Liver and circulating NK1.1(+)CD3(-) cells are increased in infection with attenuated Salmonella typhimurium and are associated with reduced tumor in murine liver cancer.

An attenuated (DeltacyA, Deltacrp) strain of Salmonella typhimurium (chi4550) containing a gene for human IL-2 (chi4550pIL2) reduces hepatic tumor burden when orally inoculated into mice with liver cancer; however, wild-type S. typhimurium is also associated with cancer regression. Therefore, experiments were designed to clarify the invasiveness and the anti-tumor properties of three strains of S. typhimurium. S. typhimurium chi4550pIL2, chi4550, or wild type (WT) was incubated with mature Caco-2 and HT-29 enterocytes, and S. typhimurium internalization was assessed. For infectivity experiments, mice were orally inoculated with saline or 10(9)S. typhimurium chi4550pIL2, chi4550, or WT; 48 h later mice were sacrificed for analysis of cecal bacteria and S. typhimurium translocation to mesenteric lymph nodes. For experiments involving tumor implantation, four groups were studied: saline control, tumor alone, chi4550pIL2+tumor, and chi4550+tumor. Mice were orally inoculated with saline or S. typhimurium and underwent laparotomy 24 h later with 5 x 10(4) MCA38 murine adenocarcinoma cells injected into the spleen. On day 14, liver tumors were counted and peripheral blood and hepatic lymphocyte populations were analyzed by FACScan. Attenuated S. typhimurium exhibited decreased internalization by cultured enterocytes and decreased infectivity after oral inoculation. Mice treated with chi4550pIL2 or chi4550 had fewer liver tumors and increased populations of hepatic and circulating NK1.1(+)CD3(-) lymphocytes compared to mice treated with saline (P < 0.01). These data suggest that attenuated S. typhimurium may have an application as an anti-tumor agent.

Recurrent congenital diaphragmatic hernia: A novel repair.

BACKGROUND/PURPOSE: Repair of recurrent diaphragmatic hernia continues to be a difficult problem. An innovative method using a nonabsorbable polypropylene prosthetic mesh plug placed via the thoracic approach using minimal dissection is presented. METHODS: A retrospective analysis showed 39 children with congenital diaphragmatic hernia (CDH) who underwent repair between January 1997 and March 2000. Five children suffered a recurrence and underwent repair via the thoracic approach using the Bard Marlex Mesh Perfix Plug (C.R. Bard Inc, Billerica, MA). Follow-up was available in all children and ranged from 1 to 33 months (average, 13.8 months). RESULTS: Age at recurrence ranged from 2 to 48 months (average, 14.8 months), and the average time between initial repair and recurrence was 8.2 months (range, 2 to 16 months). There were no recurrences after the transthoracic mesh plug diaphragmatic hernioplasty. One child died of multiple congenital anomalies 6 months after repair. CONCLUSION: The transthoracic repair of recurrent diaphragmatic hernias using a nonabsorbable polypropylene prosthetic mesh plug represents an innovative approach to a difficult problem in which 5 repairs have been accomplished without recurrence in nearly 14 months of follow-up. J Pediatr Surg 36:1768-1769.

The slipping rib syndrome in children.

The slipping rib syndrome is an infrequent cause of thoracic and upper abdominal pain and is thought to arise from the inadequacy or rupture of the interchondral fibrous attachments of the anterior ribs. This disruption allows the costal cartilage tips to sublux, impinging on the intercostal nerves. Children with this entity are seldom described in the literature. We present a retrospective review of 12 children and young adults with slipping rib syndrome and a systematic approach for evaluation and treatment.

Prospective results of a standardized algorithm based on hemodynamic status for managing pediatric solid organ injury.

