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The process of bacterial translocation (BT) after ischemia/reperfusion (I/R) injury is reported to be mediated by local mucosal factors, the effects of pancreatic enzymes, epithelial disruption, and by dysfunctional intestinal motility. Octreotide (OCT), a somatostatin analog, has been postulated to protect against BT by influencing one or more of these factors. Twenty-two formula-fed piglets (weight, 3.5 +/- 0.5 kg; age, 20 +/- 5 days) were divided into four groups: control (no drug given; no I/R; n = 6), I/R (no drug given; n = 5), I/R plus low-dose OCT (LD OCT, 0.08 microgram/kg; n = 6), and I/R plus high-dose OCT (HD OCT, 8 micrograms/kg; n = 5). All experimental subjects had nonocclusive mesenteric ischemia induced by reversible pericardial tamponade with mesenteric flow decreased to 25 +/- 5% of baseline for 5 hours followed by 15 +/- 5 hours of reperfusion. Mesenteric lymph nodes (MLN), liver, spleen, blood, and peritoneum were harvested for blind microbial analysis. None of the animals in the control group experienced translocation to the tissues tested. All of the animals in the I/R group experienced BT to the MLN. The subjects in the LD OCT and HD OCT groups experienced BT to the MLN 66% and 80% of the time, respectively. Despite the reported clinical evidence that OCT can protect the intestinal mucosa from injury and increase the clearance of bacteria from the gastrointestinal tract, in this study in which variables other than I/R known to promote bacterial translocation were eliminated, OCT failed to modify or prevent the occurrence of translocation to the MLN after I/R injury.

The role of surgery in the management of Stage IV neuroblastoma is still far from clear. Seventy-nine patients with this diagnosis presented to the Children's Hospital of Philadelphia during the 10-year period, 1977 to 1986. Four-year survival was 23%. A major resection of the primary tumor was undertaken in 54 patients. The timing of the procedure (at presentation or delayed) had no effect on survival. The patients were divided into three groups based on the extent of surgical resection: Group 1, no surgery or biopsy only (25); Group 2, complete gross resection (34); Group 3, incomplete resection with residual gross disease (20). Four-year survival was 16, 15, and 45%, respectively. The patients were then classified as favorable or unfavorable, on the basis of biological prognostic factors at presentation. When this analysis was combined with the extent of surgery it was discovered that Group 3 contained a higher proportion of favorable patients, accounting for the better survival. Within each group survival correlated with the expected prognosis. The outcome for a patient with Stage IV neuroblastoma depends on the biological characteristics of the tumor, and there is currently no evidence that these can be favorably altered by the timing or extent of surgical resection. Defining the appropriate role of surgery in the management of these patients will require a prospective randomized study which takes into account the inherent biological variability of the disease. © 1995 Wi1ey-Liss, Inc.

A liquid chromatographic method capable of separating and quantitating the stereoisomers of zalcitabine has been developed and validated. The separation was achieved with an Astec Cyclobond I—RSP column and a mobile phase of 0.25% triethylamine in water adjusted to a pH of 6.5 with glacial acetic acid. All enantiomers were found to exhibit a linear response in the range of 0.1–10% in the presence of 100% zalcitabine. Precision of analysis was found to be less than 1.5% at a level of 1% relative to zalcitabine. The limit of detection for two of the three enantiomeric impurities was determined to be 0.05% relative to zatcitabine. The detection limit for the third was found to be 0.1%. This method was successfully applied to the analysis of reference standards and several production scale batches. All of these materials were found to be stereochemically pure to a level of 99.8% or better.

Obstruction of an infant's gastrointestinal (GI) tract can occur anywhere from the esophagus to the anus. For purposes of this review, the newborn infant will be defined as an infant from birth to 30 days of age. Both congenital and acquired obstructions will be addressed. In each instance, the epidemiology, pathogenesis, clinical aspects, and management of the disorder will be considered. Esophageal Atresia EPIDEMIOLOGY AND PATHOGENESIS Esophageal atresia, or interruption of the esophagus, generally occurs in association with a tracheoesophageal fistula (EA - TEF). The most common anatomic arrangement is a blind proximal esophageal pouch that has a distal tracheoesophageal fistula (Figure 1). This is seen in 85% to 90% of infants who have this anomaly. Seen less commonly is pure esophageal atresia that does not have a tracheoesophageal fistula and tracheoesophageal fistula that does not have an esophageal atresia (H-type tracheoesophageal fistula). These latter two conditions occur in approximately 10% of newborns who have these types of anomalies. Other anatomic arrangements, such as an esophageal atresia that has a fistula between the upper pouch and trachea or esophageal atresia that has a fistula to both pouches, are seen in only a tiny fraction of these infants. EA - TEF occurs in approximately 1 in 4000 live births.

