Acute appendicitis is a common disease that causes acute abdomen in children. When acute appendicitis is diagnosed, the standard treatment is excision of the appendix by surgery, but interval appendectomy (IA) may be selected for complicated appendicitis. In mild cases, it has been reported that spontaneously resolving appendicitis (SRA) can relieve symptoms without surgery or antibiotics.

At the Tokyo Metropolitan Children’s Medical Center, pediatric surgeons perform the ultrasound examination, make a definite diagnosis of acute appendicitis and classify the original severity grading, and decide on the treatment policy. If the structure of the appendix wall is maintained or if the blood flow of the wall is increased even if it is irregular, we administer an intravenous bolus infusion only with follow-up.

If the wall structure is irregular and the blood flow is reduced, or the wall structure is lost, a semi-emergency operation or IA is adopted. Appendicecal mass is preceded by antibiotic treatment, and IA is performed 3 months after the abscess has disappeared.

It was considered that the evaluation of the form of the appendix and the evaluation of blood flow by ultrasound are useful not only for diagnosis of acute appendicitis but also for determining the treatment policy.

Thoracoscopic pulmonary lobectomy (TPL) is widely used as the treatment of choice for congenital cystic lung diseases, such as congenital pulmonary airway malformation (CPAM) and intralobar pulmonary sequestration (ILPS). However, TPL in children is technically challenging, because the thoracic cavity is small, and cystic lesions may further obstruct the operative field. Thus, thorough understanding of anatomic relations preoperatively is vital for successful outcome.

Here, we introduce our preoperative imaging protocol that uses thin-slice computed tomography (CT) for investigating CPAM/ILPS cases, by introducing a case of prenatally diagnosed left-lower lobe CPAM who was asymptomatic after delivery and had elective TPL when 2 years old. Preoperative CT identified a 35 mm cystic lesion in the left-lower lobe, and a rare anomalous A6 pulmonary artery that divided into two branches. Because of the CT study, the second branch of A6 could be identified safely at surgery. An intraoperative video highlighting the value of our protocol is presented.

Thin-slice contrast CT is mandatory for assessing the anatomic relations of cystic lesions in relation to pulmonary vasculature, the bronchial tree, as well as for confirming the status of fissures between lobes, to ensure TPL is performed safely and effectively.

Indocyanine green fluorescence method can be used for detecting primary or metastatic lesions of hepatoblastoma and hepatocellular carcinoma intraoperatively. Using this method for lung metastatic surgery, extremely tiny lesions can be visualized and extirpated easily. For primary liver surgery, tiny residual tumors on cut surface of the liver can be visualized and extirpated. Users should be reminded that false positive rate is relatively high. Whether this method can improve the prognosis of these diseases is still unclear, but it can certainly reduce the operation time because of avoiding intraoperative histopathological confirmation. This navigation can permit partial resection of the lung or the liver safely instead of lobectomy or segmentectomy, confirming no residual tiny tumors on cut surface.

Precise understanding of each patient’s anatomy is crucial in hepatobiliary and pancreatic surgery, especially in liver resection. We have long been using simulation software SYNAPCE VINCENT^® to calculate graft volume and remnant liver volume in living donor hepatectomy. More recently, we introduced preoperative three-dimensional fusion imaging, which allows excellent visualization of the complex conformation of the hilar structures and major hepatic veins and their tributaries. We also applied this technique for hepatic tumors to understand the relation between the intrahepatic vasculature and lesion. Herein, we demonstrate our technique in living donor hepatectomy and liver tumor resection in our department.

Though the Nuss procedure for Pectus Excavatum is widely performed as minimal invasive surgery, some fatal cases have been reported due to cardiac or vascular injury during the mediastinum dissection. To avoid these complications, we started a new method using a lifting hook, Maryland dissector for laparoscopy. The surgical method 1. Two 5 mm ports of the thoracoscope are inserted. 2. The surgical view of anterior mediastinum is made by artificial pneumothorax. 3. The sternum is lifted with a lifting hook. 4. The dissection of the anterior mediastinum is started with the Maryland dissector. The layer of the avascular area can be easily ascertained by pneumomediastinum and the contralateral thoracic cavity can be seen.

