Surgery for long tubular intestinal duplication with massive hemorrhage: a case report and literature review.

BACKGROUND: Long tubular duplication is a rare congenital intestinal disease, that can lead to emergency situations marked by massive hemorrhage. However, preoperative diagnosis and surgical treatment are challenging. This report presents preoperative images and details a surgical procedure for long tubular intestinal duplications with massive hemorrhage. CASE PRESENTATION: A 3-year-old boy presented to the emergency department with melena. Despite undergoing a Tc-99m pertechnetate scintigraphy one year prior, which revealed nonspecific findings with enhancement of some parts of the intestine, enhanced abdominal CT revealed an edematous small intestine with luminal extravasation. The patient received a transfusion of red blood cells; however, his hemoglobin level did not improve. Arterial angiography and double-balloon endoscopy revealed no remarkable findings. Exploratory laparotomy revealed a long tubular duplication in half of the small intestine. Utilizing the Wrenn procedure, we successfully removed all duplicate mucosa. Pathological findings showed that almost all duplications contained gastric mucosa and revealed an ulcer with a ruptured arterial vessel. His symptoms were resolved, and the hemoglobin level stabilized. At 2 months postoperatively, no surgical complications were present. CONCLUSIONS: Effective management of long tubular duplications with massive hemorrhage involves timely application of the Wrenn procedure. Recognition of specific imaging findings is crucial to prompt exploratory laparotomy, ensuring optimal outcomes and preventing delays in treatment.

Gallstones in patients with severe motor and intellectual disability.

BACKGROUND: Asymptomatic gallbladder stones may be detected with ultrasound; some gallstones produce symptomatic diseases, such as cholecystitis, cholangitis, or pancreatitis. Identifying the clinical features of symptomatic gallstones may help prevent severe complications by providing intervention for asymptomatic gallstones. We aimed to investigate risk factors associated with developing symptomatic disease in pediatric patients with severe motor and intellectual disabilities (SMID). METHODS: This retrospective study enrolled 30 patients with SMID who were treated at the Nara Medical University between March 2016 and March 2019. We examined the prevalence of gallstones, and the rate at which associated symptoms of gallstones were observed in patients. Furthermore, we compared the clinical features of patients with and without gallstones. RESULTS: Among 30 patients with SMID, 7 (23%) had gallstones, with 6 (86%) of them being symptomatic: 3 patients had acute pancreatitis and 3 had cholecystitis or cholangitis. Among 23 patients without gallstones, 2 had acute pancreatitis and 2 had biliary sludge. Patients with gallstones had significantly lower daily calorie intake than those without gallstones (P = 0.042). Furthermore, the incidence of gallstones was higher in patients who received total parenteral nutrition than in those who did not (P = 0.031). Comparative analysis between symptomatic and asymptomatic patients was not performed because almost all cases were symptomatic. CONCLUSION: Gallstones were detected in 23% of patients with SMID. The gallstones were symptomatic at a very high rate. Considering this fact, patients with SMID should be surveyed for gallstones; careful management may be needed in such patients.

Nonalcoholic fatty liver disease developed after pancreatoduodenectomy for solid pseudopapillary neoplasm in a 10-year-old girl: a case report.