The authors previously reported that mesenteric ischemia and reperfusion (IR) in a chronic newborn piglet model creates dysfunctional intestinal motility. Whether this leads to inadequate bacterial clearance and translocation (BT) through the gastrointestinal tract remains unclear. To test this hypothesis the authors used their chronic piglet model (weight, 3.5 ± 0.3 kg; age, 18 ± 4 days; on formula feeding); nonocclusive mesenteric ischemia was induced via reversible pericardial tamponade. Mesenteric flow (SMA Doppler measurement via the retroperitoneal approach) was decreased to 25% ± 5% of baseline for 300 minutes in the ischemia group (n = 7) and followed by 14 hours of reperfusion in the IR group (n = 6). Control subjects had a sham operation (n = 7). Mesenteric lymph nodes (MLN), liver (L), spleen (S), ileum, peritoneum, and blood were harvested for blind quantitative microbial analysis. Subjects in the control group had no cultures positive for growth. Eighty-five percent of animals in the ischemia group had positive MLN cultures only ( P v control). All piglets in the IR group had positive MLN cultures ( P v control), and one third of them manifested bacteremia. Histological examination did not show mucosal disruption in any group. The validity of this model is confirmed by the negative cultures in the control group and by the presence of normal ileal flora in all animals. In the ischemia and IR groups, MLN cultures were consistently positive with gram-negative bacilli ( Escherichia coli and/or Klebsiella pneumoniae ). When subjects of the IR group had more than 1,000 colonies in the MLN, bacteremia with the translocating organisms was also identified. The overall solid organ (L&S) translocation rate was 17% in the IR group but absent in the ischemia-only group. Variables known to contribute to BT were eliminated in our model. The peritoneal cavity was not violated, subjects were studied without the influence of any drugs or anesthesia, and mesenteric ischemia was accomplished without mechanical manipulation of the SMA or hypovolemic shock. To our knowledge, this is the first report of an infant animal model that demonstrates the occurrence of enteric BT following ischemia and IR injury. We suggest that BT is mediated by the dysfunctional motility induced by IR, an abnormality that may be operant in many critically ill infants.

Twelve infants with diaphragmatic hernias plus other anomalies who had mosaicism for tetrasomy isochromosome 12p (Pallister-Killian syndrome) are reviewed. A newborn infant with a diaphragmatic hernia plus dysmorphic features and a normal peripheral blood karyotype should have chromosome analysis performed on fibroblasts or bone marrow.

OBJECTIVE: To determine the results of pediatric trauma care managed with a cooperative effort by general surgeons and pediatric intensivists in comparison to national standards. DESIGN: Retrospective chart review. SETTING: Referral level II trauma center in rural Wisconsin. PATIENTS: All pediatric trauma patients age 16 and younger admitted to the hospital from 1990 to 1993. METHODS: Demographics, mechanisms of injury, revised trauma score (RTS), injury severity score (ISS), surgical procedures, need for intensive care, and outcome were examined. All patients were primarily managed by the trauma surgery service. Those patients requiring intensive care were managed jointly by the trauma surgery service and pediatric intensivists. Outcome was predicted by TRISS analysis; patients identified as "unexpected deaths" underwent critical clinical review to determine potential for survival. RESULTS: There were 531 pediatric trauma admissions. The mean age was 9.0 +/- 6.2 (SEM) years and two thirds of the patients were boys. Over half of all injuries were from falls, recreational activities, and motor vehicle crashes. There were few penetrating injuries. The mean RTS was 7.58 +/- 0.05, and the majority of patients had an ISS below 10. Sixty-two percent of patients required surgical procedures, most of which were orthopedic. Fourteen percent of patients were admitted to the pediatric intensive care unit. There were 13 deaths for a mortality rate of 2.4%. TRISS analysis identified six deaths as unexpected. Four drownings were not included in TRISS analysis, and there were no unexpected survivors. Of the six unexpected deaths, there were no significant management errors identified on careful review. CONCLUSIONS: Cooperation between general surgeons and pediatric intensivists can result in excellent pediatric trauma care in a rural level II trauma center. Language: en

A bone marrow transplant (BMT) protocol including surgical excision, local and total body irradiation, and high dose multiagent chemotherapy based on melphalan and bone marrow rescue has been in effect for children with high risk or relapsed neuroblastoma at the Children's Hospital of Philadelphia since 1979. The initial results were reported in 1984 [August et al.: J Clin Oncol 2:609–616, 1984]. This report updates the initial results and those that followed changes in the original conditioning regimen. Forty-two patients were treated between May 1979 and November 1987, and included 27 whose disease had relapsed and 15 who received BMT as part of primary treatment. Allogeneic marrow was given to 12 and autologous marrow to 30; in 7 of these 30, the marrow was purged with monoclonal antibodies and magnetic beads. The 4-year actuarial survival rate is 29%. Ten patients died of early treatment-related complications, 18 died of progressive disease, and 2 died of late complications (1 AIDS and 1 acute myelogenous leukemia). Censoring the two late complications the actuarial 4-year relapse-free survival rate becomes 32%. The longest interval after BMT to relapse was 20 months. There was no significant difference in the survival for patients transplanted following relapse or in first remission. The better survival for patients rescued with autologous marrow (30%) is not statistically significantly different from the result with allogeneic marrow (17%). © 1994 Wiley-Liss, Inc.