BACKGROUND: Controversy surrounds the need for ICU admission, prolonged bed rest, and the duration of activity restrictions for children sustaining blunt trauma. Adult literature supports management based on hemodynamic status, not CT grade. STUDY DESIGN: A 3-year prospective study of a standardized management algorithm for hemodynamically normal pediatric patients with blunt liver or spleen injury was performed. Patient selection was based on vital signs, irrespective of injury grade on CT. Patients requiring ICU admission for nonliver or nonspleen injury were excluded. Patients were admitted to a surgical ward with serial hematocrit levels. Discharge occurred 48 hours postinjury if patients had no abdominal tenderness, tolerated a regular diet, and had a stable hematocrit. Patients were allowed noncontact activity, including school, after discharge. Patients were followed up at 1 month with ultrasonographic imaging. RESULTS: Eighty-nine patients sustained blunt liver or spleen injury. Forty-five patients were excluded for other injuries (Glasgow Coma Scale < 13, 32 of 45); the remaining 44 patients had a mean age of 8.9 years (range 2 to 17 years), Injury Severity Score 10.6 (range 4 to 33), liver grade 2.1, and splenic injury grade 2.3. Mechanisms of injury were predominately motor vehicle collisions (59%). All patients were managed nonoperatively without transfusion; 43 of 44 patients completed the algorithm. Mean observation was 55.2 +/- 12.3 hours. One-month followup occurred in 33 of 44 patients, with one complication detected and no delayed bleeding. CONCLUSION: Management of pediatric solid organ injury should be guided by hemodynamic status and not injury grade on CT. Hemodynamically normal children can be safely managed without intensive care monitoring, do not need prolonged hospitalization, and can resume school on discharge.

Congenital abdominal aortic aneurysm in the infant: case report and review of the literature.

Congenital abdominal aortic aneurysms are a distinct entity from acquired aortic aneurysms. The authors present the case of a 6-week-old boy with a 6-cm aneurysm involving the abdominal aorta and common iliac arteries. Three other cases of congenital aortic aneurysms are reviewed, and an approach to these rare patients is discussed. J Pediatr Surg 36:657-658.

Acidification of formula reduces bacterial translocation and gut colonization in a neonatal rabbit model.

BACKGROUND/PURPOSE: The authors hypothesized that gastric acidity is protective because it is bactericidal. They tested acidified formula for protection against gut colonization and bacterial translocation. METHODS: In vitro: Formula was acidified to pH of 2, 3, 4, 5 and innoculated with Enterobacter. Growth over time was quantitatively assessed. In vivo: 442 premature rabbit pups were sorted randomly and fed formula of pH 2, 3, 4, or 7, with ranitidine. Two models were utilized: (1) with bacterial challenge using a known acid sensitive organism, (2) without bacterial challenge to simulate natural gut colonization and to test against organisms of unknown acid sensitivity. Normal acid animals received pH 7 formula, no ranitidine. On day 3, the mesenteric lymph nodes (MLN), spleen, liver, and cecum were harvested and cultured. RESULTS: Bacterial growth was inhibited at pH 2 and 3, growth was logarithmic above pH 4 (P<.001). Total and organ-specific translocation was reduced at pH 3 and below in both models (P<.05). Translocation with formula pH 3 equaled normal acid animals. Quantitative cecal colonization was reduced in pups receiving pH 3 and below in both models (P<.05). CONCLUSION: Acidification of formula below pH 4 is bactericidal to enteric organisms. Acidified formula decreases bacterial translocation and gut colonization.

Catheter materials affect the incidence of late blood-borne catheter infection.

BACKGROUND: Adherence of bacteria and subsequent catheter-related infections (CRI) are greatly enhanced by the fibrin sheath that develops on indwelling catheters. Since the infection rate of catheters without fibrin sheaths is low and the fibrin sheath mediates bacterial adherence, catheter material is not thought to affect the incidence of late catheter-related infection. METHODS: A total of 276 rats had catheters placed in the right jugular vein with the proximal end buried subcutaneously to eliminate exit site infection. Rats were divided into two groups: silastic catheters (SC; n = 133) and polyurethane catheters (PC; n = 143). Injections of 1 x 10(8) CFU/mL of Staphylococcus epidermidis were given via the tail vein on either the day of surgery, day 0 (n = 53 SC, n = 51 PC), or on postoperative day 10 (n = 50 SC, n = 62 PC). Thirty animals from each group (SC, PC) received sterile saline injections on day 10 and served as controls. Animals were sacrificed on postinjection day 3. Catheters were removed via the chest and placed into trypticase soy broth. Broth was incubated at 37 degrees C for 48 h. Microscopy for the fibrin sheath was done on 20 randomly selected catheters (10/group). Data were compared using Fisher's exact test, with p < 0.05 considered significant. RESULTS: Incidence of CRI was equal prior to the formation of the fibrin sheath, while CRI was significantly higher in silastic catheters in the presence of a fibrin sheath. Without a fibrin sheath (day 0), 8/53 silastic catheters and 3/51 polyurethane catheters were infected (p = NS). With a fibrin sheath (day 10), 31/50 silastic catheters were infected versus 20/62 polyurethane catheters (p < 0.05). Control catheters were all culture negative (30/group). With light microscopy, 20/20 catheters had fibrin sheaths at day 10 with no visible difference between silastic and polyurethane catheters. CONCLUSION: Catheter material does affect the incidence of catheter-related infection even when catheters are coated with a fibrin sheath. This difference may relate to a difference in the fibrin sheath itself as it forms on different catheter materials.