BACKGROUND: Solid pseudopapillary neoplasms of the pancreas are rare. Moreover, pancreatoduodenectomy (PD) and postoperative care are not common in pediatric surgery. Herein, we report a case of PD and nonalcoholic fatty liver disease (NAFLD) after PD and present a literature review. CASE PRESENTATION: A 10-year-old girl with a suspected liver tumor was referred to our hospital. Echography, enhanced computed tomography and magnetic resonance imaging showed that the tumor coexisted with the solid and cystic parts of the pancreatic head. Since the patient was a young woman and the imaging findings were consistent with that of pancreatic solid pseudopapillary neoplasms (SPNs), we diagnosed her with pancreatic SPN. Thereafter, PD was performed, and she was discharged 10 days after the operation. Although her postoperative course was mostly uneventful, she experienced few episodes of abdominal pain and diarrhea before hospital discharge. These symptoms subsequently became more frequent and severe. The patient was urgently readmitted to the hospital for watery steatorrhea and lower abdominal colic pain. Her serum aspartate aminotransferase and alanine aminotransferase levels were elevated, and a fatty liver was detected on echography. The patient was diagnosed with steatorrhea, peristaltic pain, and NAFLD after PD. Pancrelipase (containing pancreatic digestive enzymes), antidiarrheal agents, and probiotics were started. Dosage increase of these drugs reduced the defecation frequency and abdominal pain and switched diarrhea to loose stools. However, more lipids in meals or more meals caused diarrhea and abdominal pain. Therefore, the doses of these drugs were further increased, and another antidiarrheal agent, loperamide hydrochloride, was added. Exocrine pancreatic enzymes supplementation and careful follow-up should prevent NAFLD progression after PD. At present, the patient has occasional abdominal pain, but has tangible soft stools once or twice a day. Although echography still shows a mottled fatty liver, her hepatic enzymes are only mildly elevated. CONCLUSIONS: Pediatric PD is rare, and residual pancreatic function is usually sufficient, unlike in adult cases. However, we experienced a case of NAFLD after PD for a pediatric pancreatic SPN, in which pancreatic enzyme supplementation effectively improved this condition. Further attention must be paid to worsening of NAFLD that can develop nonalcoholic steatohepatitis.

Laparoscopic mucosectomy for gastric duplication cysts that communicated with the spleen in a 10-year-old girl: A case report.

We report a case of laparoscopic mucosectomy for gastric duplication cysts that communicated with the spleen. A 10-year-old girl visited a local hospital with a chief complaint of intermittent left abdominal pain that had lasted for about 2 months. We diagnosed two gastric duplication cysts by ultrasonography and planned a laparoscopic extirpation. The elliptical masses were found in the splenic hilum and were 5 and 3 cm in diameter. The bigger one communicated with the spleen, so cystectomy could not be performed. Considering the risk of hemorrhage and the patient's age, we performed a mucosectomy rather than a partial splenectomy. The patient had an uneventful postoperative course. We histologically diagnosed gastric duplication cysts. Laparoscopic mucosectomy is a useful procedure for gastric duplication cysts that communicate with the spleen.

Potential role of the PD-L1 expression and tumor-infiltrating lymphocytes on neuroblastoma.

PURPOSE: The programmed death 1 (PD-1)/programmed death ligand 1 (PD-L1) pathway has garnered much attention for its roles in clinical oncology. The aim of this study was to examine the clinical impact of the PD-L1 expression and tumor-infiltrating lymphocytes (TILs) on neuroblastoma. METHODS: We evaluated the PD-L1 expression and TIL status in 31 patients with neuroblastoma who underwent a biopsy or resection by an immunohistochemical analysis. Furthermore, we performed the serial analysis of the PD-L1 status before and after chemotherapy in 15 patients. RESULTS: Among the 31 cases, 11 (35%) showed a positive PD-L1 expression. The survival analysis showed a trend toward an association between PD-L1 positivity and a decreased overall survival. PD-L1 positivity tended to be associated with higher levels of tumor markers. In the serial analysis of the PD-L1 status, positivity was noted in 8 of 15 patients before chemotherapy and 6 after chemotherapy. Notably, all four patients with a positive PD-L1 status both before and after chemotherapy had recurrence, and 3 of them died during the follow-up period. CONCLUSION: Our findings suggest that the PD-L1 tumor expression might be a good biomarker for the treatment of neuroblastoma patients, especially for advanced neuroblastoma.

A novel approach to neonatal abdominal surgery via a circular incision around the umbilical cord.

We present a new, scarless, circular incisional approach around the umbilical cord for neonates with intestinal atresia. This novel approach achieves truly woundless surgery. It is simple, safe, and can be used for an intestinal surgical treatment in neonates.

