Selective ligation of portal vein and hepatic artery for ruptured hepatocellular carcinoma in a 13-year-old boy.

Hepatocellular carcinoma (HCC) in children is rare, and the prognosis has been poor because of its advanced stage at diagnosis and unresponsiveness to chemotherapy. We report a 13-year-old boy with ruptured HCC in the left trisegment. When hemostasis of the ruptured surface was achieved in the emergency operation, the left branch of the portal vein and the left hepatic artery were ligated at the same time. The volume of the future liver remnant (FLR), that is, his right posterior sector, increased from 56% on admission to 70% of his standard liver volume on day 2. Blood level of serum protein induced by vitamin K absence or antagonist ІІ started to decrease immediately. Left trisegmentectomy was successfully performed 10 days later, followed by chemotherapy. He has been well with a 2-year survival without recurrence. When the FLR is considered relatively small for a major hepatic resection, the selective ligation of the portal vein and the hepatic artery, which feed HCC, seems to be beneficial. This is because it may induce enlargement of the FLR, increasing the safety of the hepatectomy as preoperative portal vein embolization does before a major hepatectomy in adult patients with HCC, and the latter suppresses the tumor while waiting for the planned hepatectomy.

Evaluating mortality and disease severity in congenital diaphragmatic hernia using the McGoon and pulmonary artery indices.

PURPOSE: Lung hypoplasia is associated with mortality in congenital diaphragmatic hernia (CDH). However, the association between lung hypoplasia and disease severity is unclear. Early prediction of disease severity would provide parents with more precise information about the anticipated course of treatment, minimize treatment disruption, and maximize the efficient management of patients with CDH. We aimed at identifying the relationship between McGoon index (MGI) and pulmonary artery index (PAI) scores and disease severity among infants with CDH. METHODS: We retrospectively reviewed the medical records of 19 high-risk patients with CDH born between January 2006 and December 2007. McGoon index and PAI scores were determined on admission. We evaluated statistically the relationship between these scores and variables representing severity as follows: number of vasodilators, use of inhaled nitric oxide (iNO), closed method of diaphragm, duration of intubation, duration of hospitalization, and use of home oxygen therapy. Statistical significance was P < .05. RESULTS: Overall median MGI and PAI scores were 1.40 and 108, respectively; scores for nonsurvivors were significantly (P < .05 and P < .01, respectively) lower than those for survivors. Among survivors, PAI scores were significantly (P < .05) lower in infants requiring iNO than in infants not requiring iNO and patch repair. The PAI scores were significantly correlated with the number of vasodilators (r = -0.789; P < .01) and duration of intubation (r = -0.610; P < .05). CONCLUSIONS: McGoon index (cutoff value, 1.31) and PAI (cutoff value, 90) are reliable indices for predicting mortality in CDH. Pulmonary artery index appears to be more useful than MGI for predicting disease severity among survivors.

A novel association of duodenal atresia, malrotation, segmental dilatation of the colon, and anorectal malformation.

We describe two female neonates who suffered from four gastrointestinal anomalies, including duodenal stenosis or atresia, malrotation, segmental dilatation of the colon, and anorectal malformation. Each patient was managed by two or three operations, resulting in good bowel movements. Since this is the first report of four gastrointestinal anomalies, these cases may provide clues to elucidate the etiology of gastrointestinal tract developmental abnormalities.

Repeat STEP procedure to establish enteral nutrition in an infant with short bowel syndrome.

The serial transverse enteroplasty (STEP) procedure is a safe and successful way to lengthen the small bowel in patients with short bowel syndrome. However, postoperative dilatation of the intestine may occur, which induces bacterial overgrowth and malabsorption leading to liver failure. We describe the case of an infant boy with short bowel syndrome caused by jejunal atresia requiring the STEP procedure twice. The first STEP improved the liver function, and the second STEP allowed 80% of the total calorie intake to be tolerated enterally. One should not hesitate to perform a second STEP if after the initial bowel lengthening procedure the patient develops small bowel dilatation that interferes with enteral nutrition.

