Takahiko Seo

Pancreaticobiliary maljunction without choledochal cysts in infants and children: Clinical features and surgical therapy

Pancreaticobiliary maljunction (PBM) usually is associated with choledochal cyst. PBM without dilatation of the common bile duct is rare in infants and children. This rare type of the anomaly may lead to the development of malignancy of the bile duct in later life. The authors report the clinical presentation and surgical treatment of seven pediatric patients with PBM. Symptoms and signs included repeated episodes of abdominal pain (7 of 7), nausea and vomiting (6 of 7), intermittent jaundice (3 of 7), and acholic stools (2 of 7). Some patients presented with high levels of serum and urinary amylase. These symptoms and signs might have been related to the temporary obstruction of bile flow in the common channel, where endoscopic retrograde cholangiopancreaticography (ERCP) disclosed a protein plug in four of the patients. The common bile duct proximal to the junction of the main pancreatic duct was excised, and a Roux-en-Y hepaticojejunostomy reconstruction was performed. To prevent iatrogenic injury of the main pancreatic duct, repeat cholangiography was performed with the aid of radiopaque silver clips placed on the line of dissection to evaluate the distance between the site of dissection and the main pancreatic duct. All patients have been free of symptoms since the surgery. PBM without dilatation of the common bile duct can be detected more frequently if ERCP is performed on every patient who has repeated episodes of abdominal pain refractory to conventional therapy. Complete excision of the common bile duct and gallbladder followed by hepaticojejunostomy is recommended for PBM, while the goals of decreasing the high risk of carcinoma of the bile duct and preventing recurrent symptoms.

Torsion of an accessory spleen presenting as an acute abdomen with an inflammatory mass. US, CT, and MRI findings.

Torsion of an accessory spleen is extremely rare. Only seven cases in children have been reported in the literature. This entity should be considered in the differential diagnosis of acute abdomen associated with an intraperitoneal inflammatory mass. This report describes a 10-year-old boy with severe abdominal pain and a mass that was found to be due to infarction of an accessory spleen that had twisted on its pedicle. Magnetic resonance imaging taken at two different times was helpful in detecting an inflammatory mass, while ultrasonography and computed tomography indicated only the presence of an intraperitoneal mass.

Treatment of hepatoblastoma : Less extensive hepatectomy after effective preoperative chemotherapy with cisplatin and Adriamycin

BACKGROUND Although the prognosis of hepatoblastoma was improved by the introduction of cisplatin and doxorubicin (Adriamycin) for adjuvant chemotherapy, extensive hepatectomy continues to be the usual practice. We retrospectively reviewed our recent experience with hepatoblastoma to determine whether the new modality of intensive chemotherapy could change the resectability, extent of hepatectomy, operative complications, and prognosis. METHODS The clinical features of 15 children with hepatoblastoma treated between 1985 and 1995 were reviewed. Intensive chemotherapy was added before surgical resection not only when a tumor was unresectable but also when it was large enough to increase the risk of operative morbidity. RESULTS There was 100% resectability, and the overall mortality rate was only 6.7%. Fourteen patients have been free of disease for 2 to 12 years. Preoperative chemotherapy enabled resection of six previously unresectable hepatoblastomas. Moreover, hepatic resection tended to be less invasive in several patients whose tumors had been much reduced after preoperative chemotherapy. Intraoperative and postoperative complications were minimal, with a short operative time and small amount of blood loss, especially in the group with delayed primary operation. CONCLUSIONS The preoperative administration of cisplatin and Adriamycin reduced the tumor size so that a safe hepatectomy could be performed with less blood loss and minimal technical complications. Unnecessary sacrifice of the normal hepatic tissue was avoided by performing the less extensive hepatectomy.

Operative treatment of congenital stenoses of the intrahepatic bile ducts in patients with choledochal cysts

BACKGROUND Postoperative complications including intrahepatic calculi may develop after the complete excision of a choledochal cyst. Since congenital stenoses of the intrahepatic bile ducts are more likely the cause of intrahepatic calculi, operative procedures for intrahepatic stenoses are reported. METHODS There were 16 patients with choledochal cysts who underwent surgery for stenoses of intrahepatic bile ducts. The stenoses were excised at the opening of the common hepatic duct. RESULTS In the 16 patients, 25 of the 26 stenoses that involved an intraluminal membrane or septum could be excised from the divided end of the common hepatic duct at the hepatic hilum. In 1 patient, the stenosis could not be accessed from the hepatic hilum, and a left hepatic lobectomy was required. In postoperative follow-up, all 16 patients were in good health. CONCLUSIONS Stenoses of the intrahepatic bile ducts should be treated from the divided end of the common hepatic duct at the initial operation for choledochal cysts. The need for a second operation or hepatic lobectomy may thus be avoided.