Single-site laparoscopic herniorrhaphy using needle instruments for inguinal hernias in children: a novel technique.

We describe our new technique for laparoscopic herniorrhaphy with subumbilical single-site access to treat inguinal hernias in children. First, we inserted a transparent 3-mm trocar and a 2-mm minitrocar at the umbilicus. We then inserted a 3-mm 45° camera through the 3-mm trocar and needle-grasping forceps through the 2-mm trocar. We closed the hernia defect by using a 19-gauge hooked injection needle with a nonabsorbable suture. We treated 11 consecutive female children with inguinal hernia using this operation. The mean operating time was 26.7 min (range 21-36 min) and the procedure was technically successful, without the need for additional trocars, in all 11 patients. There were no intraoperative complications and all the patients were discharged on the same day after the surgery. Single-site laparoscopic herniorrhaphy using needle instruments is feasible and seems to be safe. Further studies are required to determine whether this approach would benefit patient compared with standard laparoscopic herniorrhaphy.

Radiographic changes in the diaphragm after repair of congenital diaphragmatic hernia.

BACKGROUND/PURPOSE: The growth and function of the repaired diaphragm have not been well elucidated, which may contribute to pulmonary function and chest wall deformity. We measured the lower lung diameter at the top of the diaphragm (LLD), diaphragmatic diameter (DD), and diaphragmatic height (DH) on the posteroanterior plain chest radiograph using a picture archive and communication system. METHODS: Thirty-six children aged 10.4 +/- 4.8 years with congenital diaphragmatic hernia underwent clinical evaluation, including plain chest radiograph and a lung ventilation/perfusion scan. As a control, chest radiographs of 89 children aged 9.0 +/- 5.5 years with minor surgery were analyzed. RESULTS: The LLD, DD, and DH in controls were significantly correlated with age; each value was then expressed as a percentage of age-based estimated values. Ipsilateral LLD and DD were significantly decreased. The perfusion of the ipsilateral lung was best correlated with ipsilateral DD. Five patients had chest wall deformity, and 7 had scoliosis (Cobb angle >10 degrees ). Patients with scoliosis had decreased ipsilateral LLD, DD, and DH. The Cobb angle was correlated with LLD and DD. CONCLUSION: The growth of the repaired diaphragm may be impaired, which contributes to decreased perfusion of the ipsilateral lung and scoliosis. The LLD and DD are simple but useful parameters in the follow-up of patients with CDH.

Prenatal detection of pulmonary hypoplasia in giant omphalocele.

Respiratory insufficiency has sometimes been reported in giant omphalocele. To determine whether ultrasonic fetal lung measurements including lung/thorax transverse area ratio (L/T) and chest/trunk length ratio (C/T) may be useful in predicting associated pulmonary hypoplasia, 28 fetuses with abdominal wall defects between 1991 and 2003 were reviewed. Nine patients with gastroschisis and 19 with omphalocele were classified into three groups. A group with neonatal death and postmortem lung/body weight ratio below 0.012, which was defined as pulmonary hypoplasia (PH group), included two ruptured giant omphaloceles and two giant omphaloceles with intact covering membrane in utero. A group with artificial ventilation more than 3 months, which was defined as prolonged ventilation (PV group), included one ruptured giant omphalocele and three giant omphaloceles with intact covering membrane. Others were defined as ordinary group. In 12 fetuses with giant omphalocele, the evisceration rate of the liver (LER) was measured in the fetal transverse abdominal dimension including the base of the liver. The L/T in PH group was significantly decreased to other groups. The C/T in PH group was significantly increased to ordinary group. There was no significant difference in the LER among three groups. A measurement of L/T may be useful in predicting associated pulmonary hypoplasia in giant omphalocele. However, antenatal detection of patients required prolonged ventilation may be difficult and require further study.

Relationship between L/T ratio and LHR in the prenatal assessment of pulmonary hypoplasia in congenital diaphragmatic hernia.