Ovarian masses in children are uncommon. We reviewed all cases of ovarian masses presenting to this hospital from 1979 to 1990. Ninety-one patients fulfilled the criteria and had medical records available. All patients were less than 18 years old. Four were diagnosed antenatally. Thirty-four tumors presented prior to 8 years of age and 1 (2.9%) was malignant. Fifty-eight tumors presented after 8 years of age and 18 (33%) were malignant. Seventy-two patients had benign disease and 19 had malignant tumors. Of those with benign disease 22 had simple or epithelial cysts, 25 had teratomas, 13 had torsion with cyst formation, 3 had granulosa cell tumors, and 9 had other less common lesions. Analysis of symptoms could not distinguish between benign and malignant lesions; however, age was less (P < .03) and tumor size smaller (P < .001) in patients with benign lesions. Benign lesions presented at a mean age of 8.8 years. Fifty-four patients had an ultrasound, all were diagnostic: simple mass (14), complex mass (8), or cyst (32). Mean size of the masses was 9.5 x 7.7 cm. Fourteen patients had a contralateral ovarian cyst. The malignant lesions included 14 germ cell tumors (4 endodermal sinus, 4 teratoma, 2 choriocarcinoma, 2 dysgerminoma, 1 embryonal, and 1 mixed), 4 epithelial tumors (1 mucinous cystadenocarcinoma, papillary cystadenocarcinoma, papillary serous cystadenocarcinoma, and endometrioid adenocarcinoma), and one patient with leukemic infiltration (ALL). Germ cell tumors presented at a mean age of 11.8 years. Eight of these patients had an ultrasound and all showed a mass (7) or cyst (1).(ABSTRACT TRUNCATED AT 250 WORDS)

Hypoplastic left heart syndrome (HLHS) has been widely viewed as a uniformly fatal form of congenital heart disease. Between January 1984 and December 1990, 387 patients with the diagnosis of HLHS were treated at this institution. Mesenteric ischemia was clinically diagnosed in 31 patients (8% incidence) and confirmed by pathology or surgery in 25 of those patients. The mean age at the time of onset was 17.5 +/- 5.4 weeks and only 13% were premature newborns. In 80% of the patients a low perfusion state and significant hypotension were documented within 48 hours prior to the diagnosis of bowel ischemia. Nine patients (29%) required operative intervention (bowel resection 4, diffuse ischemia 3, and simple drainage 2). Overall, at operation or at autopsy, nine patients (29%) had diffuse gastrointestinal ischemia. Of 31 patients with mesenteric ischemia, 26 children (84%) died shortly after onset of the gastrointestinal symptoms regardless of means of management. Five patients (10%) initially improved with aggressive medical and/or surgical management; however, 4 subsequently died secondary to complications of their primary cardiac disease. Therefore, the overall mortality of patients with mesenteric ischemia was 97%. Previous reports have estimated that up to 7% of full-term newborns with symptomatic congenital heart disease may develop necrotizing enterocolitis (NEC). Our unique group of patients with HLHS is comprised mostly of full-term infants who developed onset of mesenteric ischemia at a mean age of 4 months associated with an underlying low perfusion state. This mesenteric ischemia has been erroneously identified as NEC.(ABSTRACT TRUNCATED AT 250 WORDS)

The interdigestive migrating motor complex (MMC) has been demonstrated to be a reliable indicator of intestinal motility and function. The effects of low perfusion on the MMC have never been studied. Fourteen newborn Yorkshire piglets (5 to 18 days old, weighing 2.9 +/- 0.4 kg) underwent celiotomy under general anesthesia with placement of four jejunal electrodes (50 cm apart) as well as a superior mesenteric artery (SMA) Doppler flow probe and a pericardial catheter. Group 1 (n = 5) had operation alone. Group 2 (n = 9) had nonocclusive mesenteric ischemia induced by reversible cardiac tamponade for 5 hours between postoperative days 6 to 12. All subjects had MMC phase III electrical activity, cycling time, and propagation velocity recorded daily. In group 2 MMCs were recorded prior to and during ischemia, and during reperfusion. Group 2 animals had 75% +/- 4% decrease in SMA flow during the tamponade period. During the ischemic period, the MMC cycling time (CT) increased from 67 +/- 10 (mean +/- SEM) to 98 +/- 12 minutes (P < .05) and MMC propagation velocity (PV) decreased to 4.2 +/- 2.2 from a baseline value of 10.5 +/- 1.5 cm/min (P < .05). During reperfusion CT and PV values were not significantly different from baseline. The validity of this model is confirmed by the comparable baseline recordings in groups 1 and 2, and by the return of MMC to baseline values within 4 to 7 hours of reperfusion, as seen in group 2.(ABSTRACT TRUNCATED AT 250 WORDS)

Forty-one patients with high-risk neuroblastoma were treated between September 1977 and December 1987 at the Children's Hospital of Philadelphia with supralethal chemotherapy and total-body irradiation rescued by bone marrow transplantation. Twenty-six patients were treated following relapse and 15 were newly diagnosed. At the time of evaluation, January 1991, 11 of 41 patients (26.8%) remained in complete remission. Actuarial survival rates of patients transplanted following relapse were 0.35 and 0.31 at 2 and 5 years, respectively, and actuarial disease-free survival rates were 0.38 at 12 months and 0.27 at 24 months. The 2- and 5-year actuarial survival values for the patients with newly diagnosed disease were 0.53 and 0.25, respectively, and the 12- and 24-month disease-free survival rates were 0.47 and 0.27, respectively. There was no significant difference in survival between these groups. Twenty-nine of the 41 patients reviewed were available for analysis of the effect of local treatment. Thirteen had a combination of surgery and radiation (RT), 2 had surgery alone, 9 had RT alone, and in 5 patients no local treatment was given. The local relapse rate was 17%; it was 15% following surgery plus RT and 22% following RT alone. The failure rate combining local and distant relapse is 62% for surgery plus RT and 44% for RT alone. Although a local relapse rate of 17% is imperfect, it is a relatively small contribution to the overall relapse of 62%.

