.

Persistent pulmonary hypertension of the newborn infant. Comparison of conventional versus extracorporeal membrane oxygenation in neonates fulfilling Bartlett's criteria.

Persistent pulmonary hypertension of the newborn (PPHN) is a challenge for the neonatologist and a common indication for treatment with extracorporeal membrane oxygenation (ECMO) when medical management fails. We observed 132 neonates born between January 1985 and December 1988 with the diagnosis of persistent pulmonary hypertension of the newborn: 73 (55%) met the Bartlett criteria for treatment with ECMO with 80% predicted mortality; 21 (29%) deteriorated despite conventional medical treatment, were thought to be dying, and were sent for ECMO. Among the 52 patients who were medically treated 40 (77%) survived, a marked difference compared with a predicted 20% survival. All ECMO-treated neonates survived. Although conventionally treated infants showed a trend toward less dependence on supplemental oxygen at > 28 days of life, this study failed to detect a significant difference between those two groups. We conclude that mortality was lower for ECMO-treated infants than for those who were medically treated (0 of 21 vs 12 of 52, p < 0.05); mortality for infants with persistent pulmonary hypertension of the newborn who met Bartlett's criteria and were medically treated was lower than published data; and there was no significant difference in oxygen dependence at > 28 days between the survivors who received ECMO and those who received medical therapy.

Neonatal presentation of cystic fibrosis requiring ECMO.

This is a case of cystic fibrosis causing respiratory distress in a newborn who was treated successfully with extracorporeal membrane oxygenation (ECMO). To date, this is the only such case reported to the Neonatal ECMO Registry at the University of Michigan. ECMO can be a life-saving intervention in these neonates. Advances in the total care of these patients make them acceptable candidates for neonatal ECMO, but the patholophysiology of cystic fibrosis raises many questions about their suitability for ECMO at older ages, as the associated pulmonary disease becomes less reversible.

Protective effect of residual splenic tissue after subtotal splenectomy.

Studies in animals and clinical experience in man have demonstrated that splenectomy leads to increased susceptibility to infection with encapsulated bacteria. Splenic tissue has an excellent ability to regenerate, even when implanted into subcutaneous tissue or the abdominal cavity. These implants, however, do not protect against bacterial challenge despite the fact that a number of other functions can be restored. We therefore studied the ability of residual splenic tissue to protect against challenge following subtotal splenectomy in Sprague-Dawley rats. Subtotal splenectomy was performed on 48 animals in which approximately 75% of the spleen was removed and left with a branch of its normal blood supply; 48 animals underwent total splenectomy and 48 had sham operations. Six months after surgery the groups were challenged intravenously with type 25 pneumococci to determine the LD50 for each group. Animals that had undergone subtotal splenectomy were more resistant to pneumococcal challenge than were asplenic animals, but they were not as resistant as normal animals. In addition, there was marked delay in death in the animals with subtotal splenectomy as compared with asplenic animals. Thus residual splenic tissue after subtotal splenectomy appears to confer some degree of protection against pneumococcal challenge.

The effect of age and postsplenectomy interval on the susceptibility of the asplenic adult rat to pneumococcal challenge.

The response to intravenous challenge with pneumococcus was examined in asplenic and sham-operated adult Sprague-Dawley rats eighteen weeks following surgery. The LD50 was over 2,0000 fold greater in the sham-operated animals than in the asplenic animals. These results suggest that the age of an asplenic host, the length of time following splenectomy, and the presence of a normal immune system other than the asplenic state do not in themselves confer immunity.

Early diagnosis and treatment of Hirschsprung's disease in New Mexico.

Four decades ago, the mortality of congenital aganglionosis of the distal part of the colon (Hirschsprung's disease) approached 100 per cent. The development of diagnostic methods and curative operations lowered the mortality to 5 to 10 per cent. Enterocolitis remained the major cause of death. In this series of 18 children with Hirschsprung's disease, the majority were diagnosed as neonates. Although enterocolitis was present at diagnosis in four (22 per cent), all children survived after prompt surgical therapy. Neonatal diagnosis and treatment may prevent the development of fatal enterocolitis, virtually eliminating the mortality of Hirschsprung's disease.