We evaluated the operation time and complications in patients under 15 years of age who underwent the Nuss procedure, categorized according to the operation method into Group A (new method), Group B (the introducer only without the lifting hook). The anterior dissection times were 111.3, and 220.5 seconds (p < 0.05), for Group A (n = 21), Group B (n = 34), respectively. The complication rate were 4.8% and 8%, respectively. Anterior dissection of our method shortens the time of operation and reduces the occurrence of complications.

[Background] Fibromatosis colli is an infantile neck mass resulting in torticollis. Previous studies have reported numerous image findings linked to fibromatosis colli and that they may depend on the patient’s age at examination.

[Purpose] We aimed to reveal the diversity of ultrasonogram (US) and magnetic resonance image (MRI) findings by assessing the patient’s age (in days) at examination.

[Material and Methods] We enrolled 13 infantile (6 male; 7 female) patients clinically diagnosed with fibromatosis colli in this retrospective study. US was performed in 12 cases (one examination in eight cases, two examinations in one case, three examinations in one case, and four examinations in two cases) and MRI was performed in five cases (one examination in three cases and two examinations in two cases). Using US, we evaluated the maximum diameter in the long axis and echogenicity of the sternocleidomastoid, discontinuity of the muscular bundle, and presence/absence of hypoechoic lesion in the sternocleidomastoid. Using MRI, we evaluated the diameter of the sternocleidomastoid, patchy or diffuse hyperintensity areas on T2-weighted images (T2WI), and swollen sternal or clavicle head of the sternocleidomastoid. We assessed the imaging findings’ time course in four and two cases using several US and MRI examinations, respectively.

[Result] We observed hyperechoic sternocleidomastoids on US in all cases (12/12). Discontinuity of the muscular bundle was seen in 4/12 cases; hypoechoic lesion in the sternocleidomastoid was seen in 8/12 cases. In two/five and three/five cases, we observed patchy and diffuse hyperintensity on T2WI, respectively. The sternal head was swollen in four/five cases and the clavicle head was swollen in one/five case. No correlation was observed between the diameter of the sternocleidomastoid and patient age on US examination (R = 0.111). Echogenicity of the sternocleidomastoid was normalized in two/four cases following several US examinations. In one/one and two/four cases, disappearance of discontinuity of the muscular bundle and hypoechoic lesions were observed, respectively.

[Conclusion] Our study shows various findings on US and MRI during the clinical course.

Cecal volvulus is very rare in pediatric patients but is often reported among those with multiple, severe disabilities, especially Cornelia de Lange syndrome. Early diagnosis prevents intestinal necrosis and is therefore paramount. Delayed diagnosis and treatment may lead to increased mortality in these patients.

In our department, 13 patients with Cornelia de Lange syndrome are currently being followed. Of these patients, four had cecal volvulus and presented either typical findings or dramatic changes on plain abdominal radiographs. Subsequent, preoperative contrast-enhanced CT suggested cecal volvulus in all the patients. A comparison of patients with and without cecal volvulus showed that all patients with cecal volvulus had a history of fundoplication, which is suggested to be a contributing factor for volvulus. Compared with past reports, the mortality and complication rates were lower in the present cases. Whenever children with Cornelia de Lange syndrome and a history of a fundoplication present intestinal symptoms, cecal volvulus should be suspected, and plain abdominal radiography followed by prompt contrast-enhanced CT should be performed for early diagnosis and treatment.

Trichobezoars are formed by ingested hair that coagulates in the stomach and sometimes causes intestinal obstructions. We report a pediatric case of gastrointestinal obstructions as a result of trichobezoars, which were successfully removed by laparotomy. An 11-year-old girl was referred to our hospital with acute abdominal pain and vomiting. An abdominal CT showed that the small intestine was dilated. At first, we suspected a non-strangulation of the ileum by an internal hernia. An emergency laparotomy was performed with a vertical midline incision. A trichobezoar was discovered in the ileum and removed. On postoperative day 6, an upper gastrointestinal contrast X-ray was performed and showed a second trichobezoar in her stomach. We attempted to remove the mass using an endoscopic procedure, but this proved difficult. A decision was made to undertake another laparotomy. The trichobezoar was found near the pylorus and removed. Her postoperative progress was satisfactory, with no recurrence. We need to consider that the gastrointestinal trichobezoar as a differential diagnosis of intestinal obstruction, if the patient has no history of surgery. In addition, we should try to better understand the imaging characteristics of trichobezoars and recognize the possibility of multiple trichobezoars before performing surgery.