Coronary arterial perfusion during venoarterial extracorporeal membrane oxygenation

The effects of venoarterial extracorporeal membrane oxygenation on left ventricular performance have not been studied in detail. Coronary arterial flow obtained by direct measurement with an electromagnetic flowmeter and blood gas analysis from the aortic root were tabulated during venoarterial extracorporeal membrane oxygenation 14 puppies, and these parameters were evaluated with respect to changes in the venoarterial extracorporeal membrane oxygenation flow. Unique automatic blood pumps generating pulsatile flow were used for the venoarterial extracorporeal membrane oxygenation bypass. Coronary arterial flow decreased as the extracorporeal membrane oxygenation flow increased (106 +/- 26 ml/min per 100 gm of left ventricle at 20 ml x min(-1) x kg bypass flow to 71 +/- 17 ml/min per 100 gm of left ventricle at 100 ml x min(-1) x kg bypass flow, p < 0.01). There were no significant changes in the mean or diastolic pressures in the ascending aorta despite changes in the extracorporeal membrane oxygenation flow. Arterial oxygen tension in the ascending aorta was not increased even under high-flow venoarterial extracorporeal membrane oxygenation. This result indicates that oxygenated blood from the extracorporeal membrane oxygenation circuit does not pass in a retrograde fashion into the aortic root and thus does not perfuse the coronary arteries. The diastolic aortic pressure did not correlate with the changes in extracorporeal membrane oxygenation flow. The decrease in coronary arterial flow is therefore predominantly caused by increased coronary arterial resistance. Tension-time index, an indicator of myocardial oxygen consumption, did not decrease with venoarterial extracorporeal membrane oxygenation. In conclusion, high-flow venoarterial extracorporeal membrane oxygenation causes undesirable hemodynamic effects on the left ventricle.

Acute respiratory failure associated with intrathoracic masses in neonates

BACKGROUND Intrathoracic masses are uncommon in children. Occasionally, they present with acute respiratory failure in the neonatal period. Although emergency resection usually is the treatment of choice, other modalities are sometimes necessary to stabilize the patient. METHODS Seven neonates with intrathoracic masses were treated. Five had congenital cystic adenomatoid malformations (CCAM), 1 had a mediastinal teratoma, and 1 had a pneumatocele. These cases were reviewed retrospectively. RESULTS Four of the 7 infants had respiratory failure in the neonatal period. A patient with a large mediastinal teratoma and 1 with a CCAM that increased rapidly after presentation underwent emergency operation, relieving respiratory distress. The other 2 large CCAMs presented with severe respiratory distress immediately after birth because of pulmonary hypoplasia. One neonate with a Stocker-I CCAM died after emergency resection. One more recent patient with a Stocker-III CCAM survived after successful treatment with delayed resection, performed 3 days after birth. Nitric oxide (NO), and extracorporeal membrane oxygenation (ECMO) were instituted as supportive care because of profound persistent fetal circulation (PFC). CONCLUSIONS Acute respiratory failure associated with intrathoracic masses in neonates may be managed in 1 of 2 ways. A small mass that increases rapidly should be resected soon after presentation. In neonates with large masses with associated PFC, surgery can be delayed until the patient is stable. ECMO, NO, and high-frequency oscillation (HFO) can be used aggressively for stabilizing such neonates.

Attenuated nitrergic inhibitory neurotransmission to interstitial cells of Cajal in the lower esophageal sphincter with esophageal achalasia in children

Abstract Background : Esophageal achalasia (EA) is a rare disease in children, the etiology and pathogenesis of which remain controversial. Previous studies have suggested that a specific class of interstitial cells of Cajal (ICC) act as mediators in nitrergic inhibitory neurotransmission in the lower esophageal sphincter (LES). The aim of this investigation is to clarify the status of ICC and nitrergic inhibitory neurons in the LES of EA using immunohistochemistry.

