Lung transplantation in cystic fibrosis

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Quantitative Assessment of Intracranial Pressure by the Tympanic Membrane Displacement Audiometric Technique in Children With Shunted Hydrocephalus .... .. ..... ... ... .._............................................ 523

Neoplasms Abdominal Recurrence of Osteogenic Sarcoma . ... ... Hepatocellular Carcinoma in Children With Gardner’s Syndrome . .. ... .... ... ... .... .. ... .... ... ... ... .... .. ... .... .. Risk of Cancer Among Offspring of Childhood Cancer Survivors ... ... ... ... ... .... ... .... .. ... ... .... ... ... .. .... Unusual Presentation of Clear Cell Sarcoma of the Kidney .. ... ... ... ... ... ... .... ... ... .. .... ... .... ... .. .... ... ... ... .... .. Recombinant Human Erythropoietin in Children With Cancer ... ... ... .. .... ... .... .. .... .. .... ... ... ... ... ... ... .... .. GENERAL

Mild Hemophilia in Children: Prevalence, Treatment. L. Venkateswaran, J.A. Wilimas, Hematol/Oncol20:32-35, (January/February),

ABSTRACTS

Complications, and D. Jones, et al. J Pediatr 1998.

This report on 55 patients from St Jude’s indicates that mild hemophilia (35% of all patients) can escape clinical detection until trauma or surgery intervenes. This mild form may affect females, as it did in five patients in this review. Thirty-five of the 55 patients were identified from family history. Unusual bleeding after surgery or trauma should prompt a work-up for this possibility.-Michael P. Hirsh

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CONSIDERATIONS

Injuries and Deaths Due to Firearms in the Home. A.L. Kellerman, G. Somes, RP: Rivarn, et al. J Trauma 45:263-267, (August), 1998. The authors sought to determine the relative frequency with which guns in the home are used to injure or kill in self-defense, compared with the number of times these weapons are involved in an unintentional injury, suicide attempt, or criminal assault or homicide Three cities (Seattle, Memphis, and Galveston) were chosen for study. Of the 626 shootings in or around a residence, there were 438 assaults/ homicides (70%), 118 attempted or completed suicides (19%), and 54 unintentional shootings (9%). Only 13 shootings (2%) were an act of self-defense or legally justifiable. Handguns were used in nearly 90% of the shootings studied. For every time a gun in the home was used for self-defense or justifiable reasons, there were 4 unintentional shootings, 7 criminal assaults or homicides, and 11 attempted or completed suicides. These finding contradict advertisements that claim firearms provide effective home protection. In addition, measures to reduce home firearms and to promote safe and secure storage of firearms must be emphasized.-Steven Stylianos

Septicemia and Septic Shock in Pediatric Patients: 140 Consecutive Cases on a Pediatric Hematology-Oncology Service. A. Aledo, G. Heller; L. Ren, et al. J Pediatr Hematol/Oncol20:215-221, (May/June), 1998. This review of septic patients over a 12-month period at SloanKettering revealed several interesting observations: (1) When fever work-up showed bacteremia, the rate of development of septic shock was 19%. (2) The absolute neutrophil count (ANC) was >5OO/pL in 65% of these episodes. (3) Only 5 fungal infections were noted (4 with Candida). (4) The most common etiologic bacterium was coagulasenegative Staphylococcus. (5) 65% of patients in whom bacteremia developed had hematologic forms of cancer. (6) 46.9% of isolates contain Gram-positive cocci. (7) 40.6% were Gram-negative rods. (8) 83% of patients who required ICU admission for septic shock had Gram-negative rods. (9) There were 85.3% episodes of bacteremia per 1,000 admissions. (10) 95% of the patients had indwelling IV catheters. (11) All deaths (12%) were in patients with Gram-negative sepsis. (12) Current recommendation for antibiotic therapy is a B-lactam antibiotic combined with aminoglycosides. (13) Vancomycin is controversial for Gram-positive sepsis, but the authors do use it.-Michael P Hirsh

