Neonatal subpial hemorrhage along the medial side of the temporal lobe: Two case reports.

Neonatal subpial hemorrhage has been underrecognized until recently and its pathophysiology remains unclear. Advances in magnetic resonance imaging have facilitated the identification of hemorrhage within the subpial space and cohort studies recently reported its imaging and clinical features. We encountered two cases of neonatal subpial hemorrhage along the medial side of the temporal lobe. Case 1: A 1-day-old boy had repeated apneic attacks with cyanosis from 2 hours after birth at 39 weeks of gestation by vacuum extraction delivery. Computed tomography and magnetic resonance imaging showed subpial hemorrhage from the medial to caudal side of the right temporal lobe with T2 prolongation in the underlying cerebral parenchyma. Case 2: A 0-day-old boy had repeated apneic attacks with cyanosis from 3 hours after birth at 39 weeks of gestation by vaginal delivery. Subpial hemorrhage was observed from the anterior to medial side of the left temporal lobe on computed tomography and magnetic resonance imaging. On magnetic resonance imaging, the adjacent brain parenchyma showed a hyperintense signal on T2-weighted imaging. No abnormalities or signs of fetal distress were noted in the course of delivery. A mildly prolonged activated partial thromboplastin clotting time, an elevated D-dimer level, and low fibrinogen level were detected in a blood examination after birth in both cases. Both cases had subpial hemorrhage along the medial side of the temporal lobe, which suggested that an external mechanical force with fetal head molding during delivery caused subpial hemorrhage; however, other factors, including coagulopathy, may be involved in its pathophysiology.

The comparison of acute toxicities associated with craniospinal irradiation between photon beam therapy and proton beam therapy in children with brain tumors.

INTRODUCTION: This study aimed to evaluate acute toxicities associated with irradiation between the X-CSI (photon beam craniospinal irradiation) and P-CSI (proton beam craniospinal irradiation) groups in children with brain tumors. METHODS: Sixty-two consecutive patients who received initial craniospinal irradiation (CSI) for brain tumors in our center between January 1, 2011 and May 31, 2021, were included in the study. Acute toxicities were retrospectively evaluated during CSI using Common Terminology Criteria for Adverse Events version 5.0. Maximum grades of fatigue, headache, insomnia, nausea, vomiting, dermatitis, constipation, abdominal pain, oropharyngeal mucositis, and hematological toxicities were evaluated. RESULTS: Thirty-six patients received X-CSI, and 26 patients received P-CSI. The median dose of CSI was 18.0 Gy in the X-CSI group and 23.4 Gy (relative biological effectiveness) in the P-CSI group (p < 0.001). The P-CSI group had a lower incidence of more than grade 2 nausea (11.5% vs. 69.4%, p = 0.008) and vomiting (7.7% vs. 38.8%, p < 0.001), compared with the X-CSI group. Multivariate logistic regression analysis with adjustments for potential confounding factors of doses of CSI showed that proton radiation therapy was associated with a marked reduced risk of more than grade 2 nausea and vomiting during CSI (adjusted odds ratio, 0.050; 95% confidential interval, 0.011-0.24; p < 0.001). CONCLUSION: The present study suggests that P-CSI reduces the acute gastrointestinal toxicities associated with irradiation.

A case report of fetus in fetu with an aorta-like structure visualized by contrast-enhanced CT.

Fetus in fetu (FIF) is a rare congenital anomaly resulting from abnormal embyogenesis in monochorionic diamniotic twins and appears as a cystic mass containing fetus-like structures mainly in the retroperitoneum of infants. Although there is a theory that FIF is a highly differentiated teratoma, it is commonly distinguished from teratoma as a mass containing a vertebral axis with appropriate arrangement of limbs or other organs around this axis. Here we present a case of FIF with aorta-like structure visualized by contrast-enhanced computed tomography. A 5-day-old girl was pointed out a cystic mass in the abdomen by ultrasound examination. Abdominal contrast-enhanced computed tomography revealed a retroperitoneal cystic mass with spine- and limb-like bone structures and blood vessel-like elongated structures and it was confirmed as FIF by surgery. The presence of major vascular structures along the skeletal axis is clearly different from teratoma and suggests that it occurred as an embryo and underwent some stage of development. Our findings strongly support the monozygotic twin theory.

A girl with CLOVES syndrome with a recurrent PIK3CA somatic mutation and pancreatic steatosis.

