A rare case of neonatal colonic obstruction caused by a solitary intestinal tumor.

BACKGROUND: Intestinal obstruction caused by a tumor is very rare in newborns, and the preoperative diagnosis is difficult. We herein report a rare case of neonatal colonic obstruction due to solitary intestinal myofibroma with characteristic findings on gastrografin enema and the surgical strategy. CASE PRESENTATION: A 4-day-old female infant presented to our neonatal intensive-care unit with abdominal distention and bilious vomiting after feeding. A gastrografin enema showed that the transverse colon near the hepatic flexure was not delineated at the oral side. When pressure was applied, a small amount of contrast material moved into the mouth in the form of threads. Microcolon was not observed, and stenosis of the transverse colon was found 9 cm from the Bauhin valve. Partial resection and end-to-end anastomosis were performed. A pathological examination of the resected specimen suggested gastrointestinal stromal tumor (GIST). After obtaining a second opinion, the histology and immunohistological markers were deemed characteristic of infantile myofibroma. CONCLUSION: If string sign and a napkin ring appearance are found in a case of neonatal intestinal obstruction, surgery should be performed with a tumor in mind. In cases of neonatal intestinal obstruction caused by a tumor, the lesion should be resected with a sufficient surgical margin before the pathological examination.

A Case of a Two-Month-Old Boy Diagnosed with Infantile Crohn's Disease Based on an Atypical Perianal Lesion.

Inflammatory bowel disease is rare in infants, and the early diagnosis is very important. We herein report an infant who received an early diagnosis of infantile Crohn's disease (CD). A two-month-old boy presented with bloody stool. He developed a poor sucking tendency and a painful perianal lesion at three months of age. He was suspected of having infantile CD because of his atypical perianal lesion. Colonoscopy revealed that his perianal lesion had induced rectal longitudinal ulcers. Histology showed no granulomas but patchy inflammation reaching the submucosal layer. He was diagnosed with infantile CD based on the Japanese criteria. CD should be suspected in infants with atypical perianal lesions, irrespective of their age. Early colonoscopy with histology should be considered in these cases in order to prevent adverse outcomes in children.

Ultrasound-guided ilioinguinal/iliohypogastric block did not reduce emergence delirium after ambulatory pediatric inguinal hernia repair: a prospective randomized double-blind study.

PURPOSE: Emergence delirium (ED) is a common postoperative complication of ambulatory pediatric surgery done under general anesthesia with sevoflurane. However, perioperative analgesic techniques have been shown to reduce sevoflurane-induced ED. The primary objective of this investigation was to examine whether an ultrasound-guided ilioinguinal/iliohypogastric (II/IH) nerve block for ambulatory pediatric inguinal hernia repair could reduce the incidence of sevoflurane-induced ED. METHODS: The subjects of this prospective randomized double-blind study were 40 boys ranging in age from 1 to 6 years, who were scheduled to undergo ambulatory inguinal hernia repair. The patients were randomized to either receive or not to receive an ultrasound-guided II/IH nerve block (Group B and Group NB, respectively). General anesthesia was maintained with sevoflurane and nitrous oxide. The primary outcome assessed was ED, evaluated using the Pediatric Anesthesia Emergence Delirium (PAED) scale 30 min after emergence from general anesthesia. The secondary outcomes assessed were postoperative pain, evaluated using the Behavioral Observational Pain Scale (BOPS), and the amount of intra-operative sevoflurane given. RESULTS: The median PAED scale scores did not differ between Groups B and NB at 30 min (P = 0.41). BOPS scores also did not differ significantly between the groups, but the mean amount of intraoperative sevoflurane given was significantly lower in Group B than in Group NB (P < 0.01). CONCLUSIONS: Ultrasound-guided II/IH nerve block for ambulatory pediatric inguinal hernia repair did not reduce ED, but it did decrease the amount of intra-operative sevoflurane needed. CLINICAL TRIAL REGISTRATION: UMIN000008586.

Contrast-enhanced multidetector-row computed tomography can predict pathological findings of acute appendicitis in children.