Complications and risks of central venous catheter placement in children.

BACKGROUND: Since Aubaniac's first description in 1952, the use of central venous catheters has increased dramatically; they are now considered commonplace. Placement of these catheters, however, has an associated risk of morbidity and mortality. In most cases, this risk is outweighted by the benefit gained, especially when long-term access to the central venous system is needed for multiple transfusions, chemotherapy, antibiotics, or parenteral nutrition. A large number of central venous catheters are placed in children at our institution, usually by interns and residents. METHODS: To identify associated risks and complications, we reviewed the records of 1435 consecutive catheterizations in children over a 10-year period. Data collected included age, sex, site of catheterization, type of catheter, primary disease, prior catheterizations, indication for placement, failed attempts, number of attempts, catheter misplacement, level of physician training, new needle punctures, and complications. We then used logistic regression analysis to identify independent risk factors for complications. RESULTS: We noted 39 (3.1%) perioperative complications, including 19 (1.5%) arterial punctures, 10 (0.8%) pneumothoraces, 6 (0.5%) hemothoraces, 2 (0.2%) cases of superior vena cava syndrome, 1 (0.1%) episode of ventricular fibrillation that required cardioversion, and 1 episode of bleeding that required a cutaneous suture. Univariate analysis revealed that catheters placed in a subclavian vein (vs all other sites combined, P < .01) were less likely to have an associated complication. In addition, multiple attempts (vs success on first attempt, P < .0001), failed attempt (vs success at initial site, P < .0001), catheter misplacement (vs proper initial position, P < .01), and prior catheterizations (vs no prior catheterization, P < .0005) were associated with complications. Logistic regression revealed multiple attempts (vs success on first attempt, odds ratio (OR) = 5.4), failed attempt (vs success at initial site, OR = 5.2), and catheter misplacement (vs proper initial position, OR = 6.9) to be independent risk factors for complications. Age, sex, type of catheter, primary disease, indication for placement, and level of physician training (intern or resident vs staff) were not associated with complications. CONCLUSIONS: Central venous catheterization in children is relatively safe, with only a 3.2% complication rate and no mortality in our series.

Timing of colonic necrosis in hemolytic uremic syndrome.

Hemolytic uremic syndrome (HUS) consists of an acute onset of microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction. HUS-associated colitis can be seen in up to 100% of patients and is usually associated with severe abdominal pain and distention. Colonic perforation is a complication of HUS that has a reported incidence of 1%-2%, and although there are several case reports in the literature describing perforation of the colon, it is still very difficult to discern the abdominal symptoms associated with HUS colitis from perforation. Four cases of colonic perforation are reported here from a consecutive series of 57 patients, in which a trend in the length of time from the onset of symptoms of HUS to colonic perforation was determined. A review of the literature for cases of HUS-associated colonic perforation was also performed. The time from the onset of HUS symptoms to colonic perforation in our series was similar to that found in the literature review (11 +/- 5 vs 14 +/- 8 days). Awareness that this complication has a tendency to occur towards the end of the 2nd week during the course of HUS is essential to avoid an unnecessary and untimely surgical intervention.

Cystic renal lymphangiectasia: a distinctive clinicopathologic entity in the pediatric age group.

This report describes the features of unilateral cystic renal lymphangiectasia in a 2-year-old child who presented with hypertension, massive ascites, a left flank mass, and no evidence of familial renal cystic disease. The child became normotensive and is now asymptomatic more than 3 years after surgery. The clinical presentation and diffuse pathologic involvement are similar to findings for the few pediatric patients with cystic lymphangiectasia described in the literature and appear distinct from the more localized form of the disease seen in adults.

Antitumor mechanisms of attenuated Salmonella typhimurium containing the gene for human interleukin-2: a novel antitumor agent?

