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More precise analysis of causes of death is needed to focus research efforts and improve morbidity and mortality in sickle cell disease. In this study, the morphological evidence of the cause of death was studied in 306 autopsies of sickle cell disease, which were accrued between 1929 and 1996. The most common cause of death for all sickle variants and for all age groups was infection (33-48%). The terminal infection was heralded by upper respiratory tract syndromes in 72.6% and by gastroenteritis in 13.7%. The most frequent portal of entry in children was the respiratory tract but, in adults, a site of severe chronic organ injury. Other causes of death included stroke 9.8%, therapy complications 7.0%, splenic sequestration 6.6%, pulmonary emboli/thrombi 4.9%, renal failure 4.1%, pulmonary hypertension 2.9%, hepatic failure 0.8%, massive haemolysis/red cell aplasia 0.4% and left ventricular failure 0.4%. Death was frequently sudden and unexpected (40.8%) or occurred within 24 h after presentation (28.4%), and was usually associated with acute events (63.3%). This study shows that the first 24 h after presentation for medical care is an especially perilous time for patients with sickle cell disease and an acute event. Close monitoring and prompt aggressive treatment are warranted.

Atlanto-occipital dislocation occurs more often in children due to the laxity of the ligamentous structures anchoring the occiput to the axial skeleton. The mechanism of action usually involves a sudden acceleration-deceleration force on the head of the child. The dislocation usually severs the spinal cord at the foramen magnum, resulting in acute respiratory arrest. We have managed four patients who sustained this injury and arrived at our trauma center with signs of life. Two patients were hemodynamically unstable, had positive diagnostic peritoneal lavage, and underwent splenectomy. Both patients had obvious separation of the occiput and C1 on lateral cervical spine films. Both remained very unstable and died soon after celiotomy. The other two patients were stabilized, and both met criteria for brain death; one family agreed to organ donation. A 5-year analysis revealed 57 pediatric deaths, with 10 patients sustaining atlanto-occipital dislocations (17.5%). Nine of 10 patients sustained other injuries, but in only 2 patients were the injuries immediately life-threatening. With continued improvement in emergency medical systems and pediatric trauma care, we can expect to see more pediatric patients with this injury arriving in trauma centers with signs of life. In our experience, 50 per cent of patients may meet organ donor criteria, and our incidence of this injury (17.5%) reveals atlanto-occipital dislocation as a major contributor to pediatric trauma mortality.

Background/Purpose: Hypothermia (HT) remains a significant stress to the newborn and has been implicated in the pathogenesis of necrotizing enterocolitis (NEC). The authors assessed the effect of transient HT (32°C) on regional organ blood flow in anesthetized piglets at age 7 to 10 days preterm (PREM), 1 to 2 days (NB), and 1 to 2 weeks (NEO). Methods: Radiolabeled microspheres were used to determine organ blood flows (mL/min/g) at baseline, 15, and 60 minutes after HT and 60 minutes after rewarming to baseline core temperature. Results: Heart rate and cardiac output decreased significantly in all groups. Cardiac flow decreased significantly in the NEO group, and central nervous system (CNS) flow decreased significantly in the NB and NEO groups. Both returned to baseline levels after rewarming. The PREM group experienced decreased cardiac, CNS, and intestinal blood flows but not to significant levels. NB and NEO intestinal blood flow showed significant decreases, which remained so after rewarming (a response not seen in hypoxia or hypovolemia). Cardiac output did not return to baseline levels in any group. Conclusions: HT causes derangements in organ blood flows that differ from other deleterious stimuli such as hypoxia and hypovolemia. The prolonged intestinal ischemia supports HT as a factor in the development of NEC. This delay may offer opportunity to intervene in an attempt to lessen ischemiareperfusion injury.