Prognostic significance of circulating tumor cells and bone marrow micrometastasis in advanced neuroblastoma.

PURPOSE: The aim of this study was to study the prognostic significance of circulating tumor cells (CTC) and the appropriate indications for aggressive surgery in advanced neuroblastoma. MATERIALS AND METHODS: Micrometastasis was sequentially explored using our reverse transcriptase-polymerase chain reaction method in 29 neuroblastoma patients (International Neuroblastoma Staging System stage 4, n = 24; stage 3, n = 5) who treated at our department with the united chemotherapeutic regimen since 1991. Their medical records and detection of CTC and/or the bone marrow micrometastasis were retrospectively reviewed then analyzed statistically. RESULTS: The overall survival rate was 58.6% (17/29). Circulating tumor cells were detected in 55.6% of the stage 4 patients, and all deaths were related to systemic metastases in the CTC-positive patients. The detection of CTC scarcely associated with MYCN amplification. In the patients showing MYCN amplification but no CTC, all deaths were related to local relapse or chemotherapy-associated complications. The survival rate was not significantly different between the patients with and without MYCN amplification (56.8% vs 52.7%). However, it was significantly lower in the patients with CTC and/or persistent bone marrow micrometastasis compared to those without detectable micrometastasis (33.8% vs 87.5%; P < .05). CONCLUSIONS: The presence of CTC and/or persistent micrometastasis may indicate a significantly high risk, regardless of MYCN amplification. Patients with MYCN amplification but no micrometastasis would be most benefited by highly intensive surgery.

An association of gastroschisis and fatal respiratory distress: does prenatal bile aspiration cause early-onset respiratory failure in neonates?

We present a neonate with gastroschisis and evidence of bile aspiration in utero, who developed severe respiratory distress that did not respond to postnatal intensive respiratory care. Although rare, a newborn with gastroschisis may develop severe respiratory distress due to bile aspiration in utero. Given the poor outcome in this case, we suggest a possible role for prenatal diagnosis and therapy.

The impact of strict infection control on survival rate of prenatally diagnosed isolated congenital diaphragmatic hernia.

BACKGROUND/PURPOSE: Although the prognosis of congenital diaphragmatic hernia (CDH) is determined by the degree of pulmonary hypoplasia, there may be an occult contribution of infection to outcomes. The purpose of this study is to evaluate the effects of our new supportive therapy to prevent infectious complications on mortality and morbidity of CDH. METHODS: Among 57 cases with CDH treated between 2002 and 2007, 43 prenatally diagnosed isolated cases were enrolled in this study. All patients were managed by a lung-protective strategy and delayed surgery. Since January 2006, we have optimized our perioperative care to reduce infectious complications by using peripherally inserted central catheters and restriction of invasive procedures including extracorporeal membranous oxygenation (ECMO). The survival rate, intubation period, maximum serum C-reactive protein (CRP) level, and complications were compared before and after the introduction of refined supportive therapy. RESULTS: There were 25 cases (12 liver-up, 13 liver-down) treated before 2006 and 18 cases (8 liver-up, 10 liver-down) after 2006. ECMO was required for stabilization in five cases before 2006. The survival rates of total, liver-up, and liver-down cases improved from 60, 42, 77, to 83, 63, 100% after 2006, respectively. The intubation period was shortened from 37.8 +/- 24.3 to 22.2 +/- 10.8 days, and the maximum serum CRP level declined from 12.8 +/- 11.5 to 2.2 +/- 1.6 mg/dl after 2006. Nine cases developed sepsis before 2006 whereas no patients suffered from sepsis or pneumonia after 2006. CONCLUSION: The new supportive therapy with strict infection control improved survival rate of prenatally diagnosed CDH without using ECMO.

High dose intravenous methylprednisolone resolves esophageal stricture resistant to balloon dilatation with intralesional injection of dexamethasone.