Spontaneous Involution of Pulmonary Sequestration in Children: A Report of Two Cases and Review of the Literature. p. Garciu-PeAa, .I. Lucaya, G. Hendry. et al. Pediatr Radio1 281266.279, (April), 1998. Pulmonary sequestration is defined as a segment of lung parenchyma separated from the trachea-bronchial tree and receiving its blood supply from the aorta. Spontaneous involution of pulmonary sequestration is exceedingly rare, and the authors present two cases of pulmonary masses with systemic blood supply, diagnosed by ultrasonography and Doppler in one case, and with contrast-enhanced CT in the other. The mass disappeared after 6 years in the first case, and showed almost complete involution after 5 years in the second. The authors reviewed the literature and raise the question as to whether all pulmonary sequestrations in asymptomatic patients should be treated surgically.A.B. Mathur Longitudinal Follow-Up of Pulmonary Function After Lobectomy in Childhood-Factors Affecting Lung Growth. C. Nakajimu, C. Kijimoto, Z Yokoyama, et al. Pediatr Surg Int 13:341-345, (July). 1998. The authors evaluated 27 patients who had undergone pulmonary lobectomy because of pulmonary cystic disease (9 lung cysts, 6 bronchial atresias, 6 congenital cystic adenomatoid malformations [CCAM], and 6 intralobar pulmonary sequestrations). Lobectomy was performed at 1 month to 10 years 8 months of age. Four patients were excluded because of very low percent vital capacity. The remaining cases were evaluated with respect to disorder, age at surgery, and occurrence of preoperative infections. Pulmonary function was evaluated once or twice per year from 1 month to 13 years after operation. The percent vital capacity (%VC) decreased after lobectomy, but normal values were regained 1 year after the operation. The percent forced expiratory volume (%FEV) 1.0 increased after lobectomy and remained high for 10 years after surgery. Residual volume to total lung capacity (RV/TLC) increased after lobectomy, but had returned to normal after 1 to 2 years following surgery. The disorder for which the patient underwent operation did not influence correlated %VC or RV/TLC, but correlated %FEV 1.0 was higher after CCAM compared with bronchial atresia. Younger patients had higher correlated %VC and lower RViTLC values than older ones. Infection preoperatively decreased correlated %VC and increased RV/TLC values in comparison to noninfected cases. The authors conclude that VC after childhood lobectomy increases to higher values than expected with respect to the remaining lung volume within 2 years. Age over 4 years and preoperative infections are the main factors restricting pulmonary function.--Z Wester Lung Transplantation in Cystic Fibrosis. E.A. Rendina, I: De Giacomo, et al. Eur J Pediatr Surg 8:208-211, (August), The authors report on 13 children in performed for cystic fibrosis. Bilateral (BSLT) was performed successfully in pneumonia and sepsis, and 12 are alive

F Venuta, 1998.

whom lung transplantation was sequential lung transplantation all patients. One patient died of and well after follow-up of 1 to

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ABSTRACTS

13 months. Blood gas analysis improved from mean values of Paos 56 mm Hg and Paces 43 mm Hg to Pa02 85 mm Hg and Pacoz 37 mm Hg. Pulmonary function test results also improved dramatically. There was also marked improvement of life quality. Ideal body weight moved from about 84% to normal values within 9 months, and the 6-minute walking test increased from a preoperative distance of 325 to 600 meters. The authors emphasize the importance of lung transplantation for patients with severe lung affection in cystic fibrosis.-Thomas A. Angelpointner

ALIMENTARY

TRACT

Oesophageal Replacement in the Management of Corrosive Strictures: When Is Surgery Indicated? E. Panieri, H. Rode, A.J.W Mi[Zu< et al. Pediatr Surg Int 13:336-340, (July), 1998. From 1976 to 1994, the authors treated 39 children with corrosive strictures of the esophagus. The mean age was 36 months, with a preponderance for boys. The strictures were dilated every 10 to 14 days for an average of 17 dilatations per patient. Twenty-three patients had strictures that did not respond appropriately to dilatations and required surgical treatment. In the remaining 16 cases, dilatations were sufficient. Dilatations were less efficient when the patient presented late. Patients with strictures longer than 5 cm also had unfavorable results with dilatations. In 22 patients, colonic interposition was performed. In one case it was possible to resect a limited stricture and anastomose the esophagus. Surgery was undertaken an average of 351 days after initiation of dilatations. The most serious complication of dilatation was perforation of the esophagus, which was encountered in 18% of the cases. Five of these 7 patients eventually required esophageal replacement. Respiratory tract infections were common. Four patients underwent antireflux procedures, two of whom eventually required substitutions Six cases had severe pharyngo-esophageal bums, all of which eventually needed colonic interposition. The authors conclude that dilatations are the basis for the management of corrosive strictures. The present study defines several factors that may be unfavorable with regard to the outcome of dilatations--P Puri

Gastric Necrosis Pelizzo, R. Dubois, (July), 1998.