CLOVES syndrome is characterized by congenital lipomatous overgrowth, vascular malformation, epidermal nevi, and scoliosis/spinal malformation. It is caused by somatic mosaicism of gain-of-function variants of PIK3CA. Here, we describe a novel case of a 5-year-old Japanese girl with CLOVES and concurrent pancreatic steatosis. She had a recurrent somatic mutation in PIK3CA (NM_006218.3: c.1357G>A, p.Glu453Lys), elevated HbA1c levels, and pancreatic steatosis. This case indicates that pancreatic screening is critical for PIK3CA-related disorders.

Pazopanib maintenance therapy after tandem high-dose chemotherapy for disseminated Ewing sarcoma.

The dismal prognosis of patients with disseminated Ewing sarcoma necessitates the development of novel treatment strategies. Pazopanib is an oral multi-targeted tyrosine kinase inhibitor that is active against advanced soft tissue sarcoma. However, the clinical activity and feasibility of pazopanib for treating Ewing sarcoma remain poorly understood. Moreover, clinical information on the use of tandem high-dose chemotherapy for Ewing sarcoma is limited. A 14-year-old boy with Ewing sarcoma was transferred to our hospital for treatment. Magnetic resonance imaging, computed tomography scans, and bone scintigraphy revealed multiple lesions in the pubis, ilium, ischium, femur, rib, cranial bone, thoracic vertebrae, sacrum, obturator muscle, adductor magnus muscle, testicular cord, and lungs. Bone scintigraphy after intensive chemotherapies confirmed that multiple abnormal accumulations were still present in the cranial bone and pubis. Subsequently, the patient received tandem high-dose chemotherapy including topotecan, and radiotherapy. Abnormal accumulations have disappeared in bone scintigraphy. Subsequently, pazopanib maintenance therapy was initiated. Despite the presence of innumerable lesions at diagnosis, the patient has been in near-complete remission for the past 1 year with pazopanib administration. This confirms that adding pazopanib maintenance therapy after tandem high-dose chemotherapy is a therapeutic option for cases with disseminated Ewing sarcoma.

Fibrocartilaginous Embolism of the Spinal Cord in Children: A Case Report and Review of Literature.

Fibrocartilaginous embolism is assumed to be caused by fibrocartilaginous nucleus pulposus component migration through retrograde embolization to the spinal cord artery. Fibrocartilaginous embolism is currently not well recognized among pediatricians because of its rarity. We describe a previously healthy 15-year-old soccer player who, after kicking a ball, developed progressive weakness in both legs and urinary retention the next day. Magnetic resonance imaging revealed T2 hyperintensity in the anterior horn of the spinal cord at the Th12/L1 level with Schmorl node at the level of L1/2. We also review the previous literature on fibrocartilaginous embolism of the spinal cord in children (less than18 years age); a total of 25 pediatric patients, including our patient, were identified. The median age was 14 years, and 64% of the reviewed patients were female. The most common trigger event was intense exercise or sports. The neurological symptoms started within one day in most cases, and the time to symptom peak varied from a few hours to two weeks. The most common initial neurological symptoms were weakness or plegia (100%), followed by paresthesia or numbness (48%). Affected areas of the spinal cord were distributed evenly from the cervical to thoracolumbar regions. Although steroids and anticoagulants were most commonly used, the prognosis was quite poor (mild to severe sequelae with three deaths). Although fibrocartilaginous embolism is a very rare condition, physicians should be aware of the characteristics and include fibrocartilaginous embolism of the spinal cord in their differential diagnosis, especially for physically active patients.

Lung Ultrasound in Children with Acute Respiratory Failure: Comparison between Chest X-ray, Chest Computed Tomography, and Lung Ultrasound: A Case Series.

Respiratory failure is one of the most common and critical problems in pediatric wards. Assessments by chest X-rays (CXRs) are common and prevalent for determining the cause of respiratory failure in children. However, CXRs can be misinterpretated. Some patients may require further evaluation with other tools, such as chest computed tomography (CCT). Lung ultrasound (LUS) has proven useful for detecting lung abnormalities with respiratory failure in adults, but its usefulness in children is still not clear. We present a series of eight children who were admitted in a tertiary children's hospital. Each child underwent CXR, CCT, and LUS. In seven of eight cases, both LUS and CCT were able to detect abnormal findings. However, the radiological findings in CXRs were not sufficient. These cases included children with pleural effusion that was comorbid with consolidation, or cases with substantial consolidation that required thoracentesis or proper physical therapy. KEY MESSAGES: LUS can be beneficial for evaluating children with respiratory failure that are admitted in pediatric intensive care unit (PICU) and may contribute towards appropriate therapy for children. HOW TO CITE THIS ARTICLE: Fukuhara S, Yamaguchi Y et al. Lung Ultrasound in Children with Acute Respiratory Failure: Comparison between Chest X-ray, Chest Computed Tomography, and Lung Ultrasound: A Case Series. Indian J of Crit Care Med 2019;23(2):95-98.