Acute hemorrhage in premature infants occurs as the result of obstetrical complications, birth trauma, or operative procedures. This study evaluates the response of the preterm piglet to acute hemorrhage and resuscitation. Piglets delivered by Caesarean section 7-14 days preterm underwent acute arterial/venous hemorrhage (20 cc/kg). At 0, 15, and 60 rain after hemorrhage, hemodynamic parameters and regional blood flows (reference organ technique) were measured. Animals were then resuscitated with shed blood (20 cc/kg), crystalloid (60 cc/kg), or colloid (dextran 40, 20 cc/kg) with study parameters obtained 30 min later (statistical analysis by ANOVA). Significant decreases in blood pressure (BP) and cardiac output (CO) occurred after hemorrhage. BP and CO were reestablished to near control levels following resuscitation with blood and crystalloid. Dextran failed to return BP to baseline; however, it resulted in CO 1.7 × control. Heart and CNS blood flow were not significantly influenced by hemorrhage; however, dextran increased flows to these organs significantly above control levels. In the kidney and small bowel, flows decreased significantly following hemorrhage; blood and crystalloid restored flows to near baseline, while dextran resulted in flows significantly greater. In summary, the preterm piglet responds to hemorrhage with maintenance of blood flow to the heart and brain and significantly decreased flows to the kidney and small intestine. Flows following resuscitation with blood and crystalloid are comparable to those in control. Dextran resuscitation resulted in flows significantly greater than baseline; this response might be detrimental with ongoing hemorrhage and/or prolonged ischemia.

OBJECTIVE: The authors determined the effects of dopamine and dobutamine on organ blood flow in newborn piglets. SUMMARY BACKGROUND DATA: Although the hemodynamic effects of dopamine and dobutamine are well described in adults, little is known of their consequences in neonates, and their impact on organ perfusion in premature infants is unclear. METHODS: Cannulae were placed in the femoral vessels and left atrium of term (1-14 days old) and prematurely delivered (Ceasarean section at 90% of term gestation) piglets. After stabilization, radiolabeled microspheres were injected. A continuous infusion of dopamine or dobutamine was started, and other microspheres were injected at 5, 10, and 15-micrograms/kg/minute drug doses (allowing a 20-minute equilibration period at and between each dose). Blood flows (mL/minute/g tissue) to organs were determined using reference organ techniques. RESULTS: In term animals only, dobutamine and dopamine increased systemic mean arterial pressure at the 15-micrograms dose. In term piglets, dobutamine produced dose-dependent increases (p < 0.05, analysis of variance) in heart and brain blood flow, although small intestinal blood flow decreased (1.47 +/- 0.13 mL/minute/g baseline to 1.31 +/- 0.11 mL/minute/g at 15-micrograms dose). There were no significant changes in blood flow to these organs in preterm animals. Dopamine significantly (p < 0.05) increased heart blood flow in both groups. Dopamine also increased small intestinal blood flow in term (1.63 +/- 0.22 mL/minute/g baseline to 3.13 +/- 0.34 mL/minute/g at 15-micrograms dose) and premature (0.31 +/- 0.10 mL/minute/g baseline to 1.11 +/- 0.29 at 15 micrograms dose) piglets. CONCLUSIONS: Dopamine may prove a valuable adjunct when a premature infant is at risk for conditions that reduce alimentary tract blood flow.

Newborn piglets (aged 1 to 2 days and 7 to 14 days) were used to study (1) the redistribution of organ blood flow after a 25% acute blood loss and (2) the response to resuscitation with shed blood (20 mL/kg), crystalloid (normal saline [NS] or lactated Ringer's [LR]; 60 mL/kg), and colloid (dextran-40, 20 mL/kg). Hemodynamic parameters showed little differences in the response to hemorrhage and resuscitation. The two age groups had no significant differences in parameters or blood flow (results combined). The animals maintained flow to the heart and central nervous system (CNS) and had significantly decreased flow to the kidneys and splanchnic organs. In the gastrointestinal tract, the small intestine was affected most severely, with a significant decrease in blood flow, especially to the mucosa. In all organ systems, Dextran 40 restored blood flow to levels significantly above the baseline. Shed blood and crystalloid restored flow to organs sustaining decreased flow, but crystalloid did not restore flow to the baseline level in the kidney and all segments of the gastrointestinal tract.

Acute splenic sequestration crisis (ASSC) in children with various forms of sickle cell disease can result in life-threatening circulatory collapse due to the loss of circulating blood volume. Over a 6-year period we have treated 12 patients ranging in age from 5 1/2 months to 7 years presenting with acute sequestration crisis. Eleven had homozygous sickle cell disease and the other had sickle-thalassemia. One patient died of acute circulatory collapse. Eight patients underwent splenectomy after a major episode of sequestration with no serious infectious complications up to 5 years following splenectomy. Three patients with minor episodes have been followed with no recurrences. To foster early detection of this potentially lethal complication of sickle cell disease, an educational program in our Comprehensive Sickle Cell Center instructs the parents to examine the spleen and bring their child in for evaluation if the spleen enlarges. A newly developed videotape describes the common symptoms of ASSC and illustrates the technique of palpating the spleen. With early detection of sickle cell disease by neonatal screening and the educational program, the morbidity and mortality from this complication of sickle cell disease can be reduced.