The protection of small amounts of splenic tissue against intravenous pneumococcal challenge following subtotal splenectomy.

The asplenic state increases susceptibility to overwhelming bacterial infection. We studied the ability of small amounts of splenic tissue to protect against intravenous pneumococcal challenge following subtotal splenectomy in which approximately 75% of the spleen was removed. Animals that had undergone subtotal splenectomy were more resistant to challenge than were asplenic animals, but were not as resistant as were control animals. The small amount of residual splenic tissue confers some degree of protection against intravenous bacterial challenge.

Repair of agenesis of the hemidiaphragm by prosthetic materials.

Creation of a substitute hemidiaphragm for an infant born with agenesis of the hemidiaphragm is a formidable task. Subsequent growth of the infant may produce distortion or fracture of diaphragmatic prostheses. Our experience with a surviving two year old infant with agenesis led us to conclude that a Silastic prosthesis is unsatisfactory and that a polypropylene mesh prosthesis is satisfactory as a diaphragmatic substitute.

Haemophilus influenzae b infection in rats: effect of splenectomy on bloodstream and meningeal invasion after intravenous and intranasal inoculations.

We investigated the effect of splenectomy on the susceptibility of rats to intravenous or intranasal inoculation of Haemophilus influenzae, type b. The 50% lethal dose for asplenic rats inoculated either by intravenous (i.v.) (10(4.7)) or intranasal (i.n.) (10(4.6)) injection was similar, but significantly lower than the 50% lethal dose value in sham-operated rats (10(8.6) i.v. and 10(9.0) i.n.). Mean survival time was significantly longer for asplenic rats inoculated i.n. (49.3 h) compared to asplenic rats inoculated i.v. (24.4h). Similarly, sham-operated rats inoculated i.n. survived significantly longer after i.n. challenge (mean survival time, 171.4 h) than after i.v. challenge (34.7 h). Bacteremia was detected in 100% of asplenic rats and in 80% of sham-operated rats. The geometric mean number of bacteria in the blood of asplenic rats (10(4.90) per ml) was significantly greater than in sham-operated rats (10(3.29) per ml). Meningitis was detected in 7 of 15 randomly sacrificed asplenic rats, whereas none of 15 sham-operated rats had evidence of meningeal invasion. Thus, the asplenic rat was more susceptible to experimentally induced H. influenzae bacteremia, meningitis, and fatal sepsis and offers a biologically relevant experimental model for investigating the role of the spleen in defense against infection with encapsulated bacteria.

Treatment of precocious pseudopuberty associated with follicular cysts of the ovary.

Two girls, aged 3 and 6 years, were treated for precocious pseudopuberty associated with a follicular ovarian cyst. Both patients had breast enlargement, areolar pigmentation, and elevated serum estradiol levels. In one girl, a normal displaced uterus was palpated; in the other, an ovarian cyst was identified by ultrasonography. Surgical treatment consisted of limited resection of the follicular cysts. Rapid regression of breast hypertrophy and return of estradiol levels to normal occurred in both children, who were followed for six and 1.5 years. We believe that a conservative operation that preserves normal ovarian tissue bilaterally is the procedure of choice for treating this condition.

Lack of protective effect of autotransplanted splenic tissue to pneumococcal challenge.

Studies in animals and clinical experience in patients have demonstrated that splenectomy may lead to an increased susceptibility to infection. The infections are usually caused by encapsulated bacteria such as penumococcus. It has been shown in a variety of experimental animals that autotransplanted splenic tissue is capable of regenerating into implants that are microscopically indistinguishable from normal spleen and of restoring a number of normal splenic functions. The response to intravenous challenge with Streptococcus pneumoniae, type 25, was therefore studied in control, asplenic, and autotransplanted Sprague-Dawley rats. Despite previous observations that a number of immune functions can be restored in this animal model by autotransplanted splenic tissue, the present study indicates that splenic tissue autotransplants do not restore the ability to resist intravenous pneumococcal challenge.