Solitary intrapulmonary cystic lymphangioma in an infant: a case report with literature review.

Lymphangioma rarely presents as a solitary pulmonary lesion. We encountered a case of solitary cystic lymphangioma and present its clinicopathologic and immunohistochemical findings. A 2-month-old boy was referred to the hospital after developing a persistent cough. Chest X-ray showed a large cyst in the right lung. Under the preoperative diagnosis of bronchogenic cyst, he underwent right lower lobectomy at the age of 11 months. The resected specimen contained a 5.5-cm septate cystic lesion. Microscopically, the lesion consisted of a large cystic space and interconnected slit-like spaces surrounding bronchovascular islands. The cyst was lined by a monolayer of flat cells with focal multinucleated giant cells. Immunohistochemically, the cells lining the cystic lesion were positive for D2-40, Prox1, CD34, and CD31, and weakly positive for VEGFR-3, but were negative for AE1/3, HMB45, VEGF-A, VFGF-C, VEGFR-1. Differential diagnoses included lobar or interstitial emphysema, bronchogenic cyst, congenital pulmonary airway malformation and alveolar adenoma. D2-40 and Prox1 were useful in differentiation and in determining the extent of the lesion. A review of the literature found only 15 cases of solitary pulmonary lymphangioma. In younger patients, the lesions tend to occupy more of the lung. Focal giant cell reaction has not been described in the reported papers.

Laparoscopic enucleation of a gastroenteric duplication cyst arising in a pancreatic tail that did not communicate with the pancreatic duct: report of a case.

Gastroenteric duplication rarely occurs in locations such as the pancreas. We report a case of gastroenteric duplication of the pancreatic tail, which was noncontiguous with the stomach and had no communication with the pancreatic duct, in a 3-year-old girl. The cyst was enucleated by laparoscopy, without the need for pancreatic resection. The optimal treatment procedures vary considerably, depending on where the gastroenteric duplication is located in the pancreas and, most importantly, whether there is communication with the pancreatic duct.

Comparison of the postoperative bowel function between transanal endorectal pull-through and transabdominal pull-through for Hirschsprung's disease: a study of the feces excretion function using an RI-defecogram.

PURPOSE: Herein, we compared the bowel function after a transabdominal and a transanal procedure for Hirschsprung's disease (HD) using the clinical score and a quantitative evaluation of the feces excretion function based on the findings of an RI-defecogram. MATERIALS AND METHODS: The subjects included 35 patients with short segment aganglionosis. In the two groups with transabdominal Z-shaped anastomosis (open group) and transanal endorectal pull-through (TEPT) (transanal group), the postoperative bowel function were evaluated based on the clinical score. In the RI-defecogram study, a time-activity curve was drawn for the (99m)Tc remaining in the rectum on defecation. The feces excretion function was thus quantified, with the time until the (99m)Tc in the rectum became 50% as T0.5 and the time until 90% of the feces were excreted from the rectum as T0.9. RESULTS: The clinical score could be evaluated in 9 cases in the open group and in 15 cases in the transanal group. No significant difference was observed in the total clinical score between the two groups, but the urge to defecate and the constipation scores in the subcategories were significantly lower in the open group. The defecogram was performed included seven cases in the open group and five cases in the transanal group. When an analysis of covariance of the two groups was conducted for the T0.5 and T0.9 values using the postoperative months as a covariate, there was a significantly negative slope, and moreover, there was a significant difference between the two groups. CONCLUSIONS: The RI-defecogram showed that feces excretion time improves with the postoperative months in both the groups, but the transanal group has higher feces excretion function in the early postoperative period compared with the open group. We consider the RI-defecogram to therefore be a useful examination method for evaluating the feces excretion function.

Patent ductus arteriosus flow patterns in the treatment of congenital diaphragmatic hernia.

BACKGROUND: Congenital diaphragmatic hernia (CDH) mortality still remains high, due to lung hypoplasia and persistent pulmonary hypertension of the neonate (PPHN). Effective management of PPHN and time of operation are quite important to the improvement of CDH treatment. In order to determine the optimal time for operation, we monitored PPHN with cardiac ultrasound. METHODS: PPHN was assessed with three parameters: patent ductus arteriosus flow patterns (PDAFP), %left ventricular diameter at diastole, and left ventricular fraction of shortening (LVFS). Four patients with an antenatal diagnosis were treated under this protocol. Diaphragm repair was performed when PDAFP became left to right shunt dominant and the pre- and postoperative course was analyzed with regular chart reviews. RESULTS: The alveolar-arterial oxygen difference levels of four patients were 590, 335, 613 and 530 mmHg, and operations were carried out when the patients were 2, 2, 3 and 2 days old, respectively. In three of the four patients (all except case 3) the PDAFP changed from right to left shunt dominant or bidirectional (BD), to left to right shunt dominant within 48 h. The %left ventricular diameter at diastole was relatively stable around the time of operation. The LVFS of all patients decreased after the operation. Only the LVFS of case 3 decreased temporarily to less than 30% (which indicates poor left ventricular function) but recovered. No patients needed extracorporeal membrane oxygenation support. All patients survived the procedure and were extubated. Case 3, who took 10 days to become left to right shunt dominant after the operation, needed home oxygenation therapy for 10 months. CONCLUSIONS: PDAFP was a reliable marker of PPHN on a high-frequency oscillatory ventilator to determine the optimal time for the operation for CDH. The optimal time for operation is supposed to be the time when PDAFP become left to right shunt dominant.