The lung to thorax transverse area ratio (L/T ratio) and the lung area to head circumference ratio (LHR) have been widely used for the assessment of pulmonary hypoplasia in fetal congenital diaphragmatic hernia (CDH). The aim of this study was to evaluate the relationship between the L/T ratio and the LHR, and to clarify the characteristics of these two indicators as prognostic predictors by means of retrospective concurrent measurements from the same subjects with prenatally diagnosed fetal CDH. The medical records of 55 fetuses who had undergone a prenatal evaluation of isolated CDH from 1988 to 2006 were studied. The L/T ratio and the LHR were determined as the early values (earliest measurement performed earlier than 33 weeks of gestation) and as the late values (latest measurement performed later than 34 weeks of gestation) and analyzed, as well as the clinical data. Of the 55 infants, 13 died resulting in a 76.4% survival rate. A correlation expressed in the linear equation [(LHR) = 14.4 x (L/T ratio) - 0.11] was recognized between the early L/T ratio and the early LHR. All cases with an early L/T ratio of less than 0.08, or with an early LHR less than 1.2, died. Of the 13 cases, 5 with an early L/T ratio not lower than 0.08, but less than 0.13, died. Of the 17 cases, 4 with an early LHR not lower than 1.2, but less than 2.0, died. All cases with an early L/T ratio not lower than 0.13, or with an early LHR not lower than 2.0, survived. In 24 cases, the late values, which were measured at an interval of more than 4 weeks, were compared with the early values. Although the L/T ratio was consistent, the LHR increased in the late value compared to the early value. A good linear correlation was recognized between the L/T ratio and the LHR in the early phase of gestation, and the cutoff point of the prognostic prediction was determined in both indicators. In contrast to the L/T ratio, a definite cutoff point throughout the gestation may not be available in the LHR, because there is a natural increase of the LHR in the late phase of gestation.

Long-term outcome in patients with prenatally diagnosed cystic lung disease: special reference to ventilation and perfusion scan in the affected lung.

BACKGROUND/PURPOSE: The natural history of cystic lung disease (CLD) such as congenital cystic adenomatoid malformation (CCAM) and pulmonary sequestration has been altered by the advent of prenatal diagnosis. Although recent advances including fetal therapy have gradually improved outcome, the long-term course and the function of the residual lung have not been well clarified. METHODS: Twenty-two patients with CLD who had been prenatally diagnosed and treated between 1990 and 2004 were reviewed. The clinical outcome and growth measurements were established, and, where possible, all infants underwent ventilation and perfusion lung scan. RESULTS: Mediastinal shift was present in 14 fetuses. Fetal hydrops was present in 5 fetuses. Antenatal intervention was performed for hydrops in 2 fetuses (cyst-amniotic shunt and aspiration). Twenty-one infants underwent appropriate excisional surgery. Final diagnosis included CCAM (n = 12) and pulmonary sequestration (n = 7). No late death was observed. Common complications were failure to thrive (n = 5), frequent respiratory tract infection (n = 4), and asthmatic attack (n = 4). A significant decrease in lung ventilation and perfusion on the affected side was observed in patients with hydrops, lobectomy, and CCAM. CONCLUSION: Long-term follow-up including respiratory care and growth assessment should be performed in prenatally diagnosed patients with CLD, especially those who present with hydrops.

Long-term follow-up of patients with high-risk congenital diaphragmatic hernia.

BACKGROUND/PURPOSE: Recent advances including prenatal diagnosis, high-frequency oscillatory ventilation, and nitric oxide inhalation therapy have gradually improved the survival of high-risk congenital diaphragmatic hernia. However, the factors affecting the long-term outcome of these patients have not been well established. METHODS: Thirty-three children with ages 4.1 +/- 2.5 years underwent clinical examination including growth measurements, echocardiography, ventilation, and perfusion scintigraphy. RESULTS: No late death was observed. Common complications were frequent respiratory tract infection (13 patients) and bowel obstruction (5 patients underwent surgery). Although frequent respiratory tract infection decreased with increasing age, patients with frequent respiratory tract infection had a decreased uptake of lung ventilation and perfusion scintigraphy on the affected side and had a decreased height for age and weight for height. No significant difference in lung ventilation and perfusion scintigraphy was observed between patients treated with and without extracorporeal membrane oxygenation, those requiring oxygen more than 1 month, and between those with and without prenatal diagnosis. Patients with a patch repair had decreased uptake on lung perfusion scintigraphy. Although frequent respiratory tract infection may be owing to hypoplasia of the ipsilateral lung, it may impair recovery of the hypoplastic lung. CONCLUSION: These results indicate that monitoring for respiratory tract infection in addition to nutritional assessment should be required in the follow-up of patients with congenital diaphragmatic hernia at high risk.