Currently, there is no long-term effective treatment for unresectable hepatic malignancies. Salmonella species are known to naturally track to the liver during active infection. To develop a biological vector for delivery of interleukin-2 (IL-2) to the liver for antitumor purposes, the thi 4550 attenuated strain of Salmonella typhimurium was used as a vector for IL-2. The gene for human IL-2 was cloned into plasmid pYA292 and inserted into the attenuated S typhimurium and renamed (thi 4550(pIL-2)]. MCA-38 murine adenocarcinoma cells were injected intrasplenically into C57BL/6 mice to produce hepatic metastases that were subsequently enumerated after 12 days. We previously have demonstrated that the thi 4550(pIL-2) produces biologically active IL-2 and that a single gavage feeding of 10(7) thi 4550(pIL-2) significantly reduced the number of hepatic metastases when compared with animals fed salmonella lacking the IL-2 gene or nontreated controls. The aims of the current studies were to determine which host effector cell populations were responsible for the antitumor effect seen with thi 4550(pIL-2) by depletion of natural killer (NK), cytotoxic T lymphocytes (CD8+), T helper (CD4+) cells, and Kupffer cells. Multiple experiments were conducted for each host effector cell population depleted. We found a consistent reduction in the mean number of hepatic metastases in animals fed thi 4550(pIL-2) (55.6 metastases; n = 54) when compared with controls (162.3 metastases; n = 53) (P < .0001). Depletion of NK cells and CD8+ T cells significantly inhibited the antitumor effect of thi 4550(pIL-2) (analysis of variance [ANOVA], P < .01). Elimination of CD4+ T cells and Kupffer cells had no significant impact on the antitumor effect of thi 4550(pIL-2) (ANOVA, P value was not significant). Salmonella IL-2 may represent a novel form of in vivo biotherapy for unresectable hepatic malignancies that employs the oral route of administration. Furthermore, both NK cells or CD8+ cells are required for the antitumor effect seen while CD4+ T cells and Kupffer cells do not appear to be as essential.

Patterns of hepatic and splenic colonization by an attenuated strain of Salmonella typhimurium containing the gene for human interleukin-2: a novel anti-tumor agent.

Currently, there is no effective treatment for unresectable hepatic malignancies. Salmonella sp. are known to naturally track to the liver during active infection. A live biological vector was developed for delivery of Interleukin-2 (IL-2) to the liver for anti-tumor purposes. The avirulent and highly immunogenic c4550 strain of Salmonella typhimurium was used to express the IL-2 protein [renamed c4550(pIL-2)]. We have previously demonstrated that the c4550(pIL-2) produces biologically active IL-2 (up to 46.2 IU/ml) and that a single gavage feeding of 10(7) colony forming units (cfu) of c4550(pIL-2) significantly reduced the number of hepatic metastases when compared to animals fed salmonella lacking the IL-2 gene or non-treated controls. The goal of the current studies was to determine the pattern of splenic and hepatic colonization of Salmonella-IL2. Hepatic and splenic colonization was determined following administration of 10(7) cfu of c4550(pIL-2) and c4550(pYA292) via a single gavage feeding to C57BL/6 mice. Five experiments of antibiotic regimen administration were conducted where splenic and hepatic homogenates were cultured after 14 days of parenteral and/or oral antibiotics. The natural history of hepatic and splenic colonization was also determined for animals without antibiotic treatment. Despite administration of various antibiotic regimens using different routes, eradication of salmonella with and without IL-2 was not achieved. Salmonella, however, was not cultured from hepatic and splenic tissue at 4 months after a single gavage feeding of salmonella with no specific treatment. In conclusion, oral administration of c4550(pIL-2) may represent a novel form of in vivo biotherapy for unresectable hepatic malignancies. Antibiotics do not accelerate eradication of this bacteria and it appears that c4550(pIL-2) follows the natural pathophysiological of salmonella infection in which eradication from the splenic and hepatic tissue occurs over a period of 2-4 months.

Transanal mucosectomy: a modification of the Soave procedure for Hirschsprung's disease.