Aims: To retrospectively evaluate the correlation between multidetector-row computed tomography findings of acute appendicitis and the pathological status of acute appendicitis, and evaluate the capability of multidetector-row computed tomography to predict the pathological status of acute appendicitis in children. Methods: The presence of a distended appendix (>6 mm in transverse diameter) was used as a primary sign to indicate the presence of appendiceal inflammation. The presence of appendiceal wall thickening (>1 mm) and enhanced appendiceal wall continuity were also used as predictive findings to reflect the degree of progression of acute appendicitis on multidetector-row computed tomography findings. The findings of each individual case were classified into four grades. The final pathological diagnosis was classified into four groups: normal findings, only mucosal inflammation, inflammation with intramural spreading, and gangrenous. The relationship between the pathological grades and computed tomography grades was analyzed using Spearman's rank correlation test. Results: Four of six cases in Grade 0 reflected normal appendiceal findings (66.7%) and 3 of 5 cases in Grade I reflected only mucosal inflammation status (60.0%). Forty-four of 51 cases in Grade II reflected intramural inflammation status (86.3%), and 40 of 57 cases in Grade III reflected gangrenous status (70.2%). The multidetector-row computed tomography grade was significantly correlated to the pathological grade with Spearman's rank correlation coefficient of 0.689 (P < 0.001). Conclusions: There was a close relationship between the multidetector-row computed tomography imaging findings and the pathological findings. This preoperative information is extremely useful for decision-making in the treatment strategy for acute appendicitis in children.

Near-infrared fluorescence cholangiography with indocyanine green for biliary atresia. Real-time imaging during the Kasai procedure: a pilot study.

INTRODUCTION: Hepatoportoenterostomy (HPE) with the Kasai procedure is the treatment of choice for biliary atresia (BA) as the initial surgery. However, the appropriate level of dissection level of the fibrous cone (FC) of the porta hepatis (PH) is frequently unclear, and the procedure sometimes results in unsuccessful outcomes. Recently, indocyanine green near-infrared fluorescence imaging (ICG-FCG) has been developed as a form of real-time cholangiography. METHODS: We applied this technique in five patients with BA to visualize the biliary flow at the PH intraoperatively. ICG was injected intravenously the day before surgery as the liver function test, and the liver was observed with a near-infrared camera system during the operation while the patient's feces was also observed. RESULTS: In all patients, the whole liver fluoresced diffusely with ICG-containing stagnant bile, whereas no extrahepatic structures fluoresced. The findings of the ICG fluorescence pattern of the PH after dissection of the FC were classified into three types: spotty fluorescence, one patient; diffuse weak fluorescence, three patients; and diffuse strong fluorescence, one patient. In all five patients, the feces evacuated after HPE showed distinct fluorescent spots, although that obtained before surgery showed no fluorescence. One patient with diffuse strong fluorescence who did not achieve JF underwent living related liver transplantation six months after the initial HPE procedure. Four patients, including three cases involving diffuse weak fluorescence and one case involving spotty fluorescence showed weak fluorescence compared to that of the surrounding liver surface. CONCLUSION: We were able to detect the presence of bile excretion at the time of HPE intraoperatively and successfully evaluated the extent of bile excretion using this new technique. Furthermore, the ICG-FCG findings may provide information leading to a new classification and potentially function as an indicator predicting the clinical outcomes after HPE.

Successful balloon dilatation for postoperative caval stenosis caused by primary venorrhaphy for traumatic retro-hepatic caval injury in a three-year-old child: Report of a case.

Inferior vena cava injuries are highly lethal. We experienced a case of retrohepatic inferior vena cava injury as a result of blunt trauma in a three-year-old female. Because the site of bleeding of the IVC was identified, we repaired it with running sutures. An attempt at primary repair resulted in postoperative narrowing of the vena cava. There was pressure gradient of the right atrium and inferior vena cava, and collateral circulation developed. Since it was also found that the haemodynamics was unstable, the child underwent another intervention before the stenosis of the IVC was fixed. To the best of our knowledge, there have been no previous reports of therapeutic radiological intervention for stenosis that developed after treatment of a traumatic IVC injury. The IVC in the present case recovered enough patency so that the collateral venous flow could be decreased after balloon dilatation angioplasty.

Intestinal neuronal dysplasia-like histopathology in infancy.