Assessment of obstructive apnea by using polysomnography and surgical treatment in patients with Beckwith-Wiedemann syndrome.

PURPOSE: Obstructive apnea is sometimes seen in patients with Beckwith-Wiedemann syndrome. The cause of apnea is not limited to macroglossia, and the surgical indication for obstructive apnea has not yet been established. The authors performed polysomnography for the assessment of apnea. METHOD: Overnight polysomnograms were obtained in 2 patients who developed obstructive apnea after 1-stage repair for omphalocele. CASE 1: Apnea index (AI), defined as apneic events per hour, indicated 17.3, and SpO2 below 95% occupied 80% of the total sleep time. Computed tomography and magnetic resonance imaging indicated obstruction of the airway between macroglossia and the hypopharynx. Central tongue resection and the division of the frenulum linguae for associated ankyloglossia were performed 97 days after birth. One month after surgery, apneic events disappeared and SpO2 below 95% occupied only 1% of the total sleep time. CASE 2: Obstructive AI indicated 28.1. Division of the frenulum linguae and anterior glossopexy were performed 55 days after birth. Postoperative polysomnogram indicated a marked reduction of AI. CONCLUSIONS: These results indicated that polysomnography was useful for evaluating obstructive apnea and that advancement of the tongue by division of the frenulum linguae may be recommended for the treatment of obstructive apnea in patients with Beckwith-Wiedemann syndrome.

Outcome predictors for infants with cystic lung disease.

BACKGROUND/PURPOSE: This study aimed at identifying characteristic features indicating congenital cystic adenomatoid malformation of the lung (CCAM) and evaluating the outcome predictors to identify prenatally subgroups of fetuses with significantly different probabilities of mortality or severe respiratory difficulty. METHODS: Twenty-eight neonates who had undergone antenatal evaluation for cystic lung disease (CLD) were reviewed retrospectively. The patients were divided into 3 groups according to the severity of their clinical course; mild (n = 7), moderate (n = 13), and severe (n = 8). Ultrasonographic findings in the fetus and their pulmonary lesion were evaluated. The normal lung to thorax transverse area ratio (L/T) was measured by ultrasonography. RESULTS: High echogenicity of the lesion throughout pregnancy and polyhydramnios were frequently seen in CCAM. All of the patients with other CLD showed isoechogenicity at the end of pregnancy. All patients in the severe group had both polyhydramnios and fetal hydrops. L/T was increased in mild and moderate groups, whereas no patient in the severe group had an increase in L/T at the final measurement. Each value of final L/T in the severe group was less than 0.25. CONCLUSIONS: The subgroup of fetuses with an increased probability of mortality or severe respiratory difficulty could be predicted from the combination of polyhydramnios, fetal hydrops, and a final L/T value of less than 0.25.

Fetal mesenchymal hamartoma of the liver: report of a case.

The authors report a case of hepatic mesenchymal hamartoma diagnosed prenatally with ultrasound scan and magnetic resonance imaging (MRI) and confirmed histologically postdelivery. The fetus had a multicystic mass in the left upper abdomen, which showed a rapid enlargement accompanying maternal hypertension and preterm labor. The patient was delivered by cesarian section at 30 weeks and 5 days weighing 1,190 g, and, at birth, a large abdominal mass and severe anemia were noted. Surgical resection and neonatal management were successful, and the patient is alive in a good condition after 3 years follow-up. Although histologically benign, because this lesion frequently results in perinatal complications such as fetal hydrops, maternal toxemia, and preterm labor, early careful fetal ultrasonography should improve the prognosis of this lesion.