In the Soave procedure for Hirschsprung's disease, the mucosectomy of the aganglionic segment of colon is performed transabdominally. The authors describe an innovative modification of the standard Soave procedure, in which a transanal mucosectomy is performed as the abdominal incision is made. The transanal mucosectomy technique was compared with the conventional approach. From 1974 to 1993, 51 patients underwent a Soave procedure for Hirschsprung's disease. Twenty-six (51%) had the standard Soave procedure (STD) (1974-1984), and 25 (49%) had the modified procedure (MOD) (1984-1994). In the latter, the entire mucosal dissection was performed through a transanal approach. Surgical data were accumulated by database analysis. Each case was entered into a database at the time of operation, and follow-up data were obtained through mail-in questionnaires or personal communication. The two groups were comparable with respect to number, gender distribution, and average age at the time of operation. There were two superficial wound infections in the STD group and two superficial wound infections and one death (secondary to cardiac arrest in a patient with trisomy 21 and a severe congenital cardiac anomaly) in the MOD group. The average postoperative stay for the STD group was 10.8 days; that for the MOD group was 6.8 days (t test: P < .0001). The operating time was shorter for the MOD procedure. Follow-up data were available for 25 (96.2%) patients in the STD group and 25 (100%) in the MOD group. The two groups were comparable in all respects except for the average length of follow-up, which was longer for the STD group. No patients had, urinary incontinence or sexual dysfunction (when applicable). Overall, patients/parents in both groups rated the outcome as "good." This modification of the Soave technique for Hirschsprung's disease is both safe and effective. Compared with the standard procedure, it is associated with shorter operating time (because the transanal mucosectomy is performed as the abdomen is opened) and a shorter stay postoperatively. The results have been excellent.

Attenuated Salmonella typhimurium containing interleukin-2 decreases MC-38 hepatic metastases: a novel anti-tumor agent.

Currently, there is no long-term effective treatment for unresectable hepatic malignancies. Salmonella sp. are known to naturally track to the liver during active infection. To develop a biological vector for delivery of Interleukin-2 (IL-2) to the liver for anti-tumor purposes, the avirulent and highly immunogenic chi 4550 strain of Salmonella typhimurium was used as a vector for IL-2. The gene for human IL-2 was cloned into plasmid pYA292 (renamed pIL-2) and inserted into the attenuated Salmonella typhimurium and renamed [chi 4550 (pIL-2)]. This transformant was found to produced biologically active IL-2 demonstrated by NK cell activation in a 4 hour chromium release cytotoxicity assay. To determine anti-tumor potential, MCA-38 murine adenocarcinoma cells were injected intrasplenically into C57BL/6 mice to produce hepatic metastases and metastases were subsequently enumerated after 12 days. Statistical significance was determined by ANOVA with Fisher's test for significance. Hepatic metastases enumerated by blinded observers revealed that the mean number of metastases was 106.4 in control mice, 103.7 in mice gavage fed attenuated salmonella without IL-2 [chi 4550(pYA292)], and 44.3 in mice fed the chi 4550(pIL2); (ANOVA: p < 0.01). Culture of livers and spleens in mice administered a single gavage dose of salmonella demonstrated persistent colonization for up to 4 weeks. No observable toxicity was seen to either IL-2 or salmonella. These studies demonstrate that the chi 4550(pIL2) is a novel form of in vivo biotherapy which produces biologically active IL-2 and employs the oral route of administration to stimulate an immune response against malignancy in the liver.

Aggressive metastasectomy for pulmonic sarcomatous metastases: a follow-up study.

Pulmonary metastases are the primary cause of death due to bone and soft tissue sarcomas. We have previously shown that an aggressive approach and a new technique of multiple pulmonary metastasectomies have resulted in improved survival for patients with pulmonary metastases. In this follow-up study, an expanded database of patients was retrospectively analyzed to determine survivability as well as to evaluate potential prognostic indicators. Forty-nine patients, 26 of whom had osteogenic sarcoma (OGS), were evaluated. A number of patients had been referred from other institutions where their disease had been considered inoperable because it was extensive or recurrent. Using lateral thoracotomies exclusively, employment of a laser technique, and excision of minimal pulmonary parenchymal tissue, we performed aggressive metastasectomy. A mean of 3.0 thoracotomies was performed, in which an average of 10.2 nodules per thoracotomy were excised. Operative morbidity and mortality were minimal. The disease-free interval, the number of nodules resected, the number of thoracotomies performed, and the size of the nodules were evaluated as potential prognostic indicators. Statistically significant correlation could be established only for the size of the nodules resected. The 5-year survival rate for all patients was 39%; it was 24% for patients with OGS and 71% for those without OGS. Aggressive surgical resection of pulmonary metastases from bone and soft tissue sarcoma should be considered when there is control of local disease, no evidence of extrapulmonary metastasis, and adequate post-resection pulmonary reserve. The presence of bilateral, extensive, or recurrent disease is not a contraindication to thoracotomy. Aggressive resection of multiple nodules and improved chemotherapy appear to prolong survival of these patients when compared with survival rates of historical control subjects.