Ischemia-reperfusion injury has been implicated as playing a major role in the development of necrotizing enterocolitis, a major cause of morbidity and mortality in the newborn. A tungsten-supplemented molybdenum-free diet can reduce xanthine oxidase (XO) enzyme activity in the intestine, which in turn reduces the generation of oxygen radicals after an ischemia-reperfusion insult. This study evaluated the ability of this diet to be effective by indirect means, ie, transplacental and breast-feeding routes. XO activity of the intestine was measured in three groups of CD-1 white rats: I, weanlings fed the tungsten diet or standard chow for 1 week; II, 1-day-old rat pups whose mothers were maintained on the tungsten or standard chow for 7 to 10 days prior to term; and III, rat pups at 1 and 3 weeks after birth whose lactating mothers were maintained on the tungsten or standard chow. Some animals from group III also underwent either a 30- or 60-minute episode of occlusion of the superior mesenteric artery (SMA) to evaluate the protective effects of the diet. XO activity was significantly reduced in all groups receiving the tungsten diet (P less than .0001). Blinded histopathologic studies of the entire small bowel showed significantly less villar necrosis (P less than .05) and fibrosis (P less than .0001) in the tungsten-treated group than in the controls. In the 60-minute occlusion study all tungsten-group animals survived, whereas 7 of 12 in the control group died of intestinal infarction within 24 hours (P less than .001).(ABSTRACT TRUNCATED AT 250 WORDS)

Hepatoblastoma is the most common primary liver tumor in children, accounting for 79% of pediatric liver malignancies in children younger than 15 years, with most cases reported before the age of 5 years. Localization of primary and recurrent disease is necessary for appropriate clinical decision-making and treatment. We present a case of recurrent hepatoblastoma heralded by rising alpha-fetoprotein levels. After unsuccessful localization by conventional CT and MRI, positron emission tomography CT imaging localized the sites of recurrence.

Context.—Magnetic resonance imaging of bone marrow in homozygous sickle cell disease (hemoglobin [Hb] SS) shows nonhomogeneous, mottled signals that increase with age and number of crises. The pattern of these signals is reminiscent of the underlying vascular architecture, but histopathology of this tissue has not been adequately studied.Objective.—To elucidate the histopathology of blood vessels in the bone marrow in sickle cell disease.Design.—Retrospective histochemical morphometric study of bone marrow arteries by point counting in HbSS (13 cases) and sickle cell Hb C (HbSC) (8 cases) compared to nonanemic normal controls (HbAA) (10 cases). All patients were nondiabetic, normotensive, younger than 37 years, and matched for age group.Results.—The mean point count for perivascular fibrous tissue was significantly greater in the HbSS group (P < .001) in both small (P < .001) and medium-sized (P = .002) vessels, and in both age groups (pediatric, P < .001; adult, P = .005) compared with the HbAA group. Additional analysis showed the difference was significant in HbSS pediatric small vessels (P < .001) and in pediatric and adult medium vessels (P = .045 and P = .03, respectively). Ratios of fibrous tissue to muscle showed proportionately greater fibrous tissue in HbSS pediatric small (P < .001) and medium-sized vessels (P = .02), and in adult large vessels (P = .03). Mean point counts for muscle were significantly decreased in HbSS small vessels when all ages were compared as a group (P = .02), but when compared by age groups, counts were significantly increased in adult HbSS medium-sized vessels (P = .01). Overall mean point counts for muscle and fibrous tissue in the HbSC group were intermediate between those of the HbSS and HbAA groups, but were not significantly different from counts in the HbAA group (P = .78 and P = .35, respectively).Conclusion.—In sickle cell disease, arterial vessels in the bone marrow show significantly increased fibrous connective tissue and changes in muscle that vary with age and vessel size.