Laparoscopic repair of late-presenting Bochdalek hernia in 2 infants.

Laparoscopic repair was performed on 2 infants with late-presenting Bochdalek hernia. Intraoperatively, the entire small intestine was herniated in 1 case and the stomach, small intestine, and part of the colon and spleen were herniated in the other case. Laparoscopic repair of Bochdalek hernia was successfully completed in both the cases. On the basis of our experience, 4 points seem important in laparoscopic surgery for Bochdalek hernia: (1) avoiding damage to the spleen while reducing organs back into the abdominal cavity; (2) ensuring visualization of diaphragmatic defect after reducing the spleen and intestinal tract; (3) ensuring sufficient width to suture the dorsal side of the diaphragm; and (4) identifying intestinal malrotation. We believe that the fourth point represents an advantage of a laparoscopic approach, which seems superior to the thoracoscopic approach and could represent a useful therapy for Bochdalek hernia in infants and older patients.

Is high amplitude propagated contraction present after transanal endorectal pull-through for Hirschsprung's disease?

The transanal endorectal pull-through (TAEPT) procedure is now widely performed for Hirschsprung's disease (HD), however, the colorectal function after TAEPT has not yet been adequately assessed. We evaluated the postoperative clinical outcome and colorectal function based on lower colonic manometry after TAEPT. Twenty-one cases of HD underwent TAEPT from 1998 to 2005. We examined the clinical outcome based on the requirement of enemas/suppositories, the number of defecations per day, the severity of perianal erosion, and the incidence of enterocolitis requiring hospital stay. Using a three-channel Dentsleeve catheter and UPS-2020 measuring device, we performed postoperative colonic manometry at three locations: (1) the anal canal, (2) 5 cm proximal to the anal canal, and (3) 10 cm proximal to the anal canal, during defecation, and then we measured the appearance of high-amplitude contraction (HAC) (duration >/=10 s, amplitude >/=100 cmH(2)O). Four of 21 cases were treated with enemas/suppositories no longer than 1 year. The number of defecations decreased gradually from 4 to 5 per day in the early postoperative period, reaching 2 to 3 per day about 1 year postoperatively. Perianal erosion was noted in 3 of 21 cases but it disappeared within three postoperative years. Two cases had enterocolitis. Lower colon manometry was performed in eight cases. Manometry during defecation was successfully monitored in six cases. HAC occurred in five of six cases (83.3%). The clinical outcomes after TAEPT were satisfactory in almost all cases. These good outcomes were possibly due to the occurrence of HAC during defecation, though HAC did not fill the definition of high amplitude propagated contractions (HAPCs). The occurrence of HAC after TAEPT might be caused by keeping any damage to the mesenteric ascending cholinergic nerve to a minimum during surgery. Further long-term observations are still required to make an adequate assessment of such cases.

Changes in urinary level and configuration ratio of D-lactic acid in patients with short bowel syndrome.

The present study showed that the D-lactic acid configuration ratio in the urine rose earlier than that in blood or the urinary or blood D-lactic acid levels upon disease onset, and that the D-lactic acid measurement in urine is more sensitive and useful than that in blood. As this result, a prediction of a D-lactic acidosis may be possible. To simplify the procedure for detecting D-lactic acid, we first showed a correlation between the D-lactic acid configuration ratio in urine and blood, indicating urine could be used. To separate the optical isomers of lactic acid, we simplified our previous procedure. For chiral recognition, we chose O-acetyl-(-)-menthylation and analyzed the samples under GC/MS by capillary gas chromatography on a DB-5 MS column. This procedure is less sensitive than the former method, but it is faster and simpler, requiring only one derivatization step. This method may be useful for predicting D-lactic acidosis in patients with short bowel syndrome.

Insulin responses to selective arterial calcium infusion under hyperinsulinemic euglycemic glucose clamps: case studies in adult nesidioblastosis and childhood insulinoma.

