Laparoscopic extirpation of splenic hamartoma

We presented a case of splenic hamartoma. A 12-year-old girl has had an abdominal pain since 9 years of age. Contrast-enhanced computed tomography scan at a local hospital revealed heterogeneous enhancement of a mass which is 5 cm in diameter, located near the surface of the spleen, diagnosed as hemangioma. She had an investigation with Levovist-enhanced ultrasonography and superparamagnetic iron oxide-enhanced magnetic resonance imaging on T2-weighted gradient-echo imaging, which showed a hypervascular echoic mass and a decrease of signal intensity. The imaging diagnosis was splenic hamartoma. She underwent laparoscopic splenectomy to prevent the spontaneous rupture. The splenic artery at the pancreas body was exposed, and doubly ligated by vessel clip and furthermore ligated by 3-0 silk. At the splenic hilum, the splenic artery and vein were dissected with the Endo GIA vascular linear cutting stapler. The spleen was captured into the Endocatch II retrieval bag and removed from a transverse lower abdominal (suprapubic) incision extended up 7 cm in length through a port site. In pathological findings, the tumor was compatible with hamartoma. Splenic hamartomas in pediatric patients have been described extremely rare. In clinical symptoms, spontaneously ruptured splenic hamartoma has been rarely reported.     

Radiological features of child maltreatment

Child maltreatment is serious, highly prevalent and topical. Approximately 1% of children are neglected or psychologically maltreated and 4%–16% are physically abused each year.     

Radiological features in generalized lipodystrophy

Seven patients with congenital generalized lipodystrophy and one patient with acquired generalized lipodystrophy have been followed up for 38 years. Various radiological techniques have been used, and the major findings are summarized. All patients had an advanced skeletal age. A sclerotic skeleton was a major feature in childhood in the congenital form, later turning into osteolytic lesions or more distinct sclerotic patches in adolescence or early adulthood. In the acquired form the skeleton was slightly osteoporotic without cystic changes. Ultrasonography and computerized tomography examinations revealed hepatosplenomegaly with fatty infiltration in both types. Lack of subcutaneous and intrabdominal fat was easily confirmed with computerized tomography. Five patients with the congenital form underwent pneumoencephalography, which revealed dilated brain ventricles and basal cisterns. Cardiomegaly and large kidneys were also a major feature.     

Radiological features in Trisomy 8

Radiological study may prompt the correct diagnosis of Trisomy 8 mosaicism when the clinical features are mild and could be overlooked. Skeletal features characteristic of Trisomy 8 are found in the skull, elbows, spine, pelvis, hands and feet.     

Radiological features of non-accidental skeletal injury

Imaging plays a significant role in confirming the extent of non-accidental skeletal injury (NASI) and excluding any predisposing cause. Most NASI occurs in the pre-mobile child, where a clear history of injury is not always evident. In the older child the history is crucial to differentiating accidental from inflicted injury.When one injury is found, a skeletal survey is usually performed that may reveal other bony injuries or an underlying bone disorder. Whether the presenting injury is skeletal or not, a skeletal survey is important to detect occult injury in an infant presenting with symptoms of non-accidental injury. This should be performed to the agreed standard and checked by a radiologist for adequacy. The local radiologist should offer an opinion and refer onwards for a specialist opinion if they lack experience in paediatric trauma imaging. Supplemental or follow-up radiographs may confirm a diagnosis of non-accidental injury and assist in dating fractures.Clear communication by the radiologist is important for management, and forensic assessment re-timing, anticipated mechanism and symptoms of injury for the fractures identified. This involves multidisciplinary clinco-radiological correlation.Common presentation of NASI includes a fracture that is either unlikely from the child’s developmental level, or an explanation for an injury that alters between seeing the general practitioner, Accident and Emergency department and paediatrician. A careful family history and clinical examination are needed to identify any predisposing disorder, supplemented by imaging and appropriate laboratory investigations.Multidisciplinary working is essential to enable paediatricians, radiologists, child protection services and the police to assist the court to determine the true cause of injury.     