Umbilical cord ulcer associated with fetal jejunal atresia: report of 2 cases.

We report 2 cases of umbilical cord ulcer associated with fetal jejunal atresia. Both of them developed a severe intrauterine hemorrhage, followed by fetal heart rate decelerations, and underwent emergency cesarean section. Bloody amniotic fluid and umbilical cord ulcers were observed in both cases. Although both cases were successfully resuscitated, neurological impairment and renal failure developed in 1 case due to prolonged asphyxia. In a review of the literature, umbilical cord ulcer was associated only with congenital duodenal atresia or jejunal atresia, but not with ileal atresia. Although the prenatal diagnosis of duodenal or upper jejunal atresia has been established, the prenatal diagnosis of this complication has not been reported. In such cases, detailed examination of the umbilical cord by ultrasonography may be useful for the prenatal diagnosis of this disease.

Ultrasonographic prediction of clinical pulmonary hypoplasia: measurement of the chest/trunk-length ratio in fetuses.

Pulmonary hypoplasia is involved in patients with various surgical diseases. The aim of this study was to evaluate the clinical usefulness of measurement of the chest/trunk-length ratio (C/T) for predicting pulmonary hypoplasia in patients with congenital anomalies, with the exception of mass-like lesions in the thorax such as diaphragmatic hernia and cystic lung diseases. For measurement of C/T on fetal ultrasound, the sagittal section of the body trunk, including the spine, was analyzed. C/T was calculated as the chest length, defined from the top of the thorax to the top of the diaphragm, divided by the trunk length, defined from the top of the thorax to the bottom of the urinary bladder. From 1986 to 2000, measurements of C/T were undertaken in 49 healthy fetuses from 17 to 37 weeks of gestation and 98 fetuses with congenital anomalies, with the exception of intra-thoracic mass lesions, omphalocele, and fetal hydrops. Pulmonary hypoplasia was clinically assessed by the following criteria: (1) a lung-to-birth-weight ratio of 0.012 or less; (2) patients who required high-frequency oscillatory ventilation with mean airway pressure of 15 cmH(2)O or more with pure oxygen and/or who died presenting respiratory failure without evidences of meconium aspiration, congenital pneumonia, sepsis or hyaline membrane disease. For a predicting value for pulmonary hypoplasia to be obtained, sensitivity, specificity, positive predictive value and negative predictive value were quoted between C/T in patients with pulmonary hypoplasia and those without pulmonary hypoplasia. Healthy fetuses revealed the mean value as 0.38+/-0.03, with no significant change after 20 weeks of gestation. Pulmonary hypoplasia was assessed in 25 fetuses with urethral atresia and stenosis, renal agenesis, polycystic kidney, cloacal anomalies, diaphragmatic eventration, bronchopulmonary foregut malformation, chest deformity, meconium peritonitis and sacrococcygeal teratoma. As a predicting value for pulmonary hypoplasia, 0.32 or less of the maximum value of C/T indicated good accuracy, with a sensitivity of 92.0%, specificity of 95.9%, positive predictive value of 88.5% and negative predictive value of 97.2%. Ultrasonic measurement of C/T is useful in predicting postnatal respiratory conditions with regard to pulmonary hypoplasia.

Case of congenital bronchial atresia detected by fetal ultrasound.

Congenital bronchial atresia is an anomaly characterized by a mucocele associated with a blindly terminating segmental or lobar bronchus. We report on a case of congenital atresia of the left superior segmental bronchus (B6) followed by fetal ultrasonography, which showed a large simple cyst with a surrounding small echogenic area. A large cyst and persistent lung fluid in the affected segment were noted in the early neonatal period.

Lysophosphatidylcholine alters enterocyte monolayer permeability via a protein kinase C/Ca2+ mechanism.