Dilatation of the common channel in pediatric congenital biliary dilatation remaining after radical operation.

BACKGROUND: In congenital biliary dilatation, the protein plug is likely impacted, especially in a dilated common channel. However, nobody has discussed whether this dilatation of common channel remains after radical operation. The aim of this study was to investigate the situation of the dilated common channel after radical operation. METHODS: The status of the common channel was investigated at radical operation and follow-up. We then evaluated whether dilation of the common channel remained postoperatively. Moreover, the association between status of the common channel and postoperative pancreatic complication was evaluated. RESULTS: Postoperative condition of the common channel was able to be evaluated ultrasonographically in 42 patients. The common channel was dilated at the time of operation in 18 patients and remained dilated postoperatively in 11 of these 18 patients. Dilatation of the common channel was not identified in any patients without dilatation at the time of operation. No patients in this series showed any postoperative pancreatic complications, even if a dilated common channel remained. CONCLUSIONS: Dilatation of the common channel can remain postoperatively in congenital biliary dilatation, but does not appear to be associated with postoperative pancreatic complications.

A new approach to risk stratification using fetal MRI to predict outcomes in congenital diaphragmatic hernia: the preliminary retrospective single institutional study.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a condition with a wide range of severity. Prenatal diagnosis is essential to optimize postnatal management, especially for severe cases. The lung to head ratio (LHR) and liver herniation estimated by prenatal ultrasound has been used as prenatal predictors in CDH. However, reliability of these factors remains to be proven and prediction of outcome from prenatal imaging is still challenging. We propose our new stratification system using lung to liver signal intensity ratio (LLSIR) in fetal MRI, which has been shown to be related to pulmonary maturation. METHODS: Retrospective chart review was conducted on 25 infants with CDH treated from 2009 through 2016 in our hospital. We stratified patients according to fetal T2-weighted MRI as Grade I, detectable ipsilateral lung at the apex; Grade II, undetectable ipsilateral lung at the apex and contralateral LLSR ≥2.0; Grade III, undetectable ipsilateral lung at the apex and contralateral LLSR <2.0. To evaluate this stratification system, we analyzed survival, severity [inhaled nitric oxide (iNO) usage with or without extracorporeal membrane oxygenation (ECMO)], and requirement of patch closure. RESULTS: All 15 patients survived in Grade I, while 2 out of 6 died in Grade II, and 3 out of 4 died in Grade III (P=0.003). Four were severe in Grade I, and all in Grade II and III who survived (P=0.007). One needed patch in Grade I, and all in Grade II and III (OR: 414,238,332; 95% CI, 0-∞). Liver herniation was noted in five patients, and significantly associated with survival (P=0.04), however, neither with severity (P=1.00) nor with the requirement of patch closure (P=0.52). CONCLUSIONS: The risk stratification algorithm using contralateral LLSIR in fetal MRI could be useful and more reliable than liver herniation to predict survival, severity, and need of patch closure. Further investigation is warranted.

A case of biliary atresia with pancreaticobiliary maljunction.

BACKGROUND: The pathogenesis of biliary atresia (BA) is still unknown. There are several reports on the etiology of BA, including pancreaticobiliary maljunction (PBM). We experienced a case of Kasai type IIIa BA with PBM, in which we found elevation of pancreatic enzymes in the gallbladder. We evaluated whether PBM is related to the pathogenesis of BA based on our findings. CASE PRESENTATION: The patient was born at 40 weeks of gestation. His body weight at birth was 2850 g. At the age of 4 days, he had an acholic stool and was referred to our hospital. Abdominal ultrasonography showed that triangular cord sign was negative. The gallbladder was isolated with a diameter of 19 mm, and it contracted in response to oral feeding. His ultrasonographic findings were atypical for BA, but his jaundice did not improve. Therefore, we performed an operation at the age of 56 days. Intraoperative cholangiography showed a common bile duct and pancreatic duct and a common channel patent, while the common hepatic duct or intrahepatic duct was not visualized. Bile in the gallbladder contained colorless fluid, which showed elevated lipase level (34,100 IU/L). We performed Kasai portoenterostomy under the diagnosis of Kasai type IIIa BA with PBM. The patient's postoperative course was uneventful, and he was discharged on day 30 after the operation. Histopathological evaluation showed that the lumens of the common bile duct and cystic duct were patent. However, the common hepatic duct was closed, and only bile ductules with diameters of less than 50 µm were isolated. Infiltration of lymphocytes was detected in the porta hepatis. No apparent inflammation was observed around the cystic duct, which was constantly exposed to pancreatic juice because of reflux through PBM. CONCLUSIONS: Reflux of pancreatic juice through PBM might not be an etiological factor for BA, but might be associated with patency of the common and cystic bile ducts in Kasai type IIIa BA.