Postoperative intussusception in children is a rare but well recognized phenomenon. The diagnosis is often delayed due to the protean manifestations of the disorder (ileus, distention, and nausea and vomiting) which, when encountered shortly after an abdominal operation, usually result in a low index of suspicion because they are common after laparotomy. Experience with two cases of postoperative intussusception within 24 hours heightened our index of suspicion. Review of our records indicated we had diagnosed and treated postoperative intussusception in 14 children during the preceding 4 years. Patient ages ranged from 4 months to 12 years (mean 39 months, median 20 months), and symptoms appeared on postoperative days 3 to 36 (mean 10 days, median 6 days). Initial operations included excision of a retroperitoneal or abdominal tumor (five cases), Nissen fundoplication and gastrostomy (three), ileal resection (two), Ladd procedure (one), Duhamel operation (one), and operative reduction of ileocolic intussusception (the two most recent cases). Eleven patients had appendectomy (five by the inversion technique), and three had placement of a transgastric small bowel feeding tube. Nine children had had either barium enema or upper gastrointestinal studies because of the postoperative suspicion of obstruction; one patient had both. Diagnostic studies were not done in four patients. Operative reduction was successful in all but one child, who required bowel resection.

Since 1987, multiple complications related to maternal cocaine abuse have been reported. Necrotizing enterocolitis-(NEC) of the newborn has been observed with increasing frequency. We report a comparative analysis of infants with NEC born to cocaine abusing mothers (n = 11) to a standard population of newborns with NEC (n = 50) treated in this institution from January 1987 to July 1989. We also evaluated whether prenatal cocaine abuse predisposes infants to NEC by performing a case-control analysis using 51 of 61 infants and controls matched for race, sex, and birthweight +/- 250g. Significant differences were apparent between the cocaine-affected infants (COC) and the noncocaine-affected infants (Non-COC) with regard to surgical intervention (72.7% v 38%, P less than .05), the presence of massive gangrene (54% v 12%, P less than .01), mortality (54.5% v 18%, P less than .01), and maternal age (28.13 +/- 3.82 years v 24.12 +/- 6.21 years P less than .05). No differences between these groups could be demonstrated for other known NEC risk factors such as gestational age, birthweight, feeding patterns, umbilical artery catheters, or asphyxia. In the matched case-control study, infants born to mothers who were cocaine abusers demonstrated a 2.5-fold increased risk of developing NEC (95% Cl = 1.17 to 5.32, P = .02) when compared with the noncocaine-exposed group. Maternal cocaine abuse appears to play a contributory role in the pathogenesis of NEC, its extent, and its outcome.

Parathyroid surgery in children is uncommon. Spontaneously occurring cases of hyperparathyroidism are almost always due to single-gland disease; however, on exploration all four parathyroid glands should be identified. Most of the other instances in which the surgeon needs to perform a parathyroidectomy on an infant or a child will be situations were multiple-gland disease is the rule rather than the exception. Therefore, the surgeon must have in his mind a well developed logical approach to the management of children with parathyroid disorders on the basis of multiple glandular disease. We believe that the technique of parathyroid autotransplantation very satisfactorily addresses the surgical needs of children with familial hyperparathyroid states, including the multiple endocrine neoplasias. We believe that it is mandatory treatment in patients presenting with neonatal primary hyperparathyroidism and is also the procedure of choice in children with secondary and tertiary hyperparathyroidism. The workup and diagnosis of parathyroid disorders should be familiar to the surgeon who undertakes neck exploration on children, and the entity of familial hypocalciuric hypercalcemia should be looked for, as these patients have a strong likelihood of not benefiting from parathyroidectomy.

A 60 percent degloving injury involving the torso and lower extremities of an 8-year-old boy is described. Successful management employed the use of a new trilaminar skin coverage technique. With the avulsed flap still attached to its bed, a 0.14-inch split-thickness graft of epithelium and superficial dermis is raised with a power-driven dermatome. From the same harvest site, one level deeper, a second layer consisting of split-thickness dermis (0.14 inch) is taken. Both the first and second layers are meshed and expanded. The remaining degloved flap is excised and, on a sterile bench, defatted to produce a third layer of deep dermis. In our case, this third layer was ultimately lost, but it functioned well as a temporary biologic dressing. Depending on donor-site morbidity, other potential applications of this method (i.e., major burn injuries) may be feasible.