Intradural extension of a sacrococcygeal teratoma (SCT) is extremely rare and only well-documented in presacral tumors that have been associated with a familial history, anorectal stenosis, and sacral dysraphism. This case documents the extension of a type I SCT into the dural sac with attachment to the filum terminale. A full-term female was transferred to our tertiary newborn intensive care unit with a sacral mass measuring 12 × 13 cm. It protruded from the buttocks and displaced the anus anteriorly. Rectal examination showed no presacral component. Radiographs demonstrated calcification in the soft tissue mass and a normalappearing sacrum with the last sacral segment not visualized. At operation during dissection of the cephalad component, the SCT extended into the spinal canal. Neurosurgical consultation resulted in a sacral laminectomy which revealed the tumor to be attached to the tip of the filum terminale. The tumor was removed in toto with all sacral roots preserved. The infant required a second operation to revise a wound dehiscence and suspected cerebrospinal fluid leak. The final pathology report was benign SCT. Follow-up at 2 years showed no recurrence, normal sphincter tone, and a normal computed tomography scan. This represents the first well-documented intradural extension of a Type I SCT with attachment to the spinal cord. This extremely rare occurrence requires awareness with the availability of neurosurgical support to expedite operative management.

Over a 5-year period, 20 infants of multiple-gestation births (16 twin, 2 triplet) developed necrotizing enterocolitis (NEC) (15 infants) or suspected NEC (5 infants). During the same period, 532 infants of multiple gestations were admitted to our neonatal intensive care unit, yielding a NEC incidence in this population of 3.8%. In two twin sets, both infants developed NEC or suspected NEC, and in three sets only the affected twin was transferred to our nursery. Five infants required surgical intervention (25%) and three infants died (overall mortality, 15%). Fifteen siblings who did not develop NEC served as a control group. Analysis showed that the 1-minute Apgar score was the most significant factor in predicting NEC (P less than .028) and need for surgical intervention (P less than .020). In this series, 82% of the infants with 1-minute Apgar less than 6 developed NEC, whereas 31% with 1-minute Apgar greater than 6 developed NEC.

This study was designed to determine the effects of severe hypoxemia on newborn piglet visceral blood flow. While the hemodynamic effects of a severe hypoxemic insult are well characterized in newborn animals, its impact on organ perfusion in premature infants is not well characterized. Cannulas were placed in the femoral vessels and left atrium of term (1-14 days old) and prematurely delivered (cesarean section at 90% of term gestation) piglets. After stabilization, some animals were subjected to 1 h of ventilator-controlled hypoxia (yielding PaO2 approximately = 30-40 torr) followed by 30 min of reoxygenation; the remaining animals served as unchallenged controls. Radiolabeled microspheres were injected in all animals at times 0 min (baseline), 5 and 60 min (hypoxia), and 90 min (reoxygenation). Blood flows (mL/min/g tissue) to organs were determined using reference organ techniques. Control animals displayed no alterations in any of the variables monitored. Throughout the experimental period, organ blood flows were almost uniformly lower (p.05, ANOVA) in premature versus term animals. The trend toward increased cerebral and cardiac blood flows during hypoxia observed in the premature piglets was similar to that of term animals, but of lower magnitude. In term piglets, hypoxia produced an immediate and significant (*p.05) decline in small-intestinal blood flow followed by autoregulatory escape (2.02+/-0.17 mL/min/g at time 0, 1.56+/-0.15 mL/min/g at 5 min hypoxia, 1.88+/-0.18 mL/min/g at 60 min hypoxia, 2.26+/-0.19 mL/min/g at 30 min reoxygenation), an effect not readily observed in the premature piglets (0.48+/-0.10 mL/min/g at time 0, 0.44+/-0.07 mL/min/g at 5 min hypoxia, 0.46+/-0.10 mL/min/g at 60 min hypoxia, 0.42+/-0.08 mL/min/g at 30 min reoxygenation). However, mucosal blood flows measured in these younger animals declined throughout the experimental period to almost 50% of baseline, compared to a complete restoration to baseline blood flow observed following reoxygenation of term piglets. Intestinal blood flow in premature infants is small when compared to term animals, and alterations in small intestinal blood mucosal flow induced by hypoxia appear less well tolerated by the premature animals. Taken together, this may in part account for the increased risk of developing intestinal ischemic diseases in premature infants who are even temporarily exposed to a severe hypoxic challenge.