Bone marrow magnetic resonance imaging of the clivus in pediatric leukemia patients and normal controls.

PURPOSE: An MRI-based pre-test to determine the probability of pediatric leukemia prior to bone marrow aspiration would be useful to prevent unnecessary exposure to this invasive test. We aimed to evaluate the clivus-to-pons signal intensity ratio (CPR) and visual scoring (VS) on T1-weighted images (T1WI) and diffusion-weighted images (DWI) to distinguish pediatric leukemia patients from normal controls. MATERIALS AND METHODS: We retrospectively reviewed 1.5-T brain MR images of 13 consecutive leukemia patients (3 girls, 10 boys; mean age, 8.23 years; range, 1-17 years) and 40 age- and gender-matched normal controls. We evaluated differences between leukemia patients and normal controls using Wilcoxon rank-sum and Mann-Whitney U tests with respect to the following parameters: (1) CPR on T1WI (CPRT1WI); (2) CPR on DWI (CPRDWI); (3) VS on T1WI (VST1WI); and (4) VS on DWI (VSDWI). RESULTS: The CPRT1WI values for leukemia patients and normal controls were 0.77 ± 0.12 and 1.39 ± 0.47, respectively (P < 0.001). The corresponding CPRDWI values were 1.03 ± 0.38 and 0.50 ± 0.17, respectively (P < 0.001). VST1WI and VSDWI were significantly different between the groups (P < 0.001 for both). CONCLUSION: MRI-based quantitative and qualitative analyses of clival bone marrow on T1WI and DWI can distinguish pediatric leukemia patients from normal subjects.

[A case of complete response to multiple liver metastasis of gastric cancer after discontinuation of S-1 administration].

An 80-year-old man was diagnosed with advanced gastric cancer and underwent distal gastrectomy. Although the pathological Stage of the cancer was III A, he refused adjuvant chemotherapy. One year later, CT revealed multiple liver metastases. Therefore, he was started with S-1 administration and a complete response was obtained at 10 months after starting S-1 administration. He has maintained a complete response for 22 months after S-1 discontinuation.

Can biliary atresia be diagnosed by ultrasonography alone?

BACKGROUND/PURPOSE: The triangular cord sign (TCS) is a specific ultrasonographic finding, reflecting a fibrotic mass at the porta hepatis in biliary atresia (BA). We evaluated whether BA can be diagnosed by ultrasonography alone using 3 findings: TCS, gallbladder length (GBL), and gallbladder contractility (GBC). METHODS: Subjects comprised 85 infants (median age, 47 days; range, 4-144 days) with cholestatic jaundice who underwent ultrasonographic examination for diagnosis between May 1996 and June 2006. Medical records were reviewed with regard to TCS, GBL, and GBC. Positive findings for BA included TCS >or=3 mm, GBL <15 mm, and GBC <68% (for < 12 weeks) or <25% (for >or=12 weeks). RESULTS: Biliary atresia was diagnosed in 48 patients surgically, with other cholestatic diseases diagnosed in the remaining 37 patients. Triangular cord sign was positive in 41 of 48 infants with BA and negative in 35 of 37 infants without BA. The 7 patients with BA who displayed negative results for TCS displayed positive findings for GBL or GBC. Positive predictive value in the diagnosis of BA was 98% if positive TCS was combined with abnormal gallbladder findings, whereas negative predictive value in diagnosis of BA was 100% if negative TCS was combined with findings of a normal gallbladder. CONCLUSIONS: Biliary atresia can be accurately diagnosed by ultrasonography using the findings of TCS combined with GBL and GBC.

Development of acquired tracheal stenosis in premature infants due to prolonged endotracheal ventilation: etiological considerations and surgical management.