The present patient was delivered at a gestational age of 27 weeks. She had abdominal bloating with symptoms of respiratory distress. We suspected Hirschsprung disease (HD) or small intestinal stricture, but examinations were not definitive. Exploratory laparotomy was performed at 97 days of age. Intraoperative findings showed no evidence of small intestinal stricture or changes in intestinal caliber. A transanal drainage tube was inserted, and decompression therapy and intestinal lavage were started. Rectal mucosal biopsy was performed at 184 days of age, and HE and acetylcholinesterase staining showed intestinal neuronal dysplasia (IND)-like pathological findings. At 15 months, giant ganglia were no longer present on follow-up rectal mucosal biopsy, and the pathological diagnosis was normoganglionosis. It should be recognized that while the enteric nervous system is developing, IND-like pathological findings may be seen as a pathological condition during the maturation process.

Intraoperative near-infrared indocyanine green fluorescence angiography (NIR-ICG AG) can predict delayed small bowel stricture after ischemic intestinal injury: report of a case.

We herein report the case of a 15-year-old male who developed delayed intestinal stricture after undergoing massive intestinal resection due to severe small intestinal volvulus. At the time of the initial surgery, the laparotomy findings showed a massive intestinal volvulus without malrotation. Most of the small intestine appeared to be necrotic; therefore, massive necrotic intestinal resection was performed. The residual intestine comprised only the proximal jejunum and short ileum, including the ileocecal valve and entire colon. After the resection, the serosal surface color of the distal part of the residual jejunum (DPRJ) initially showed a slightly darker hue than normal. However, the color improved with time, and the other clinical findings also improved, which were considered to indicate that the perfusion of the DPRJ was preserved. The perfusion of that area was therefore clinically expected to improve with time. On the other hand, repeated intraoperative near-infrared indocyanine green fluorescence angiography (NIR-ICG AG) consistently showed abnormal vascular flow patterns in the same region, which were suspected to indicate the presence of perfusion damage of the DPRJ, in spite of improvements in the clinical findings. Although the necessity of additional resection was discussed at the time of reconstruction, we finally estimated that the perfusion of the DPRJ was preserved, mainly based on the improvement of the clinical findings of the intestine. The primary anastomosis was performed without additional resection, to maximize the lengths of the residual intestine. However, after the initial surgery, the patient developed a delayed partial stricture of the residual intestine, and an additional resection was necessary on the 22nd postoperative day. The stricture segment corresponded to the area that presented abnormal findings by NIR-ICG AG. This case suggests that abnormal NIR-ICG AG findings may predict delayed intestinal ischemic complications. We believe that NIR-ICG AG can intraoperatively provide more useful real time information for the assessment of intestinal perfusion, than conventional clinical assessment methods.

Abdominal actinomycosis masquerading as an omental tumor in a 12-year-old female.

We herein report a case of abdominal actinomycosis in a 12-year-old girl in whom an omental primary tumor was suspected before surgery. The patient began to experience intermittent lower left abdominal pain. Abdominal computed tomography (CT) scans were inconclusive at this time, but 6 months later, CT and magnetic resonance imaging (MRI) examinations showed a 7-cm, tumor-like lesion in the left abdominal cavity; malignancy could not be ruled out. The tumor, which originated in the omentum and adhered strongly to the left abdominal wall, was resected along with approximately 90 % of the omentum, the peritoneum in contact with the mass, and the posterior layer of the rectus abdominal sheath, under suspicion of a malignant tumor. However, omental actinomycosis was the final pathological diagnosis. The patient's antibiotic treatment was changed to a penicillin-series oral antibiotic to prevent recurrence of the actinomycosis. The patient was discharged from our hospital 16 days after the first surgery, but she developed three episodes of ileus; the first two required surgery. The patient has had no further recurrences of actinomycosis or postoperative ileus 20 months after discharge.

Kikuchi-Fujimoto disease following vaccination against human papilloma virus infection and Japanese encephalitis.

Kikuchi-Fujimoto disease (KFD) is a benign and self-limited syndrome, characterized by regional lymphadenopathy and fever. Although the pathogenesis of KFD remains unclear, infectious agents, autoimmune causes, and physicochemical factors have been suggested as triggers. However, KFD following vaccination has never been reported. We present a 14-year-old girl who suffered from fever and cervical lymph node swelling following simultaneous administration of human papilloma virus vaccine and Japanese encephalitis virus vaccine. The patient was diagnosed with KFD based on the histopathologic findings of a lymph node biopsy, and her fever and swelling resolved with oral corticosteroid therapy. Although the exact pathogenesis of the development of KFD following immunization remains unknown, this should be added to the list of potential triggers or factors associated with the development of KFD.