One of the most serious problems in patients with long-gap esophageal atresia or corrosive esophagitis is esophageal stricture, which may require esophageal resection and replacement. We describe two cases with persistent esophageal stricture successfully managed by high dose intravenous methylprednisolone following balloon dilatation. High-dose methylprednisolone with gradual tapering (daily 25, 15, 10, 5, 2 mg/kg for 4 days each) plus cimetidine and ampicillin for 1 week was intravenously administrated immediately after balloon dilatation of the esophageal stenosis. This was followed by oral prednisolone (daily 2, 1, 0.5 mg/kg for 1 week each) for persistent esophageal stricture. High dose intravenous methylprednisolone therapy was given to two patients. One patient was a 5-year-old boy with long-gap esophageal atresia who had undergone repair of the esophagus resulting in severe anastomotic stenosis of 3 cm in length. The other case was a 10-year-old boy with corrosive stenosis caused by alkali ingestion. Both patients had been requiring balloon dilatation of the esophagus with intralesional injection of dexamethasone every 3 weeks for more than 1 year to tolerate oral feeding. After the high-dose methylprednisolone protocol was initiated, the symptoms of dysphagia or choking dramatically improved in both patients, and they remained symptom-free for 8 and 7 months. There were complications of moon faces that resolved concomitantly with the withdrawal of oral prednisolone in both cases. High dose intravenous methylprednisolone in addition to intralesional injection of dexamethasone following balloon dilation is an effective therapeutic strategy for persistent esophageal strictures.

Living donor liver transplantation with hyperreduced left lateral segments.

Liver transplantation is now an established technique to treat children with end-stage liver disease. Implantation of left lateral segment grafts (Couidaud's segments II and III) can be a problem in small infants because of a large-for-size graft. Reduced left lateral segmental liver transplantation has been recently introduced for small infants to mitigate the problem of large-for-size graft. Further reduction of the left lateral segment graft increases the possibility of supplying an adequate hyperreduced left lateral segment graft as an alternative surgical technique. We report 3 cases of our experience of transplantation using hyperreduced left lateral segment grafts from living donors.

Aberrant venous flow measurement may predict the clinical behavior of a fetal extralobar pulmonary sequestration.

OBJECTIVE: Fetal extralobar pulmonary sequestration (EPS) is sometimes complicated by a massive pleural effusion, leading to tension hydrothorax and fetal hydrops. The goal of this study was to examine sonographic signs of venous obstruction in fetal EPS with or without pleural effusion. METHODS: Records of fetal ultrasound from 6 patients with EPS were reviewed with special attention to aberrant arterial and venous flow. The results were correlated with their clinical outcomes. RESULTS: Four of the 6 cases (cases 1-4) were complicated by massive pleural effusion and required fetal thoracentesis; thoracoamniotic shunt placement was required in 3 of these 4 patients (cases 1-3). The other 2 patients (cases 5 and 6) were not associated with pleural effusion despite the comparable size of the mass and did not require any treatment, either prenatally or postnatally. In cases 1-3, aberrant venous flow was difficult to detect and, even when detected, the arterial-to-venous flow velocity ratio was >6. This is in contrast to the uncomplicated cases 5 and 6 in whom aberrant venous flow was easily detected with an arterial-to-venous flow velocity ratio of 2-3. Arterial-to-venous flow velocity ratios of 3-6 were observed in case 4. This case was complicated by pleural effusion but not by fetal hydrops. CONCLUSIONS: These data support the hypothesis that venous obstruction is related to the production of pleural effusion in fetal EPS. Ample flow in the aberrant vein may indicate benign clinical behavior, while difficulty in detecting aberrant venous flow may be correlated with the development of massive pleural effusion.

Resolution of hepatopulmonary syndrome after ligation of a portosystemic shunt in a pediatric patient with an Abernethy malformation.

This report describes a case of Abernethy malformation associated with hepatopulmonary syndrome, which was resolved after shunt ligation. The clinical course indicated that hepatopulmonary syndrome can develop in Abernethy malformation in which liver function and portal pressure is normal, and liver transplantation is not the exclusive therapy for hepatopulmonary syndrome. The levels of endotoxin and endothelin 1 in the shunt blood were high, whereas those of tumor necrosis factor alpha and interleukin-1beta were within reference range. Although pathogenesis of hepatopulmonary syndrome remains unknown, the findings in this case suggest that bacterial translocation as well as elevated endothelin 1 may play a causal role in development of hepatopulmonary syndrome.