Resection with primary anastomosis is currently being advocated for treatment of infants with necrotizing enterocolitis. To determine whether our own data would support such an approach, we reviewed retrospectively our experience with this disease since 1974. Since that time, 173 infants have been admitted for treatment of advanced (surgical) disease in its acute phase, of whom 143 underwent resection for cure; the remainder either underwent laparotomy with decompression (3), laparotomy with drainage (3), laparotomy alone (14), died at operation (1), or could not be resuscitated sufficiently to withstand operation (9). Excluded were patients who underwent operative repair of late stricture (6), all of whom survived with no morbidity. Among those resected for cure, 27 infants were carefully selected by the operating surgeon for treament by means of resection with primary anastomosis, based on the limited and apparently discrete nature of their disease; in three the procedure was combined with a decompressing enterostomy. In the majority of cases (14), the disease was found to involve multiple areas of intestine, but was limited to a particular anatomic region, usually distal ileum and/or ascending colon; in the remainder, it was due to discrete ileal or jejunal perforation or ulcer. Overall survival among those resected for cure was 65% (96/143). It was 48% (13/27) among those treated by means of resection with primary anastomosis but 72% (83/116) among those who underwent resection with enterostomy. However, if the early years of the series (1974 to 1976) are excluded, a time when resection with enterostomy had not yet become established as standard therapy, overall survival was 77% (77/100), 64% (9/14) among those anastomosed primarily, and 79% (68/86) among those diverted or exteriorized. Mortality in all cases was due to associated sepsis; however, anastomotic leaks were present in three of the seven nonsurvivors of primary anastomosis who were autopsied. There were no obvious differences between the two groups of patients with regard to details of presentation or management, or age or weight at birth or operation. It appears from these data that while primary anastomosis has been performed far less frequently, and much more selectively, in recent years, patients treated in this manner certainly fare no better than infants treated by other methods. Indeed, it may actually jeopardize the survival of an infant who should, on the basis of the limited and discrete nature of his disease, be expected to live. Thus, our experience does not support resection with primary anastomosis as a superior mode of therapy. It also casts doubt on the reliability of surgical judgment in assessing the true extent of disease at the time of resection.

Neck masses are frequent findings in the pediatric population. Unlike the adult, there are few established guidelines for evaluation of these children. The etiology of cervical masses includes many conditions. Knowledge of these conditions and their clinical presentations is essential. To elucidate the clinical characteristics which may help in establishing a correct diagnosis, the charts of 445 patients with biopsies of neck masses performed at the Children's Hospital of Philadelphia were analyzed. There were 244 (55%) congenital lesions, 118 (27%) inflammatory lesions, 23 (5%) non-inflammatory benign masses, 12 (3%) benign neoplasms, and 48 (11%) malignancies. The preoperative diagnosis was correct in 270 (61%) patients. Guidelines are established for the evaluation of the child presenting with a neck mass.

An experimental technique for transplantation of the parathyroid has been developed recently to counter renal osteodystrophy, primary parathyroid hyperplasia, and other unique problems of parathyroid hyperfunction. Operative procedure, postoperative management, assessment of graft function, and cryopreservation of parathyroid tissue are among the topics covered.

Over a 12-month period (September 1984 to September 1985), 64 children with newly diagnosed brain tumors were admitted to the Neurosurgical Service at The Children's Hospital of Philadelphia. Of these children, 29 had posterior fossa tumors. Of this population of children with posterior fossa tumors, three patients aged 4 months, 22 months, and 4 years old developed massive exsanguinating upper gastrointestinal hemorrhage within seven days of their primary neurosurgical procedure. In each instance, large posterior duodenal ulcers were encountered and were treated with oversewing of the duodenal ulcer and vagotomy-pyloroplasty. Follow-up currently ranges from 18 to 26 months. All three children have survived and none have had any gastrointestinal bleeding since then. Massive exsanguinating hemorrhage was not seen in children with brain tumors in locations other than the posterior fossa. In this population of patients, we advocate the use of prophylactic cimetidine and titration of gastric acidity with antacids.

SummaryThyroid and parathyroid glands from 8-day-old rat pups were incubated both separately and together for 8 hr in a serum-free medium. In addition, thyroparathyroid complexes were incubated in the presence or absence of added bovine PTH or rat or salmon CT. Secretion of PTH and CT was measured using radioimmunoassays that detect rat PTH and rat CT. In all experiments, secretion of CT was 5- to 10-fold greater in 2.5 mM Ca than in 1 mM Ca. Conversely, PTH release was 5- to 10-fold higher in 1 mM Ca than in 2.5 mM Ca. PTH secretion was no different whether the thyroid gland was present with the parathyroids or not, and CT release from thyroids was not affected by the presence or absence of the parathyroid glands. The results support the conclusion that the thyroid gland (CT) exerts no direct influence on the ability of the parathyroids to release PTH and that the parathyroid (PTH) exerts no direct effect on the release of CT from the thyroid gland.