Stapled anastomoses of the gastrointestinal tract, commonplace in adults and older children, are rarely performed in newborns because of the size of the instruments. The development of smaller stapling devices for laparoscopic and thoracoscopic procedures presented an opportunity to evaluate the efficacy of such anastomoses in neonates and infants. Over an 11-month period, seven newborn or young infants underwent stapled functional end-to-end small or large intestinal anastomoses using the Endo-GIA 30. The male:female ratio was 3:4, and the mean age was 72d (range, 1 to 134 days). The mean weight at the time of the stapled anastomosis was 3.7 kg (range, 1.88 to 6.6 kg). The clinical setting requiring the intestinal anastomoses included ileostomy closure after perforated necrotizing enterocolitis (3), intestinal atresia (1), distal ileal volvulus (1), left colon mesenteric cyst (1), and biliary atresia (1). There were no deaths or anastomotic leaks. Nasogastric decompression was discontinued at a mean of 6 days (range, 3 to 11 days), and oral feedings were begun at a mean of 7.0 days (range, 4 to 12 days). The patients were discharged from the hospital 7 to 117 days after the operation (mean, 33 days). The endoscopic stapling instrument is safe and effective in performing small and large intestinal anastomoses in newborns and young infants.

This study was designed to determine whether a pregnant sow could be maintained under conditions of prolonged general anesthesia in order to provide access to prematurely delivered piglets for purposes of acute experimentation. Fifteen time-dated pregnant (90% of gestation) sows were preanesthetized with intramuscular xylazine (Rompun Mobay Pharmaceutical, Shawnee, KS)/ketamine (Aveco Pharmaceutical, Fort Dodge, IA). After anesthesia was induced with intravenous sodium thiopental, the animals underwent tracheotomy and mechanical ventilation. The electrocardiogram was monitored externally; systemic arterial pressure was supervised by the insertion of a brachial artery cannula; and urine output was determined by inserting a cystostomy tube. A surgical plane of anesthesia was achieved by the "balanced" technique: an inhalant, isoflurane, supplemented by intravenous doses of sufentanil citrate, propofol (Stuart Pharmaceuticals, Wilmington, DE), and vecuronium. Sows were maintained under general anesthesia for an average of 12 h; piglets were delivered, one or two at a time, by a modified cesarean section technique. No sows or piglets were lost to anesthetic complications. It is concluded that, with appropriate precautions, pregnant sows can be anesthetized for a prolonged period of time, and provide viable premature neonates for acute experimentation.

Indomethacin (IND), widely used in premature infants to effect nonoperative closure of patent ductus arteriosus (PDA), has been implicated in gastrointestinal tract (GI) perforations and necrotizing enterocolitis (NEC). The vasoactive effects of IND could simultaneously affect regional blood flow distribution, specifically a decrease in intestinal blood flow. This study determined blood pressure (BP), heart rate (HR), cardiac output (CO), total peripheral resistance (TPR) and regional blood flows (mL/min/g) at baseline, 15, 60, and 120 minutes after intravenous infusion of IND (0.4 mg/kg) in three groups: preterm piglets delivered 7 to 10 days before term; 1- to 2-day-old piglets; and 7- to 14-day-old piglets. IND did not significantly affect hemodynamic parameters (BP, HR, CO, TPR) or regional blood flows to the heart, central nervous system, kidney or GI tract in the premature animals. In 1- to 2-day-old animals, a significant increase in BP and TPR occurred at 120 minutes, with significant decreases in blood flow to the GI tract in the esophagus, stomach, and rectosigmoid. In the 7- to 14-day group CO significantly decreased while TPR increased. Significant decreases in blood flow occurred throughout the GI tract, most pronounced in the small intestine and colon, essentially due to decreased mucosal blood flow. Our study indicates that indomethacin can cause selective GI tract mucosal ischemia, and that the effect is increased in the more developed animal. This effect on mucosal blood flow suggests the GI tract disturbances seen after IND administration are due to an ischemic injury to mucosa previously affected by ischemia-reperfusion injury from other stresses.

Eighteen very low-birthweight infants with necrotizing enterocolitis (NEC) underwent periodic neurodevelopmental testing until two years of age. They were grouped according to whether they had had surgical intervention (N = 6) or non-surgical therapy (N = 12). At eight and 15 months corrected age, a significantly greater number of the surgical group exhibited motor delay. At 24 months, only one infant in the surgical group had persistent motor delays. These findings indicate a higher incidence of motor delays in infants requiring surgery for NEC. The delays involved skills influenced by the abdominal musculature. These motor delays persisted up to 24 months, but appeared to resolve with time.