The MRI findings of the right-sided fetal lung can be used to predict postnatal mortality and the requirement for extracorporeal membrane oxygenation in isolated left-sided congenital diaphragmatic hernia.

We evaluated whether a correlation existed between fetal pulmonary magnetic resonance imaging (MRI) findings and postnatal mortality, as well as the requirements for extracorporeal membrane oxygenation (ECMO) in infants with prenatally diagnosed, isolated left-sided congenital diaphragmatic hernia (CDH). Twenty-one pregnant women carrying fetuses with CDH underwent 30 MRI scans, and the right-sided fetal lung volume (FLV) was measured. In the control, a regression analysis was performed to associate FLV with gestational age. This yielded a formula that enabled the calculation of the expected right fetal lung volume (ERFLV). In cases with CDH, the right-sided observed FLV/ERFLV (= %RFLV) was compared with both the postnatal mortality and whether ECMO was required. Additionally, we investigated the relationship between the lung shape on MRI (whether there was a complete pulmonary baseline present) and postnatal mortality. The %RFLV was significantly lower in nonsurvivors compared with survivors. Among survivors, the %RFLV was significantly lower in infants who required ECMO compared with those who did not. The pulmonary baseline was completely present in 3 (38%) and 13 (100%) of the nonsurvivors and survivors, respectively. In isolated left-sided CDH, the %RFLV is a good predictor not only of postnatal mortality but also of the requirement for ECMO.

Morphological investigation of the enteric nervous system in Hirschsprung's disease and hypoganglionosis using whole-mount colon preparation

BACKGROUND/PURPOSE A suction rectal mucosal biopsy with positive staining for acetylcholinesterase is a useful test for diagnosis of Hirschsprungs disease (HD). However, hypoganglionosis has not been diagnosed by a rectal mucosal biopsy. The authors morphologically examined the enteric nervous systems in HD and hypoganglionosis patients using whole-mount preparations. METHODS Six HD patients, two hypoganglionosis patients, and 10 with normally innervated colons were examined. Colonic specimens were incubated with the primary antibodies against protein gene product 9.5 (PGP 9.5) mixed with S-100b protein, tyrosine hydroxylase (TH), calcitonin gene-related peptide (CGRP), substance P (SP), and neurofilament protein 200 kDa (NFH). They were observed by histochemical technique using light-microscopy in whole-mount preparations. RESULTS The aganglionic distal colon had thick nerve strands stained with PGP 9.5 mixed with S100 or NFH located in the layer between the longitudinal muscle and the circular one, and the submucosal layer. The nerve strands in the myenteric layer contained few CGRP- and SP-positive fibers and ran along the long axis of the intestine. Ganglion cells appeared along with those thick nerve strands in the transitional zone of HD. In hypoganglionosis, we found small myenteric ganglia with no thick nerve strands. CONCLUSIONS The enteric nervous system in oligoganglionic segments of HD morphologically differed from the one in hypoganglionosis. A suction rectal mucosal biopsy would be of no use in the diagnosis of hypoganglionosis.

Surgical removal of Protein plugs complicating choledochal cysts : Primary repair after adequate opening of the pancreatic duct

BACKGROUND/PURPOSE Recently, pancreaticoduodenectomies have been performed for choledochal cysts complicated by protein plugs. The authors describe a new surgical procedure for removal of protein plugs or pancreatic stones that does not involve pancreatectomy. METHODS Protein plugs were detected in 43 (34.1%) of 126 pediatric patients with choledochal cysts. The plugs disappeared spontaneously in 26 patients and were removed using nonsurgical methods in seven. The 10 patients treated surgically are considered here. RESULTS Protein plugs were removed with the residual cyst in one patient. The plugs in the common channel were flushed into the duodenum by irrigation with saline through the narrow segment beneath choledochal cyst in another. The plugs were removed through the narrow segment using a blunt spoon in four. In the other four patients, the plugs that were too large or too hard to be removed through the narrow segment were taken away through an incision in the main pancreatic duct. This was followed by primary closure of the main pancreatic duct using the narrow segment as a flap to prevent stricture formation. All of the patients have done well. CONCLUSIONS Protein plugs complicating choledochal cysts can be removed without pancreatectomy. Particularly, primary repair of the pancreatic duct using a flap made of the narrow segment beneath the choledochal cyst is a safe and feasible method to remove large or hard protein plugs.