The aim of this study was to determine the etiology and appropriate surgical treatment for acquired tracheal stenosis that developed in patients who had undergone prolonged endotracheal mechanical ventilation as premature neonates. During the period 2000-2004, four patients aged 1-16 years were referred for tracheal stenosis characterized by stridor, choking, and recurrent pulmonary infection. All patients had undergone endotracheal mechanical ventilation for 2-5 months for respiratory distress related to prematurity (gestational age 25-29 weeks, birth weight 648-1,222 g). During this period, methicillin-resistant Staphylococcus aureus was predominantly cultured from the trachea. All patients exhibited a stenotic lesion encompassing 30-37% of the entire tracheal length on spiral CT. On palpation and inspection of the trachea during surgery, however, the stenotic segment appeared to encompass over 50% of the entire trachea. The carina was intact. Three patients underwent slide tracheoplasty with a tracheal resection and the other underwent resection and end-to-end anastomosis. Of the three patients treated by slide tracheoplasty, two are currently free of respiratory symptoms. However, one patient in this group required secondary resection of the remaining stenotic lesion with end-to-end anastomosis. This patient is currently asymptomatic. The remaining patient who underwent a resection and end-to-end anastomosis is doing well. The resected specimen showed fibrosis and degeneration of tracheal cartilage. A combination of prematurity, prolonged endotracheal mechanical ventilation and tracheal infection seem to be responsible for tracheal inflammation and stenosis. When considering surgical procedures for acquired tracheal stenosis, resection and end-to-end anastomosis are desirable. However, slide tracheoplasty with a partial tracheal resection is indicated for the treatment of stenosis involving a long tracheal segment.

Bleeding duodenal varices successfully treated with balloon-occluded retrograde transvenous obliteration (B-RTO) assisted by CT during arterial portography.

A 60-year-old woman with massive hemorrhage from duodenal varices was transferred to our hospital for the purpose of transcatheter intervention. Although digital subtraction arterial portography could not depict the entire pathway of collateral circulation, the efferent route of the duodenal varices was clearly demonstrated on subsequent CT during arterial portography. Balloon-occluded retrograde transvenous obliteration (B-RTO) of the varices was performed via the efferent vein and achieved complete thrombosis of the varices.

[Clinical evaluation of chemotherapy with irinotecan (CPT-11), l-leucovorin (l-LV), 5-fluorouracil (5-FU), and UFT for metastatic or recurrent colorectal cancer].

A clinical study has been conducted since August 2001 to investigate whether chemotherapy with CPT-11/l-LV/ 5-FU/UFT could be an effective regimen for advanced or recurrent colorectal cancer. The chemotherapy consisted of CPT-11 30 mg/m2 iv, as a 120-minute infusion, followed by l-LV 30 mg/m2, as a 60-minute infusion, followed by 5-FU 300 mg/m2, as a 120-minute infusion. This treatment was administered weekly for 6 weeks followed by a 2-week rest period and repeated every 8 weeks. UFT (250 mg/m2/day) was orally administered daily. All patients were evaluable for efficacy, 2 CR, 5 PR, 3 SD and 7 PD. The overall response rate was 41.1% with a median time to progression of 7.1 months and a median survival time of 12.0 months. No grade 3/4 toxicities were observed. The present study suggests that combination chemotherapy with CPT-11, l-LV, 5-FU, and UFT is well tolerated and might be a promising regimen for advanced or recurrent colorectal cancer.

Communicating bronchopulmonary foregut malformation: particular emphasis on concomitant congenital tracheobronchial stenosis.

A total of four patients with communicating bronchopulmonary foregut malformation were treated surgically at Kobe Children's Hospital between 1993 and 2004. Of these, three patients displayed congenital tracheobronchial stenosis and developed life-threatening respiratory distress soon after birth. In each case, anomalous bronchi arose from the lower portion of the esophagus and connected to the lower part of the ipsilateral lung. This anomaly involved the right lung in two patients, and the left lung in one patient. Tracheobronchial stenosis extended from the inlet of the thorax to the carina in one patient, and to the contralateral main stem bronchus in two patients. Surgical treatment included division of the esophageal bronchus and anastomosis of bronchus to the trachea in one patient. In the other patient, the ipsilateral lung was resected and the stenotic tracheobronchus was stented. The remaining patient underwent pneumonectomy of the ipsilateral lung. Details of this fatal anomaly and a discussion of appropriate surgical management are described herein.