in Newborns: A. Lapillonne,

A Report of 11 Cases. G. et al. Pediatr Surg Int 13:346-349,

The authors report 11 neonatal cases of gastric necrosis. Gestational age ranged from 34 to 40 weeks. Age at perforation varied from a few hours to 2 days after birth. The 4 full-term babies presented with gastric bleeding before perforation; the seven premature cases presented with gastric distension. The premature patients had asphyxia and required vigorous resuscitation; the full-term cases had only a brief asphyctic period. Abdominal radiographs showed gastric dilatation in all cases. Seven cases had pneumoperitoneum and underwent laparotomy. Another patient was operated on because of clinical deterioration. All patients had intraperitoneal hemorrhage as well as blood in the duodenum and small bowel. At surgery, total gastrectomy was performed in one case, subtotal gastrectomy in three cases, and segmental gastric resection in four. Three patients died within 12 hours of operation from multiorgan failure. Reperforation occurred in one case. The lesions were assessed endoscopically in six babies. In the conservatively treated cases, endoscopy was repeated after 1 week, when mucosal regeneration was observed. It is concluded that gastric necrosis is a distinct clinical entity in neonates in severe distress. Gastric dilatation is a typical sign that should lead to close clinical observation and regular abdominal radiographs. Surgery should be undertaken for signs of perforation and clinical deterioration.--Z Wester

Peritonitis Following Percutaneous Gastrostomy Management Guidelines. C.P Kimber; I.U. Khattak, al. Aust N Z .I Surg 68:268-270, (April), 1998.

in Children: E.M. Kiely, et

This article describes the incidence of peritonitis after percutaneous gastrostomy (PEG). The study is a retrospective case note analysis of 130 PEGS (120 children) inserted between 1990 and 1995. The primary indications were inability to swallow (n = 74). inadequate caloric intake (n = 30), and special feeding requirements (n = 16). Forty-three percent of the children were neurologically impaired. Eight children had peritonitis after PEG insertion (6.6%), four after the initial insertion (immediate group) and four after tube change (delayed group). Seven of the eight were neurologically impaired. All four in the immediate group went to laparotomy. Two children were found to have gastrocolic tistulas, one child had a torn gastroepiploic artery with a leaking gastrostomy, and in the fourth child (who was immunosuppressed) no specific defect was found. This child was the only patient who died. In the delayed group, the peritonitis was attributable to a gastrocolic fistula (n = 2) or disruption of the gastrostomy (n = 2). The authors mention a fifth child with a gastrocolic fistula discovered incidentally. The authors discuss the incidence of benign pneumoperitoneum (56%) early tube dislodgement, gastrocolic listula, hemorrhage, and intraperitoneal leakage following tube changes.-Trish Davidson Ultrasonographic Follow-Up of the Healing Process of Medically Treated Hypertrophic Pyloric Stenosis. A. Yamamoto, M. Kino, T. Sasaki, et al. Pediatr Radio1 28:177-178, (March), 1998. For hypertrophic pyloric stenosis (HPS), prompt pyloromyotomy generally is the treatment of choice. The authors describe the successful treatment of four cases by giving 0.1% atropine sulphate (0.05 to 0.1 mg/kg/d) orally. All four cases had satisfied the ultrasonographic criteria for diagnosis of HPS. All infants stopped vomiting and started gaining weight about 10 days after the start of treatment. Ultrasonography was performed at weekly intervals and suggested that shortening of the pyloric canal accompanied by resumption of peristalsis plays a more decisive role in symptomatic improvement than does thinning of the pyloric muscle.-A.B. Mat/w To Tube or Not To Tube: Do Infants and Post-Laparotomy Gastric Decompression? Evatts, S.H. Ein. Pediatr Surg Int 13:411-413, (July),

Children Need A.D. Sand& D. 1998.

The authors compared 94 children who underwent laparotomy without nasogastric tube postoperatively with 94 patients who were routinely managed with a nasogastric tube. The groups were comparable with respect to age. The procedures performed in the two groups also were comparable and included colostomy opening and closure, fundoplication, gynecologic procedures, large bowel resections, the Swenson pull-through procedure, etc. Pulmonary complications were twice as common in the group treated with the nasogastric tube. Vomiting was more common among those without a nasogastric tube, as was wound dehiscence, which occurred in two cases. However, the differences with respect to postoperative complications were not statistically significant. On the other hand, time until first feeding, time to first bowel movement, and time to discharge were significantly shorter for the group without a nasogastric tube. The authors conclude that nasogastric decompression is not required to reduce the incidence of complications after laparotomy, but it may be indicated in selected cases.-P Pwi Reliability of Color Doppler and Power Doppler Sonography in the Evaluation of lntussuscepted Bowel Viability. S. Hartquinet, M. Anooshiravani, A. Vinda, et al. Pediatr Surg Int 13:360-362, (July), 1998. The authors report on two patients with intussusception who presented 24 hours after the onset of symptoms. Plain abdominal radio-