Selective arterial calcium stimulation and hepatic venous sampling (ASVS) for insulin secretion is used as a diagnostic procedure in patients with insulinomas or adult nesidioblastosis. In some of those patients, severe hypoglycemia requiring urgent glucose administration occurs during the procedure. Such glucose administration, however, may affect the results and damage the validity of the test. We report two cases of hyperinsulinemic hypoglycemia, in which ASVS tests were successfully performed under hyperinsulinemic euglycemic glucose clamps. A 40-year-old male with nesidioblastosis developed continual severe hypoglycemia several years after a Billroth II-Braun gastrectomy, and continuous glucose infusion could not be stopped even during ASVS tests. A 9-year-old girl with an insulinoma that showed atypical hypovascularity on imaging examinations had ASVS tests under a glucose clamp for safety. Hyperinsulinemic (approximately 100 microU/ml) euglycemic (approximately 90 mg/dl) clamps were achieved by an artificial endocrine pancreas. The insulin analogue lispro was utilized for clamps and endogenous insulin was measured with an assay that does not cross-react with the analogue. Diagnostically significant responses (more than twofold) of insulin secretion were observed under hyperinsulinemic clamps in both cases. The use of the hyperinsulinemic glucose clamp technique during the ASVS test should be considered for maintaining the safety of some hypoglycemic patients.

Application of optical isomer analysis by diastereomer derivatization GC/MS to determine the condition of patients with short bowel syndrome.

To establish a method for separating the optical isomers of lactic acid, we modified the derivatization steps in our procedure for urinary mass-screening for inborn errors of metabolism. For chiral recognition, we chose O-trifluoroacetyl-(-)-menthylation derivatization instead of our previous method, trimethylsilyl derivatization, and the samples were then analyzed under GC/MS by capillary gas chromatography on a DB-5MS column. This method can be used to follow-up the condition of a patient with short bowel syndrome and to prevent onset and/or seizure. d-Lactic acid was also isolated from the urine of healthy controls as one of the main peaks in the chromatogram.

Sexual problems in male patients older than 20 years with anorectal malformations.

BACKGROUND/PURPOSE: The nervi erigentes in high- and intermediate-type male anorectal malformation (ARM) runs a relatively medial course and is vulnerable in sacro-perineal dissection. These types of ARM are also associated with a high frequency of sacral anomaly, and sexual problems may be expected. However, sexual function cannot be evaluated until after the individual passes puberty. Few reports have investigated the sexual status of pubescent males with ARM. The present study evaluated sexual problems in patients with high- and intermediate-type ARM. METHODS: Sexual problems such as erectile dysfunction and ejaculatory incompetence were evaluated in 17 of 23 men aged more than 20 years who underwent operation for high- or intermediate-type ARM between September 1974 and January 2005. RESULTS: Erection angle was normal in 9 patients (52.9%), mild in 6 patients (35.3%), and dysfunctional in 2 patients (11.8%). Ejaculatory function was normal in 10 patients (58.8%), with ejaculatory incompetence in 5 patients (29.4%) and retrograde ejaculation in 2 patients (11.8%). Either erectile or ejaculatory dysfunction was present in 5 patients (29.4%), whereas both were present in 2 patients (11.8%). Sexual problems were identified in 7 patients (41.2%), with sacral anomalies in 5 (71.4%) of these 7 patients. CONCLUSIONS: Sexual problems such as erectile and ejaculatory dysfunction are common in patients with high- or intermediate-type ARM. Patients with sexual distress require persistent follow-up and continuous counselling to support their sexual problems.

Usefulness of endoscopic marking for determining the location of transanal endorectal pull-through in the treatment of Hirschsprung's disease.

In the treatment of Hirschsprung's disease, transanal endorectal pull-through (TEPT) is being performed without laparoscopic assistance or laparotomy for short-segment aganglionosis. Preoperative ascertainment of the extent of aganglionosis is required, as this affects the planning for TEPT. The present study investigated the usefulness of preoperative endoscopic marking as an intraoperative marker of the location of pull-through. Since 1998, we have performed TEPT using a prolapsing technique for the treatment of Hirschsprung's disease. Subjects comprised 17 patients with short-segment aganglionosis and 2 patients with long-segment aganglionosis in whom endoscopic marking was performed preoperatively. Median age at time of surgery was 2.7 months. The relationship between pathologic findings at the marked area and location of actual pull-through was investigated. For endoscopic marking, each patient was sedated using intravenous ketamine hydrochloride. The junction between normal bowel with peristalsis and aganglionic bowel without peristalsis ("shorebreak" finding) was marked by either tattooing or clipping. Normal ganglion cells were seen in the marked area of 14 patients, and pull-through was performed at the marked area in each of these patients. In three patients, ganglion cells existed in the marked area, but the number of ganglion cells was considered insufficient. Additional frozen sections were thus prepared to ascertain the area with normal ganglion cells, showing that normal ganglion cells were seen 1, 3 or 5 cm proximal to the marked area. In one patient, no ganglion cells were seen in the marked area, but were present 5 cm proximal to the marked area. In the remaining one patient, normal ganglion cells were seen 7 cm distal to the marked area. Pathologic findings revealed ganglion cells at the shorebreak finding in 17 of the 19 patients (89.5%), suggesting that this junction basically matches the distribution of ganglion cells. Endoscopic marking of the junction is very useful for determining the tip of pull-through.