Ischiopagus tripus conjoined twins were recently encountered. All organ systems were thoroughly evaluated using radiographic, angiographic, radionuclide, and CT imaging techniques. None of these studies were capable of accurately assessing the vascular territories of the skin at the level of the pelvis, the most critical area in terms of separation. Qualitative visual assessment of tissue fluorescein delivery under ultraviolet illumination is subject to subjective errors. Perfusion fluorometry is a recently developed technique whereby tissue fluorescence can be quantitated over time in very small amounts, even with repeated injections. In these conjoined twins, using sequential fluorescein injections in each twin, it was possible to accurately determine which portions of the pelvis and the shared leg belonged to which twin and exactly where the skin incisions should be made. Additionally, during operation it was possible to accurately predict the viability of skin flaps used for closure. Both twins survived. This is the first time it has been possible to accurately assess vascular territories of the skin in a complicated form of conjoined twinning. The technique is also useful in the evaluation of flow patterns in various other parts of the body.

During a 20-year period, eight term infants admitted to a large children's hospital for treatment of dehydration associated with diarrhea (6) and vomiting (2), and who ranged in age from 10 to 60 days at onset of symptoms and 18 to 75 days at presentation, developed distal small bowel obstruction following apparently successful rehydration. With the exception of a single infant who was dead on arrival, and another whose obstruction went unrecognized, all infants came to operation shortly after obstruction was confirmed. In each case, a severely inflamed area of distal ileum (7) or proximal colon (1) was found at autopsy (2) or operation (6) to be the cause; perforation was present in four of the cases. Resection of the diseased segments of intestine, and primary anastomosis, were performed in all six operated cases; reoperation was required in four of the six for leaks (3) and adhesions (1). All but two survived. Pathologically, the resected intestinal segments showed a unique pattern of injury: numerous punctate ulcers were apparent, which undermined the muscularis mucosae, without evidence of necrosis. Regenerating epithelium extended through these defects, resulting in the presence of glandular invaginations, which were surrounded by a brisk inflammatory response: hence the term "microdiverticulitis." We believe this lesion represents a beginning or furtive attempt at repair of severely inflamed, but viable intestine, and that it is a rare but true cause of small bowel obstruction in early infancy, separate and distinct from necrotizing enterocolitis.

When an indwelling central venous catheter (CVC) is the source of bacteremia in infants with long-term nutritional problems, CVC removal controls sepsis but a central venous access site is sacrificed. If transcatheter antibiotics could resterilize infected lines, then some of the precious CVCs could be salvaged. We reviewed the management and outcome of CVC line infections at the Children's Hospital of Philadelphia from July 1980 to September 1984. Bacteremia or fungemia occurred 55 times in 47 patients, aged 1 week to 16 years (median 3 mos), an overall rate of 8% for the study period. CVCs were in place 4 to 310 days (median 27 days) at the time of infection. Staphylococcus epidermidis was the most common organism isolated, followed by Candila albicans. CVC removal was the primary mode of controlling bacteremia in 32 patients. Generally, antibiotic coverage was added (21 patients), but removal alone without antibiotics appeared to be adequate therapy in 11 patients, including 5 with Candila fungemia. In 15 patients antibiotics were given, attempting to clear the line of infection. Persistent sepsis or positive repeat cultures led to removal of the CVCs in 9 of the 15 (60%). Line infection cleared on initial antibiotic therapy alone in 6 patients, allowing line salvage. However, bacteremia recurred in 3 and in each an organism different from the one causing the original infection was isolated. Sepsis cleared after the removal in 2, and one died of Candila superinfection during a second attempt at line sterilization. Two patients remained free from sepsis with CVC salvage with antibiotic therapy alone (13%). One patient died of peritonitis not related to catheter infection with the original CVC. Of the 47 patients, 8 received replacement CVCs and 1 received a third CVC. Five (55%) suffered another bout of line sepsis, often with organisms different from the one causing the original infection. CVC removal remains the most reliable means of managing line sepsis. Those receiving another CVC are at risk of infecting their new line, often with an organism different from the original one causing the first septic bout. Although theoretically desirable, “sterilizing” CVCs with transcatheter antibiotics alone has limited success.

The advent of total parenteral nutrition (TPN) has made survival beyond infancy possible for large numbers of patients who have sustained massive small intestinal loss due to a variety of intraabdominal catastrophes. However, the quantity and quality of life have been limited by the development of late sequalae due both to the protracted use of TPN and the long-term complications of foreshortening of the gut. To determine to what extent the morbidity and mortality of short-bowel syndrome (SBS) may have improved over the last 10 years, we reviewed our experience since 1973 with patients losing more than 50% of total small intestinal mass in infancy. The etiologies of SBS in the 16 study patients were necrotizing enterocolitis (6), midgut volvulus (5), multiple atresias (3), gastroschisis (1), and congenital SBS (1). Overall survival was 81%; total small intestinal length (SIL) at the time of diagnosis was 44.2±7.9 cm in survivors and 30.3±7.8 cm in nonsurvivors, probability values not significant. Although no patient survived without an ileocecal valve whose total SIL was greater than 20 cm, the three deaths in this series were not related directly to the SIL, but to end-stage liver disease resulting from TPN-associated cholestasis. Among the survivors, adaptation to enteral feedings required 13.8±2.5 mo, during which time weaning from TPN occurred; weight at adaptation was 6.87±1.32 kg. A mean follow up of 3.3±0.8 years permitted assessment of quality of life; the mean score was 3.0±0.3 on an arbitrary scale (excellent=4, good=3, fair=2, poor=1), while the mean number of stools per diem was 3.1±0.7. Finally, near-normal somatic growth was achieved in survivors weaned from TPN in that mean weight (W), height (H), and weight-for-height (W/H) all approached or exceeded the 25th percentile (W=26th percentile, H=24th percentile, W/H=41st percentile). We conclude that the long-term outlook for most patients with SBS resulting from massive small intestinal loss in infancy is good, although TPN-associated cholestasis may lead to end stage liver disease and death in a minority of patients.