Methods for evaluating intestinal ischemia include standard clinical criteria, Doppler ultrasonography, and intravenous fluorescein injection. Each has disadvantages, and a combination of methods is often used. The purpose of this study was to determine whether surface oximetry could be used to assess perfusion in an animal whose intestinal diameter approximates that of infants. Reversible arterial, venous, and arteriovenous occlusion was studied in rabbit intestines. After 6 to 8 hours of occlusion, intestinal vascularity was evaluated by the four methods listed. Doppler ultrasonography and surface oximetry were found to be unreliable tools for assessing perfusion in this animal model. We conclude that standard clinical criteria and fluorescein remain the standards for intraoperative evaluation of intestinal perfusion.

0 Newborn piglets (aged 1 to 2 days and 7 to 14 days) were used to study (1) the redistribution of organ blood flow after a 25% acute blood loss and (2) the response to resuscitation with shed blood (20 mL/ kg), crystalloid (normal saline [NS] or lactated Ringer’s [LR]; 60 mL/ kg), and colloid (Dextran-40, 20 mL/kg). Hemodynamic parameters showed little differences in the response to hemorrhage and resuscitation. The two age groups had no significant differences in parameters or blood flow (results combined). The animals maintained flow to the heart and central nervous system (CNS) and had significantly decreased flow to the kidneys and splanchnic organs. In the gastrointestinal tract, the small intestine was affected most severely, with a significant decrease in blood flow, especially to the mucosa. In all organ systems, Dextran 40 restored blood flow to levels significantly above the baseline. Shed blood and crystalloid restored flow to organs sustaining decreased flow, but crystalloid did not restore flow to the baseline level in the kidney and all segments of the gastrointestinal tract. Copyright Q 1994 by W.B. Saunders Company

The well documented increased susceptibility of the asplenic host to overwhelming sepsis by encapsulated organisms has led to three popular prophylactic measures in patients: 1) pneumococcal vaccination; 2) vaccination with oral penicillin prophylaxis; or 3) vaccination with penicillin use at the first sign of infection. An animal model (weanling CD-1 rats) was utilized to evaluate these prophylactic measures. One hundred sixty rats underwent splenectomy and were divided into four treatment groups: I) sham vaccination; II) pneumococcal vaccination; III) vaccination and IM penicillin 24 hours after intraperitoneal inoculation of 10(6) Streptococcus pneumoniae type III; IV) vaccination and IM penicillin starting 3 days before IP inoculation. Six days following the bacterial challenge survival rates by group were: I) 5%; II) 25%; III) 100%; IV) 100% (p less than 0.001 by phi 2 likelihood ratio). Mantel- Cox log rank analysis of survival curves yielded significant differences between all groups except III and IV (p less than 0.001). This study in weanling rats supports the use of penicillin in the asplenic host. Daily prophyactic penicillin or penicillin started at the first sign of infection appears to be equally effective. Randomized studies in humans would determine the best regimen.

Weanling CD-1 male rats were subjected to 100, 75, and 50% splenectomy. One week following splenectomy, animals received either a sham immunization with 0.1 ml NS or pneumococcal immunization with 0.1 ml of a polyvalent (23) vaccine. Eight weeks following surgery, all animals received an intraperitoneal innoculation with 10 6 Streptococcus pneumoniae Type 3 organisms and were observed for mortality. Significant differences in mortality were seen between sham and immunized animals undergoing 100 or 75% splenectomy, while in the 50% group a difference was noted which did not reach statistical significance (Mantel-Cox log rank test). Patients undergoing greater than 50% splenectomy may be afforded greater protection against overwhelming pneumococcal infections by immunization with pneumococcal vaccine.

Twenty cases of pneumonectomy in infants and children performed over a 35-yr period have been reviewed. Pneumonectomy may be necessary for inflammatory, congenital, neoplastic, and traumatic conditions. The postoperative complications of progressive mediastinal shift can be minimized by the use of a pleural prosthesis.

Nineteen cases of congenital colon atresia are presented with two deaths resulting in a mortality rate of 10.5%. The authors conclude that operative management of atresias proximal to the splenic flexure should include resection of the dilated proximal colon with primary ileotransverse colostomy or ileosigmoidostomy. Atresias distal to the splenic flexure should be managed with a colostomy with later establishment of gastrointestinal continuity.