Clinicopathologic assessment of prenatally diagnosed lung diseases.

PURPOSE: To investigate the clinical features and pathologic diagnosis of prenatally diagnosed lung diseases. MATERIALS AND METHODS: The medical records of 28 fetuses with prenatally diagnosed lung diseases were reviewed with regard to perinatal courses, ultrasonographic measurement of the lesion volume ratio, and the histopathology of lung tissue. RESULTS: Of the 23 fetuses with a prenatal diagnosis of congenital cystic adenomatoid malformation (CCAM), 4 required an emergency lobectomy (1 prenatally and 3 postnatally), whereas the other 14 showed uneventful courses after birth. The pathologic diagnosis of the resected lungs included CCAM (n = 4), lobar emphysema (n = 2), intralobar sequestration (n = 2), and bronchial atresia (n = 1). The peak value of the lesion volume ratio was significantly higher in the CCAM cases that required urgent surgery than in the non-CCAM cases (2.29 +/- 0.46 vs 1.20 +/- 0.21, P < .001). The ratio decreased after the 25th to the 30th week to less than 1.0 in the 5 non-CCAM cases, but remained higher than 2.0 in the 4 CCAM cases. Prenatally diagnosed extralobar pulmonary sequestration caused massive pleural effusion requiring a fetal intervention in 4 of the 5 fetuses. CONCLUSIONS: The prenatally diagnosed lung lesions include various kinds of pulmonary diseases. Regardless of the diseases, sequential ultrasonographic assessment may predict perinatal risks.

Late complications after surgery in patients with neuroblastoma.

PURPOSE: The purpose of this study was 2-fold: to study the complications of neuroblastoma treatment and to establish surgical guidelines. MATERIALS AND METHODS: The medical records of 92 patients with neuroblastoma (stage 1 or 2, n = 33; stage 3, n = 21; stage 4, n = 31; stage 4S, n = 7) who had undergone surgery in our department between 1985 and 2001 were reviewed, with an emphasis on treatments and late complications. RESULTS: The disease-free survival rate was 98.3% (58/59) in infantile patients, whereas it was 36.4% (12/33) in advanced patients older than 1 year. The rate was improved up to greater than 50% after the introduction of intensive local therapy with intraoperative radiation (IOR). The treatment-associated morbidity rate was 15.0% in nonadvanced infantile patients, was 42.1% in advanced infantile patients, and was 33.3% in advanced older patients. In the advanced cases, renovascular problems were most frequently seen especially after IOR. Furthermore, pulmonary problems, cardiac problems, and second cancer were highlighted as fatal problems; thyroid dysfunction, vertebral deformity, and growth retardation were also noted. CONCLUSIONS: Higher incidences of late morbidity and treatment-associated mortality appeared to be more strongly related to disease stage rather than to patient age. Appropriate surgery and IOR with lower doses should be recommended as a surgical strategy for high-risk patients.

Current role of liver transplantation for methylmalonic acidemia: a review of the literature.

To evaluate the current role of liver transplantation (LT) for methylmalonic acidemia (MMA), we reviewed the literature on outcomes of this treatment, and describe three of our own cases of living-donor liver transplantation (LDLT). The total number of LT cases identified was 18. Transplantation mode was deceased donor LT in 12, including five combined liver-kidney transplantations (CLKT) from deceased donors, and LDLT in six. Three hospital mortalities were noted, because of metabolic decompensation, sepsis and aspergillosis. Although mean postoperative serum MMA level decreased to 13.8% +/- 9.2% (range 1.25-26.1%) of preoperative levels, four patients (22.2%) had renal insufficiency after isolated LT and three (16.7%) had postoperative neurological disability. Continuing metabolic damage to the kidney and brain may occur even after successful LT. Further evaluation is required to determine the long-term suitability of this treatment modality.