We report six pediatric patients who had a partial splenectomy for splenic cysts and a seventh patient who had a total splenectomy. There were six epidermoid cysts and one traumatic cyst. All patients had benign postoperative courses. All patients who underwent partial splenectomy had a significant postoperative rise in platelet count that subsequently returned toward baseline. This rise implies a loss of splenic function, although the exact meaning of this is unclear. All patients had late postoperative liver-spleen scans that showed mean spleen size and function to be normal. We have described several methods of partial splenectomy used at Children's Hospital of Philadelphia. Recommendations for patients undergoing partial splenectomy include preoperative pneumococcus and Hemophilus influenzae vaccinations and prophylactic antibiotics postoperatively. The antibiotics are terminated if a liver-spleen scan and platelet count at the end of 3 months' time are normal.

Although the protracted use of total parenteral nutrition (TPN) in infants is known to predispose to cholestasis, which in certain instances may not be reversible, failure to understand the pathogenesis of this condition has hindered the development of an effective medical treatment. That surgical treatment may reverse this process in selected patients is the subject of the present report. Two premature infants and one term infant, with short bowel syndrome acquired in infancy, developed conjugated hyperbilirubinemia following institution of balanced TPN. Infectious and metabolic etiologies for the jaundice were ruled out. No excretion of 99mTc-labeled DISIDA from the biliary tree was demonstrable 24 hours following injection by nuclear scintigraphy, despite the absence of extrahepatic biliary obstruction by abdominal sonography. When all conventional medical methods of managing the problem failed, exploration was performed. Intraoperative cholangiography showed normal intrahepatic and extrahepatic biliary ducts in each instance. Irrigation of the biliary tree with normal saline solution was then performed. In all cases, liver biopsies were obtained that were consistent with TPN-associated cholestasis and in all cases, jaundice resolved promptly following operation. We conclude that intractable cholestasis associated with TPN, regardless of its etiology, is a functional abnormality of biliary excretion that may respond in selected patients to irrigation of the biliary tree with radiographic dye and/or normal saline solution.

From October 1976 to October 1986, 126 children had bone marrow transplants at the Children's Hospital of Philadelphia. The indications were acute lymphocytic leukemia (ALL) (30), nonlymphocytic leukemia (24), aplastic anemia (15), solid tumors (47), and miscellaneous conditions (10). Of these, 21 (17%) underwent 22 open-lung biopsies. Fourteen of these patients showed no causative microorganism. When a cause was found it was viral (usually cytomegalovirus [CMV]) in three, fungal in one, Pneumocystis carinii alone in two, both viral and pneumocystis in one, and a combination of viral, bacterial, and pneumocystis in one. Thirteen patients died due to continued deterioration after the biopsy. In only two patients was there a significant change in antimicrobial therapy as a results of the biopsy. Both had Pneumocystis (one in combination with virus and bacteria). One patient with chronic infiltrates showed a lymphocytic interstitial pneumonia, which responded well to steroids. Open-lung biopsy is currently of limited value in this patient population. Survival is dismal unless the patient has Pneumocystis . We believe that prospective studies should be set up to compare open-lung biopsy with empiric antimicrobial therapy. A major emphasis must be on prevention.

We reviewed 15 years' experience with childhood trauma at two hospitals in different cities, one a city hospital, the other a children's hospital, to learn the extent, circumstances, presentations, and consequences of major blunt abdominal trauma due to child abuse. Some 10,000 children admitted to these hospitals for treatment of injuries from 1972 through 1986 provided the basis for the study; the incidence and severity of pediatric trauma at the two hospitals was similar, in that 13% of the visits to both hospitals' emergency rooms were for trauma, of which 5% resulted in admission. Major blunt abdominal trauma due to child abuse accounted for 22 of these cases, six at the former, 16 at the latter, and represented less than 0.50% of all abused children seen at both institutions. The average age was 24 mo; 14 were boys and eight were girls. In only two instances was the family unit intact; in both, the child was abused by the babysitter. Otherwise, the father, or the mother's "boyfriend," was responsible. Overall mortality was 45%, and was related both to type of injury and presenting signs. Of one who presented with an epigastric mass due to a pancreatic hematoma, none died; the pseudocyst which subsequently developed resolved on bowel rest and TPN. Of three who presented with bilious vomiting due to duodenal hematoma, none died; one required operative evacuation. Of five who presented with peritonitis due to duodenojejunal rupture, one died; this child presented greater than 24 hr following injury in profound septic shock. Of three who presented with hypovolemia due to moderate hemorrhage, none died; the former two were managed conservatively.(ABSTRACT TRUNCATED AT 250 WORDS)