A new approach to the resection of pulmonary osteosarcoma metastases. Results of aggressive metastasectomy.

Pulmonary metastases are the primary cause of death from bone and soft-tissue sarcoma. Recognition that even multiple resections of metastases can improve survival has led to a more aggressive surgical approach to these patients. The authors instituted an aggressive approach and a new technique and retrospectively analyzed the results of multiple, pulmonary metastasectomies for pulmonary metastases in 34 patients, 21 of whom had osteogenic sarcoma (OGS). A number of cases were referred from other institutions, where they had been considered inoperable because of extensive or recurrent disease. Using lateral thoracotomies, laser technique with minimal parenchymal excision, and thin gloves for palpation, aggressive metastectomy was carried out. A mean of 3.1 thoracotomies were performed, with an average of 10.6 nodules resected per thoracotomy. Operative morbidity and mortality were minimal. Evaluation of potential prognostic factors revealed no statistically significant survival difference on the basis of disease-free interval (DFI), number of nodules resected, number of thoracotomies, or size of largest nodule resected. There was a clear trend toward decreased survival of patients with larger nodules (greater than 2 cm), but because of the small number of patients in this group, no firm conclusions can be drawn. Five-year survival was 49% for the study group as a whole, and 39% for the OGS patients. Aggressive surgical resection of pulmonary metastases from bone and soft-tissue sarcoma should be considered when there is control of local disease, no evidence of extrapulmonary metastasis, and adequate postresection pulmonary reserve. The presence of bilateral, extensive, or recurrent disease is not a contraindication to thoracotomy.(ABSTRACT TRUNCATED AT 250 WORDS)

Blunt trauma in adults and children: a comparative analysis.

Trauma remains the major cause of death in children and young adults. Adult and pediatric patients differ significantly in both mechanism of and physiologic response to injury. We reviewed the records of all consecutive adult and pediatric blunt trauma patients admitted to a major metropolitan trauma center for a 10-year period. An extensive computerized database has been maintained for all patients since 1977. A comparative statistical analysis of mechanism of injury, specific organ injury, and clinical outcome was performed. Altogether, 1,722 adults and 289 children were treated during the study period. Blunt trauma accounted for 82.8% of adult and 94.3% of pediatric injury (p = 0.00005), and only these patients were considered for analysis. Diagnostic peritoneal lavage was performed in 249 children and 1,464 adults, with a respective accuracy of 99.6% and 97.2%. Mechanism of injury was comparable for both groups, although children were far more likely to be injured by falls, bicycle accidents, or struck by an automobile. Comparative analysis of specific injuries demonstrated significantly fewer pediatric chest (p = 0.001), spine (p = 0.03), and pelvic (p = 0.003) injuries. Central nervous system (CNS) injury in children was a strong determinant of outcome: serious pediatric CNS trauma was associated with a tenfold increase in mortality. Mortality for children in the absence of CNS injury was less than 3%. Spinal injury also appeared to be a predictor of poor outcome in the pediatric population, with an associated mortality of greater than 50%. Overall, survival was age independent (82.5% of adults and 85.8% of children were survivors.(ABSTRACT TRUNCATED AT 250 WORDS)

Spontaneous perforation of the extrahepatic biliary tree in infancy. A case report.

Spontaneous perforation of the bile duct in infancy is rare, with less than 55 cases described in the literature to date. The authors report the case of a 30-day-old neonate who presented with a 2-week history of progressive abdominal distension and intermittent jaundice. Disofenin technicium 99m sequential scintiscanning provided a preoperative noninvasive confirmation of the diagnosis of biliary ascites secondary to spontaneous perforation of the extrahepatic biliary tract. Distal common bile duct atresia was identified intraoperatively, and end-to-side Roux-en-Y hepaticojejunostomy was performed, with an uneventful postoperative recovery. The etiology, diagnosis, and treatment of spontaneous neonatal biliary perforation is discussed.