The activity of phospholipase A(2) (PLA(2)) is elevated in the intestinal epithelia of patients with inflammatory bowel disease. We recently reported that PLA(2) mediates the hydrolysis of phosphatidylcholine (PC) to lysophosphatidylcholine (L-PC) when both are applied to the apical surface of cultures enterocyte monolayers, resulting in increased bacterial translocation (BT) and decreased transepithelial electrical resistance (TEER). However, the mechanism by which the converted L-PC affects tight-junction permeability (TJP) as reflected by decreased TEER is unknown. There are some reports that protein kinase C (PKC) or Ca(2+) mediate TJP in enterocyte monolayer models. To investigate whether the observed change in TJP was mediated via PKC or Ca(2+) in our Caco-2 monolayer model, human Caco-2 enterocytes were grown to confluence on porous filters in the apical chamber of a two-chamber cell culture system. The filters were then transferred to an Ussing chamber for precise, real-time resistance measurements. After 30 min equilibration, PC (0.1 or 1 mM) and L-PC (0.01, 0.1 or 1 mM), PMA 200 or 300 nM (phorbol 12-myristate 13-acetate, PCK activator), or staurosporine 12 nM (PKC inhibitor) were added to the apical chamber and TEER was measured every 20 s for 2 h. The concentration of intracellular free Ca(2+) in the monolayers before and after treatment with L-PC (1 mM) was measured by fluorometry of whole monolayers using the fluorescent calcium indicator fura-2. Neither PC at any dose nor the 0.01-mM L-PC dose had an effect on TEER. The 0.1-mM dose of L-PC had its greatest effect (47% +/- 3.5% reduction in TEER vs control) within 6 min following its addition, with TEER recovery to control levels (100%) at 2 h ( P < 0.05). The 1-mM dose of L-PC had its greatest effect (6% +/- 0.5% reduction in TEER vs control) within 3 min after its addition, but the TEER did not recover to control levels after 2 h of incubation ( P < 0.05). The addition of 200 or 300 nM PMA inhibited the observed recovery of TEER by L-PC. Conversely, the addition of 12 nM staurosporine enhanced TEER recovery to control levels. The 1-mM dose of L-PC increased the concentration of intracellular free Ca(2+) immediately after the addition of L-PC. These results suggest that L-PC alters TJP via a PKC/Ca(2+) interaction in our Caco-2 monolayer model.

Usefulness of endoscopic ultrasonography in the diagnosis of congenital esophageal stenosis.

BACKGROUND/PURPOSE: Endoscopic ultrasonography (EUS) is considered a potentially useful tool to investigate structural abnormalities of the esophagus in pediatric patients, as in adults. The aim of this study was to evaluate the usefulness of EUS for the diagnosis of congenital esophageal stenosis. METHODS: High-frequency catheter probe EUS was performed under general anesthesia in 2 patients who had congenital esophageal stenosis. RESULTS: A 4-year-old boy with anorectal anomaly showed tapered narrowing in the distal esophagus, which was not ameliorated with balloon dilatation. High-frequency catheter probe EUS showed hypertrophy of the muscular layer in the esophageal wall at the narrowed portion, but no images suggested the presence of tracheobronchial remnants. The histologic diagnosis of fibromuscular hypertrophy was confirmed at esophagoplasty. A 5-month-old boy with Gross C-type esophageal atresia and symptomatic gastroesophageal reflux showed tapered narrowing in the middle esophagus on esophagography. The symptoms of stenosis were not ameliorated by balloon dilatation performed 4 times. High-frequency catheter probe EUS showed hyperechoic lesions suggesting cartilage at the esophageal narrowing. The diagnosis of tracheobronchial remnants was confirmed by the finding of 2 pieces of cartilage in the specimen obtained at the time of esophageal resection. CONCLUSION: EUS can be applied to show structural abnormalities of the esophageal wall even in pediatric patients with congenital esophageal stenosis and is useful for planning the therapeutic strategy.