Conjoined twins occur in approximately one in 50,000 or so births, and most do not survive. The authors report herein their experience with 13 conjoined twins over the last 30 years, involving those of the following forms: thoracopagus (4 cases), omphalopagus (1 case), ischiopagus (4 cases), pygopagus (1 case), craniopagus (1 case), and incomplete or parasitic varieties (2 cases). The various diagnostic and imaging studies used are described in detail for each form of twinning. Separation is best delayed until such infants are relatively mature (i.e., 6-12 months of age). Operative survival was 50% in those operated on in the neonatal period, but 90% in those over 4 months of age. Ten separations were attempted in 13 sets of twins, with 16 operative survivors. Significantly, up to 10 years after surgery, there were six late deaths due to serious associated congenital anomalies, predominantly cardiac. Improved recent survival is probably the result of the availability of more accurate imaging studies and better anesthetic and operative techniques, with great emphasis on performing immediate reconstruction whenever possible. Use of skin expanders and prosthetic mesh has facilitated wound closure. In the future, ex vivo cardiac reconstruction and autotransplantation may permit separation of twins with complicated conjoined hearts.

Percutaneous endoscopic gastrostomy (PEG) is frequently used for long-term enteral nutrition or gastrointestinal decompression in both adults and children. The rare complication of a cologastric fistula following PEG has been seen recently in two pediatric patients. One fistula did not close after removal of the gastrostomy tube. A mechanism for the occurrence of this complication in these two children is proposed and technical points are emphasized to prevent this complication.

This is a report of a case where an atypical mycobacterial process presented as a large abdominal mass.

This report details an 11-year experience with 17 patients ranging from newborn to 17 years with choledochal cyst. Two distinct groups were noted: an infantile group (mean age: 3 months) with obstructive jaundice identical to biliary atresia and a late onset group (mean age: 9 years) with various combinations of pain, mass, and jaundice. Two patients had cystoduodenostomy performed and both required revision. One of six patients who had Roux-Y cystojejunostomy required revision. All seven patients who had primary cyst excision and two patients who had secondary cyst excision with Roux-Y hepaticojejunostomy have been followed prospectively and have done well. The follow-up period ranges from 1-11 years with an average of 5.8 years. Cyst excision should be performed as a primary or secondary procedure whenever feasible. The rare patients with intrahepatic ductal dilatation (Caroli's disease) are best approached by hepatic lobectomy when possible, and those with choledochocele should be treated by unroofing the cyst as indicated by the anatomy encountered.

A high-performance liquid chromatographic method for the analysis of carprofen in solid dosage forms and as the bulk drug substance was developed. The simple, accurate, reproducible, and stability-indicating method was shown to be applicable to drug substance and dosage form stability studies, as well as the quality control of carprofen dosage forms.

Primary hyperparathyroidism in the neonate is a rare and often fatal disorder. These infants typically display severe hypercalcemia, respiratory distress, muscular hyptonia, and skeletal demineralization. They are usually diagnosed within the first three months of life and have hyperplasia of the four parathyroid glands. Twenty-nine infants with primary hyperparathyroidism are reported in the literature. Mortality is 87.5% in medically managed patients and 24% in surgically managed patients. Surgical management has not been satisfactory, in that recurrent hypercalcemia has been encountered in most patients undergoing subtotal parathyroidectomy, and total parathy-roidectomy has resulted in the need for lifelong calcium and vitamin D supplementation. We have recently cared for a term newborn female in whom the diagnosis of primary hyperparathyroidism was made clinically on the second day of life, and later was confirmed biochemically. The baby underwent neck exploration on the 11th day of life and was successfully treated with total parathyroidectomy and parathyroid autotransplantation. Although initially rendered eucalcemic, the infant subsequently developed recurrent hypercalcemia requiring the removal of some of the autograft. Currently, the child is more than 2 years following surgery, growing well, and off all medication. The world literature is reviewed in this report of one of the first and the youngest infants, to our knowledge, to undergo parathyroid autotransplantation. In view of its success in avoiding the complication of repeated neck exploration for recurrent hyperparathyroidism or the creation of permanent hypoparathyroidism, we recommend this surgical approach for the rare neonate with primary hyperparathyroidism.

The results of a kinetic and mechanistic study of the hydrolysis of a new antiarrhythmic agent, cibenzoline, are reported. The reaction is subject to specific base catalysis which proceeds via the protonated cibenzolinium ion. No evidence for the existence of a “pseudobase”-type intermediate could be found. The results support only one of two different mechanisms which have been proposed previously for the hydrolysis of this class of compounds.

SummaryThyroparathyroid glands were removed from 8-day-old baby rats and incubated in vitro for up to 8 hr. Release of both PTH and CT into medium were measured using immunoassays that measure rat CT and rat PTH. Results showed that release of CT and PTH in vitro were inversely related. At 1 mM Ca, PTH in medium increased progressively up to ∼5 ng/ml while CT was only 1-2 ng/ml. At 2.5 mM Ca, PTH was suppressed to low levels ( 90%. PTH and CT release were not altered by several other agents tested, including secretin, pentagastrin, 1,25-(OH)2D3 and 24,25-(OH)2D3.