Venous arterialization in extralobar pulmonary sequestration associated with fetal hydrops.

BACKGROUND/PURPOSE: Fetal extralobar pulmonary sequestration (EPS) is sometimes complicated by massive pleural effusion (PE) leading to fetal hydrops. The underlying mechanisms as well as the origin of the fluid are not well understood. This study was performed to find a histological hallmark of an EPS with massive PE. We hypothesized that venous obstruction has a role to play in the mechanisms of fluid production by EPS. METHODS: We recently experienced 3 cases of fetal EPS complicated by fetal hydrothorax requiring thoracentesis and eventually thoracoamniotic shunt placement. Total protein content and cell count were measured in the aspirates, which were compared with chylothorax cases (n = 5) requiring fetal shunt placement. After birth, all 3 infants required mass resection for the control of PE. The venous wall thickness was measured on pathology slides stained with Elastica van Gieson staining. Thickness of the media and adventitia was measured in approximately 40 veins per case. They were corrected by external diameter and expressed as percentage of medial thickness and percentage of adventitial thickness. An EPS not associated with PE but with congenital diaphragmatic hernia served as a control. RESULTS: Total protein and the cell count of the EPS related PE were 0.6 +/- 0.3 mg/dL and 28 +/- 14/microL (mean +/- SD), which were significantly lower than those of PE in chylothorax (2.2 +/- 0.2 mg/dL and 1900 +/- 1100/microL). Percentages of adventitial thickness of EPS with PE were 9.8% +/- 3.8%, 10.4% +/- 3.6%, and 8.3% +/- 3.7%, which were significantly increased compared with the control of 3.1% +/- 1.3% (P < .01). Percentages of medial thickness of EPS with PE were 7.0% +/- 1.9%, 7.3% +/- 1.4%, and 6.6% +/- 2.3%, which were significantly increased compared with the control of 2.3% +/- 0.7% (P < .01). CONCLUSIONS: We conclude that PE associated with EPS is the transudate rather than the lymph. The thickened media and the adventitia found in EPS with PE support the hypothesis that partial obstruction of the venous system leads to an increased transudate production, which ultimately leads to fetal hydrops.

Congenital bronchial atresia in infants and children.

BACKGROUND/PURPOSE: Congenital bronchial atresia (CBA) usually presents incidentally in asymptomatic young male adults but is rarely diagnosed in children. The aim of this study was to clarify the clinical characteristics of CBA in childhood and to describe the spectrum of this condition. METHODS: The clinical features in 29 patients with CBA, aged from 1 day to 13 years (median, 4 years), were reviewed retrospectively. Diagnosis was confirmed by pathological findings of a blind-ending bronchus associated with distal mucous-filled bronchocele surrounded by hyperinflated lung parenchyma. RESULTS: All but 1 patient were symptomatic. The most frequent symptom was productive cough and fever owing to recurrent pneumonia found in 26 children. Two infants suffered from respiratory distress. Chest x-ray showed various findings of infiltrative pneumonia, emphysema, and a large cyst. Computed tomography, bronchography, and bronchoscopy were useful modalities for demonstrating bronchocele associated with hyperinflated lung or proximal blind-ending bronchus even in infected cases. The right lower lobe was predominantly affected in 12 cases, followed by left or right upper lobe in 7 cases. Lobectomy or segmentectomy resulted in remarkable clinical improvement. CONCLUSIONS: Congenital bronchial atresia presents differently in children than in young adults. Modern imaging techniques and careful pathological analyses lead to an accurate diagnosis of bronchial atresia, which may be misdiagnosed as intralobar sequestration or pulmonary bronchial cysts. Bronchial atresia is a distinct pathological entity that accounts for recurrent pneumonia or respiratory distress in childhood, requiring surgical treatment.

Liver position in fetal congenital diaphragmatic hernia retains a prognostic value in the era of lung-protective strategy.