We herein report a case of infectious spondylitis mimicking a urinary tract infection which was successfully diagnosed by imaging studies. A 14-year-old boy visited a local clinic due to symptoms of fever and back pain and was found to have white blood cells in his urine. Antibiotic therapy was thus started. However, the symptoms persisted. As a result, he was referred to our hospital with a fever 5 days after the initial presentation. We carried out an ultrasounds examination, and an iso-hypoechoic area was observed in his right kidney. Next, we performed contrast computed tomography to examine the renal abscess, and signs of inflammation were observed around the L5/S1 region. Magnetic resonance imaging showed a high signal on the right side of the L5 vertebral body and surrounding tissue. We therefore diagnosed the patient with spondylitis based on the imaging findings. After six weeks of appropriate antibiotic therapy, the patient demonstrated a complete recovery. Although infectious spondylitis is a relatively rare disease in children, imaging studies should nevertheless be carefully utilized in cases of unknown lower back pain accompanied by a fever.

The patient was an 11-year-old girl. At the age of 8 years, a chest radiograph showed a tumor shadow. CT revealed a 44 × 32-mm-sized tumor extending from the anterior mediastinum’s right side to the right-side rim of the heart. The boundary with the surrounding tissue was clear, calcification was not observed, and no clear contrast effect was observed. On MRI, there was intermediate signal in the T1-weighted image (WI) and mixed low-to-high signal in the T2-WI. No obvious fat component was observed. Angiography revealed blood flowing from the right internal thoracic artery to the mass and multiple outflowing blood vessels to the superior vena cava. There was no significant increase in 18F-FDG accumulation on PET. Based on these findings, the tumor was deemed asymptomatic, and a follow-up policy was selected, considering extralobar sequestration first. However, CT examination at 11 years of age showed that the tumor had increased in size, at 67 × 49 mm, and superior vena caval exclusion was observed, while MRI showed a cystic structure exhibiting high signal on T1- and T2-WI inside. Surgery for extrapulmonary lung fractionation and excision was decided on. The operation involved median sternotomy and anterior mediastinal tumor extirpation (thymic complication excision). The pathological diagnosis was thymic angiolipoma, without malignant findings.

Pial arteriovenous fistula is a rare condition in which a pial artery connects directly to a pial vein in the brain. It accounts for 1.6–4.8% of all brain vascular malformations. Pial arteriovenous fistula is considered to be associated with a poor prognosis without treatment; thus, early detection and treatment are important.

We report a case of pial arteriovenous fistula which was caused by a seizure.

An inner ear malformation that causes congenital deafness, can be associated with cerebrospinal fluid leakage, which is a well-known major cause of recurrent meningitis in childhood. We encountered a case of pneumococcal meningoencephalitis in a 5-year-old girl. She had been diagnosed with hearing loss and inner ear malformation by temporal bone computed tomography (CT), when she was 2 years old. She presented with consciousness disturbance and hemiplegia. Brain CT and diffusion magnetic resonance imaging (MRI) revealed marked brain edema and a high-intensity signal in the left frontoparietal operculum, respectively. Since the earlier temporal bone CT scan had revealed a cerebrospinal fistula, she was considered to be at risk of recurrent meningitis; thus, she underwent inner ear reconstruction surgery. Since the surgical intervention, she has been free from meningitis. It is important to check for concomitant cerebrospinal fistulae and evaluate the risk of meningitis in the management of patients with inner ear malformations.

Neurocutaneous melanosis (NCM) is a rare congenital disease consisting of development of congenital giant melanocytic nevi and CNS melanocytosis. We present two neonates who had giant congenital nevi and multiple smaller satellite melanocytic nevi on the scalp and extremities, with brain MRI findings characteristic of NCM. In both neonates, MRI demonstrated multiple brain parenchymal foci of T1 hyperintensity and T2 hypointensity, signal characteristics of melanin with the T1, and T2 shortening effects. These foci were seen in the amygdalae, brain stem, and cerebellum. In one neonate, dilatation of the ventricular system and subarachnoid space was identified, representing communicating hydrocephalus. These intracranial findings are characteristic of NCM. No obvious leptomeningeal contrast enhancement was demonstrated. MRI can provide useful information for the diagnosis and follow up of NCM.