Massive gastric bleeding from a blunt abdominal trauma: report of a case.

Blunt gastric injury (BGI) is a rare condition that accounts for 0.02%-1.7% of all blunt abdominal trauma cases. Blunt gastric rupture, which occurs in less than 40% of all BGI cases, presents unstable vital signs and symptoms of peritonitis due to massive peritoneal contamination. This article presents the case of a patient with BGI who did not present with symptoms of peritonitis in spite of presenting with persistent hypovolemic shock.

Invasive cholangiocarcinoma identified in congenital biliary dilatation in a 3-year-old boy.

We herein report a 3-year-old male demonstrating invasive cholangiocarcinoma (CC) associated with congenital biliary dilatation (CBD). A 3-year-old Japanese boy was admitted to our hospital with abdominal pain and vomiting. Computed tomography and magnetic resonance imaging demonstrated a dilated extrahepatic bile duct. A diagnosis of Todani's type 1a CBD was made. Intraoperative cholangiography demonstrated the presence of pancreaticobiliary maljunction but could not reveal any tumor lesion in the bile duct. The excision of extrahepatic bile duct and gallbladder and Roux-en-Y hepaticojejunostomy were performed. On gross inspection, we could not find any tumor lesion in the resected specimen. However, the postoperative histopathologic examinations confirmed the presence of well-differentiated tubular adenocarcinoma with lymphovascular invasion. Most of the carcinoma remained within the mucosal layer, and the carcinoma was identified at both the distal and proximal surgical margins of the bile duct. We scheduled additional surgery to eradicate the residual carcinoma, but informed consent for the extra surgery could not be obtained. A close follow-up with abdominal computed tomography has been going on without either additional surgery or adjuvant chemotherapy about for 1 year. The patient has so far been clinically doing well without any obvious symptoms of recurrent disease. To our knowledge, this report is the youngest case of CC associated with CBD.

Rapidly progressive fatal pneumococcal sepsis in adults: a report of two cases.

We report two cases of a rapidly progressive fatal overwhelming pneumococcal infection. Patient 1 was a 67-year-old man with a 24-h history of fever and malaise and was transferred to our department. He was severely ill, tachypneic, and felt a chill. A purpuric discoloration with ecchymosis of the skin was noted over the body. The chest X-ray findings demonstrated thickening of the bronchovascular bundle in the right lower lung field, which later revealed the presence of bronchopneumonia. Laboratory studies revealed the presence of metabolic acidosis and disseminated intravascular coagulation. After presentation, rapid deterioration occurred followed by cardiopulmonary arrest. Despite cardiopulmonary resuscitation, the patient died only 3 h after presentation. The isolates from the patient's blood revealed penicillin-susceptible Streptococcus pneumoniae, serotype 4. Patient 2 was a 30-year-old woman with a prior history of uneventful pregnancies was transferred to our department with a 2-day history of fever, nausea, headache, and malaise. Although she was in the 19th week of pregnancy at the time, she suffered a miscarriage just prior to admission. Upon presentation to our department, she demonstrated unstable vital signs, diminished consciousness, anuria, and icterus. Purpuric discoloration with ecchymosis of the skin was noted in over most of her body, including the distal extremities. The chest X-ray findings were close to normal. Initial laboratory studies revealed the presence of severe metabolic acidosis and disseminated intravascular coagulation with multiple organ failure. Despite aggressive cardiopulmonary support, normal neurological responses disappeared on the 2nd day following admission and the patient died on the 16th day after admission. The patient's isolates from blood and vaginal swabs both later revealed penicillin-susceptible Streptococcus pneumoniae, serotype 12F. The presentation of rapidly progressive septic shock should raise the treating physician's suspicion of overwhelming pneumococcal infection, which has limited management options.

A case of a traumatic abdominal wall hernia that could not be identified until exploratory laparoscopy was performed.