• Controversy continues among surgeons over the management of pediatric patients with unilateral groin hernias. Currently, advocated treatment modalities include repair of only the clinically apparent side, exploration of the contralateral side when the ipsilateral hernia is repaired, or performance of contrast herniography before surgery. Another technique for consideration—diagnostic intraoperative pneumoperitoneum—is a simple, quick, safe, and highly accurate means of detecting a clinically inapparent contralateral inguinal hernia during the initial surgery. This technique, the results of its use, and a review of the literature are included. ( Arch Surg 1989;124:721-723)

A 5-year-old boy presented with dysphagia, and barium swallow revealed a narrowing in the upper esophagus. Biopsies of the circumferential lesion showed gastric mucosa. The heterotopic gastric mucosa was resected via a cervical approach. After resolution of a minor anastomotic leak, he remains asymptomatic 10 years following resection with no evidence of recurrence by esophagrams and endoscopy.

Unilateral scrotal inflammation was noted in an otherwise asymptomatic three-day-old male and was initially believed to be due to testicular torsion. It proved, however, to be the result of peritonitis from a thermometer-induced rectal perforation presenting via a communicating hydrocele. Treatment included primary closure of the rectal perforation with colostomy diversion combined with parenteral antibiotics. Preoperative evaluation of neonates with acute unilateral inflammation of the scrotum and a known hydrocele or hernia with flat and oblique x-ray films may reveal significant unsuspected intra-abdominal pathology.

Nine patients ranging in age from 3 mo to 15 yr presented with severe constipation and/or increasing incontinence. All had a huge, dilated atonic rectum and rectosigmoid demonstrated by barium enema. Six patients underwent resection of the abnormal bowel by an endorectal pull-through procedure with good to excellent results while 1 patient was corrected by a Swenson procedure.

Quantitative peritoneal lavage was performed in 52 children with blunt abdominal trauma to determine the presence of intraabdominal injuries. A strongly positive lavage was 100% accurate in diagnosing an intraabdominal injury requiring operation. A negative lavage demonstrated absence of a significant intraperitoneal injury with 100% accuracy. A weakly positive lavage was not diagnostic and required additional evaluation including intravenous pyelography echography, and arteriography. Diagnosis and treatment was prompt, and in 20 of 21 cases, operation was performed within 4 hr. Peritoneal lavage was found to be safe and much more accurate than physical examination in diagnosing significant intraabdominal injury.

The Pediatric Trauma Score was evaluated in 450 injured children by a paramedic in the field and a physician in an E.D. There was agreement between the scores of these two individuals 93.6% of the time, correlation coefficient 0.991, r2 = 0.982. Further testing at the 0.01 level of significance indicated that a positive association existed between these two variables in the population from which our sample was drawn. Mortality for the group was 2.9%. No deaths occurred in patients whose PTS was greater than 8, which was defined as the Critical Triage Point. The sensitivity of the PTS when used for triage at the critical triage point was 95.8%. The specificity of the PTS was 98.6%. The Pediatric Trauma Score appears to be highly accurate, reliable, predictable, and easy to use for assessing the severity of injury and hence is a straightforward modality for triage of injured children.

A three and one-half-year-old girl and a 12-year-old boy presented with features of the two clinical presentations of Castleman's disease or giant lymph node hyperplasia. The girl presented with anemia, fever, night sweats, hypergammaglobulinemia, and a palpable abdominal mass. Her symptoms were consistent with those seen in the plasma-cell type of this disease. The boy presented with acute appendicitis and a left hilar mass was noted on his chest radiograph. His asymptomatic presentation was typical of the hyaline-vascular form. Both patients are well without evidence of recurrence four years following resection. Castleman's disease is a benign disorder of lymph nodes that occurs rarely in children. Since the original report in 1954 we could find only 18 cases in the 16-year and younger age group and our patient with the symptomatic form represents the youngest patient reported in the English literature. The management requires surgical resection of the enlarged nodes both for diagnosis and therapy since the enlarged nodes can mimic malignant tumors of the lymphoid system. No recurrences have been reported in pediatric patients.

A two-year-old female presented with cholelithiasis. Preoperative work-up revealed no etiology. Analysis of the stones revealed the content to be pure calcium carbonate. The mother had taken a calcium carbonate supplement during the last four months of the pregnancy. This represents the first report of such an occurrence.