Large cell calcifying Sertoli cell tumor of the testis: comparative immunohistochemical study with Leydig cell tumor.

Large cell calcifying Sertoli cell tumor is a rare type of testicular tumor. Reported herein is a Japanese patient with this tumor not associated with Carney's complex. An 11-year-old boy was admitted to hospital because of left testicular enlargement, and radical orchiectomy was performed. Macroscopically, the tumor was well circumscribed and had a maximum diameter of approximately 2 cm. The cut surface showed a yellow-white solid mass. Histologically, the tumor was composed of large neoplastic cells with abundant eosinophilic cytoplasm with a tubular, trabecular, and solid arrangement and loose myxoid stroma with irregularly shaped calcification. Immunohistochemically, the tumor cells were positive for vimentin, S-100 protein, calretinin, inhibin-alpha, melan-A, and CD10, and type IV collagen and laminin were observed in the extracellular matrix around the tumor cells. The distributions of melan-A, CD10, and mitochondria were characteristically patchy; in contrast, they were diffusely distributed in the cytoplasm in a control case of Leydig cell tumor. The differences in immunostaining patterns for melan-A, CD10, and mitochondria as well as positivity for S-100 protein-beta might be useful diagnostic hallmarks of large cell calcifying Sertoli cell tumor for discrimination from Leydig cell tumor.

Monitoring method for pre- and post-liver transplantation in patients with primary hyperoxaluria type I.

Differential diagnosis of primary hyperoxaluria type I (PH1) may become difficult once end-stage renal failure and anuria have occurred. Here we describe a rapid and sensitive method to simultaneously quantify glycolate and oxalate in plasma using a stable isotope dilution and gas chromatography-mass spectrometry. The results are provided within 2 h. The linearity of the method was validated up to 200 micromol/l of these compounds and the inter-assay precision for glycolate and oxalate was 2.4 and 2.6%, respectively (n=5), when the control plasma was spiked with 50 micromol/l of glycolate and oxalate. For healthy subjects, 1.0 ml of plasma was required and 0.1 ml for the PH1 patients. Using this method, plasma levels in non-PH1 patients under hemodialysis and in healthy subjects were determined. This method proved to be useful when used for differential diagnosis of PH1 and for monitoring the plasma levels of glycolate and oxalate in two PH1 patients before and after dialysis and liver transplantation. Plasma glycolate in these patients was dramatically decreased after liver transplantation, but plasma oxalate decreased more slowly due to remobilization of the calcium oxalate stores deposited throughout the body.

Maternal cell traffic bounds for immune modulation: tracking maternal H-2 alleles in spleens of baby mice by DNA fingerprinting.

We have previously reported that the immunization of pregnant mice with T-dependent antigens successfully induced suppression of the antigen-specific plaque-forming cell (PFC) response to the relevant antigens in the offspring. This suppression was not caused by the administered antigens, the antibodies produced by the pregnant mother, or lactational transfer, but was dependent on the presence of the intact maternal T cells. It was major histocompatibility complex (MHC)-restricted manner tolerance, which continued for at least one-sixth of the murine life. Traditionally, the placenta acts as a natural barrier, not allowing the cells to pass through. However, the results presented strongly suggested that maternal T cells pass through the placenta and subsequently induce tolerance. In this present study, we attempted to substantiate the presence of maternal cells in the fetal circulation through the use of molecular techniques. We found that a highly polymorphic microsatellite sequence within the class II Eb gene of the H-2 complex is useful for the molecular detection of various H-2 alleles. DNA polymorphic analysis was used for tracking maternal H-2 alleles in the spleens of baby mice. The main procedure involved polymerase chain reaction amplification and restriction fragment length polymorphism analysis of the DNA sequence encompassing the H-2-specific microsatellite from the genomic DNA of baby mice. The results indicated that maternal T cells of immunized pregnant mice cross the placenta into the fetus, eventually inducing antigen-specific immunological tolerance in the offspring.