BACKGROUND/PURPOSE: The aims of this study were to analyze the outcomes of fetuses with congenital diaphragmatic hernia (CDH) treated by a lung-protective strategy using high-frequency oscillatory ventilation (HFOV) in a single center with a perinatology service and extracorporeal membrane oxygenation (ECMO) capability and to define the natural history of CDH in the era of lung-protective ventilation. METHODS: A retrospective chart review of 30 neonates with CDH seen between April 2002 and October 2004 was conducted. All fetuses with a prenatal diagnosis were evaluated by fetal magnetic resonance imaging to define the liver position, and those with a significant volume of the liver in the chest were regarded as liver-up. Patients were managed by a lung-protective strategy using pressure-limited (maximum mean airway pressure [MAP], 18 cm H(2)O) HFOV. The patients were initially placed on HFOV with a fraction of inspired oxygen (Fio(2)) of 1.0 and a MAP of 12 cm H(2)O. Hypercapnea and preductal saturation as low as 85% were accepted. Inhaled nitric oxide and ECMO were introduced when the baby could not be oxygenated with a MAP of 18 cm H(2)O. RESULTS: Twenty-six neonates (22 inborns with prenatal diagnosis and 4 outborns) were treated with this protocol. Four cases were not treated or died in utero because of severe associated anomalies. Thirteen of the 14 liver-down cases survived without ECMO and were discharged home (93% survival). On the contrary, 4 of 12 liver-up cases survived (33% survival). ECMO was required for initial stabilization in 5 cases with 1 survivor. CONCLUSIONS: Liver-down CDH babies have a good chance for survival without ECMO by a planned delivery and the lung-protective strategy using HFOV. Liver herniation demonstrated by prenatal magnetic resonance imaging retains a poor prognostic value even with this approach.

Lethal gastric rupture caused by acute gastric ulcer in a 6-year-old girl.

Gastric rupture is extremely rare in childhood beyond the neonatal period. We describe a previously healthy 6-year-old girl with a large laceration along the greater curvature on the posterior wall of the stomach. The patient was admitted to a neighboring hospital because of vertigo with 1-day history of intractable vomiting and epigastric pain. Although abdominal distension was noticed during the physical examination, muscular rigidity was not detected on palpation. Laboratory data showed severe hypotonic dehydration and moderate metabolic acidosis with hyperkalemia. She suddenly developed cardiac arrest within an hour after admission. After resuscitation, surgical consultation was obtained. Abdominal X-ray and ultrasound revealed abdominal free air and massive cloudy ascites. At laparotomy, there was a large laceration on the greater curvature of the stomach. Pathology of the gastric wall showed mucosal necrosis while the musculature remained intact consistent with an acute gastric ulcer. Since clinical condition of gastric rupture deteriorates rapidly, early diagnosis and appropriate treatment is essential for good prognosis. One should consider that ulcer formation might cause gastric rupture in childhood.

Tumor cell dynamics and metastasis in advanced neuroblastoma.

This study deals with the advancement process of neuroblastoma through clinical observations and circulating tumor cell exploration. Clinical feature, tumor biology, and circulating tumor cell detected by the previously described polymerase chain reaction (PCR) method were analyzed in 31 patients with advanced neuroblastoma treated in our department since 1991 through 2004. Treatment was completed in 28 patients, of whom 17 are alive without the disease and 11 died. The primary lesion was not confirmed in 2 patients with disseminated metastasis, both of whom showed positive circulating tumor cell. Circulating tumor cell was positive in 6 of 9 examined at their first appearance at the hospital, all had stage 4 disease, and 4 of the 6 (66.7%) died of systemic spread of the disease. N-myc was amplified in 15 patients, of whom only 2 (13.3%) died of systemic metastasis. N-myc amplification did not correlate with positive circulating tumor cell. A certain population of neuroblastoma may provide circulating tumor cells from the early period of the disease to form metastatic lesions independently of the primary lesion, which must be regulated by factors other than N-myc. Circulating tumor cells may suggest higher risk for systemic dissemination and poor prognosis.