Traumatic abdominal wall hernia (TAWH) is a rare condition secondary to blunt abdominal trauma in children. We herein report an 8-year-old boy who developed TAWH after falling onto a bicycle handlebar. Neither physical examination nor radiological findings suggested a diagnosis of TAWH at first presentation. TAWH in the right lower quadrant could not be identified until exploratory laparoscopy was performed. An open surgical repair was done, and the postoperative course was uneventful. The literature on pediatric TAWH is briefly reviewed and the findings discussed.

Effects of the herbal medicine Inchinko-to on liver function in postoperative patients with biliary atresia--a pilot study.

BACKGROUND/PURPOSE: A continuation of liver fibrosis after undergoing successful Kasai operation has become the important clinical issue in the long-term follow-up of patients with biliary atresia (BA). The aim of this study is to evaluate the efficacy of the herbal medicine Inchinko-to (TJ-135) on the treatment of liver fibrosis in patients with BA without jaundice, especially from the viewpoint of the long-term effects of TJ-135. METHODS: Six postoperative patients with BA ranging between 3 and 13 years of age with normal serum total bilirubin levels (total bilirubin < 1.0 mg/dL [17 micromol/L]) received TJ-135 from 2 to 4 years. The liver enzyme (glutamic oxaloacetic transaminase [GOT], glutamic pyruvic transaminase [GPT], gamma glutamyl transpeptidase[gamma-GTP]transpeptidase[gamma-GTP] levels and hyaluronic acid (HA) levels were compared before and after the administration of TJ-135. The monthly collected data were averaged on a 1-year basis. The record of one postoperative patient with BA and a normal serum total bilirubin level was incorporated as a control. This patient showed portal hypertension and did not receive TJ-135. RESULTS: Five of the six patients who showed abnormal values for liver enzymes, exhibited a significant decrease in serum GOT, gamma-GTP, or GPT levels after a 1 to 3-year administration of TJ-135, and the improvement in these parameters persisted thereafter. Furthermore, one patient who had an abnormally high value of HA also showed a significant decrease in the serum level of HA. In the remaining patient with normal liver enzyme values, no significant change was observed during the administration of TJ-135. The control patient exhibited a chronological decrease in the serum GOT and GPT levels by 5 years of age, but the serum gamma-GTP and HA levels remained stable throughout the postoperative period. CONCLUSIONS: The long-term effectiveness of TJ-135 was only found in those patients with abnormal liver enzyme levels and HA, thereby suggesting that TJ-135 has a protective and antifibrotic effect on the liver.

Whole-blood manganese levels and brain manganese accumulation in children receiving long-term home parenteral nutrition.

Recent reports attribute neurological and cerebral disorders to the accumulation of manganese (Mn) in the brain in patients receiving home parenteral nutrition (HPN). It is desirable to control the amount of Mn delivered to these patients, but a suitable method for monitoring an individual's Mn status and assessing Mn accumulation remains debatable. The aim of this study was to evaluate whether whole-blood manganese levels (WB-Mn) correlate with the accumulation of Mn in the brains of children who receive long-term HPN, using magnetic resonance imaging (MRI) of the brain. Six patients who had received HPN (duration of HPN, 18-137 months) were included in this study. The daily parenteral doses of Mn were calculated while on HPN. WB-Mn was measured and T1-weighted MRI of the brain was obtained for each patient with a 1.5-T MR imager. Twelve months after the withdrawal of Mn from HPN, measurements of WB-Mn and brain MRI were repeated in all patients except for one who was lost after initial examination. The same examinations were performed on an additional patient who had been successfully weaned off a 179 month course of HPN 20 months prior to the initial examination. The parenteral dose of Mn while receiving HPN ranged from 15.7 to 91.5 micro g/kg/day. Initially, MRI showed hyperintensity in the globus pallidus in all patients and in the anterior pituitary in one patient. WB-Mn was elevated in four patients, but was in the normal range in the remaining three. Following subsequent measurements 12 months later, WB-Mn was normal in all patients and MRI hyperintensity remained in the globus pallidus in one patient. One patient was lost after the initial examinations. WB-Mn does not necessarily correlate with the accumulation of Mn in the brain. Periodic MRI should be performed in patients receiving long-term NPN to monitor for excessive Mn accumulation in the brain.