Neuromuscular hamartoma: imaging features of a rare paediatric craniofacial tumour

Neuromuscular hamartoma (also referred to as neuromuscular choristoma or benign triton tumour) has not previously been described in the radiological literature. It is a rare benign lesion composed of mature elements of striated muscle and neural tissue. We report a case of neuromuscular hamartoma involving the skull base, nasopharynx, orbit and maxilla in a 2.5-year-old child who presented with facial swelling. The CT and MRI appearances of this unusual soft-tissue tumour are emphasized, together with a discussion of the pathological findings, differential diagnosis and review of the literature.     

Symptomatic splenic hamartoma with renal, cutaneous, and hematological abnormalities

Background. There is a rare association between splenic hamartomas and hematological abnormalities with, to our knowledge, only 24 reported cases in the English literature.¶Patients and methods. We report a case of a splenic hamartoma in a 14-year-old boy associated with membranoproliferative glomerulonephritis, multiple lobular capillary hemangiomas of the skin, hypertension, and anemia. Following imaging with ultrasonography, MRI, and nuclear scans, a hamartoma was suspected, but malignancy could not be excluded. The lesion was removed by partial splenectomy, and pathological examination confirmed the presence of a red pulp splenic hamartoma.¶Results. The renal, hematological, and dermatological abnormalities resolved following removal of the splenic hamartoma. This is the first reported case of a splenic hamartoma associated with renal, cutaneous, and hematological abnormalities and only the second reported case of a symptomatic splenic hamartoma treated by partial splenectomy.     

产前诊断胆道闭锁影像学研究

目的：胆道闭锁早期诊断困难,而产前诊断更是极少发现。本文对产前诊断的胆道闭锁影像学特点进行探讨。方法回顾我院2010年至2012年收治的产前诊断9例产前诊断胆道畸形患儿,入院手术年龄24 d至2岁。全部患儿行腹腔镜胆道造影,4例诊断胆道闭锁,5例诊断先天性胆管扩张症,胆道闭锁患儿中2例接受腹腔镜下肝管空肠ROUX-Y吻合术,2例接受开腹肝门空肠吻合术。观察其临床表现,超声和实验室指标,术中情况,术后恢复情况等。结果4例患儿产前超声未见胆囊或胆囊显示不清。产前超声发现肝门部囊肿的3例患儿,囊肿小且均无明显增大,张力较高,呈规则圆形。产前诊断发现肝门囊肿的3例患儿术中证实为胆总管远端闭锁（Ⅰ型胆道闭锁）,未发现胆囊也未发现囊肿的1例患儿证实为Ⅲ型胆道闭锁。生后全部胆道闭锁患儿出现黄疸,最早出现在生后第2天,但都未出现陶土样便。全部胆道闭锁患儿囊肿大小形态无明显变化。全部患儿查ALT、AST、rGGT、直接胆红素和总胆红素进行性升高。2例接受腹腔镜下肝管空肠ROUX-Y吻合术,2例接受开腹肝门空肠吻合术。全部4例患儿术后恢复好。结论产前超声检查可以确定胆道闭锁,如果发现肝门部囊肿的胎儿,应定期接受超声检查,如果囊肿在孕期变化不明显,应怀疑囊肿型胆道闭锁。如产前超声未发现胆囊结构,则应怀疑为Ⅲ型胆道闭锁。生后应密切观察、超声、生化、黄疸情况。如果黄疸进行性加重可及早进行腹腔镜胆道造影及手术治疗。     

儿童下丘脑错构瘤的临床及影像学表现

目的：为了提高对下丘脑错构瘤影像学和临床表现的认识,该文主要研究下丘脑错构瘤的临床特点、CT及MRI影像学表现。方法：回顾性分析了38例下丘脑错构瘤的CT及MRI影像资料。其中男25例、女13例,发病年龄1个月至15岁。38例均进行了MR扫描,10例又进行了CT扫描。38例全部经手术、病理证实。结果：主要临床表现为痴笑样癫癎、性早熟、其他类型癫癎（包括癫癎大发作及肢体抽搐等）和智力障碍。其中首发症状为痴笑样癫癎17例,性早熟13例,其他类型癫癎8例。影像学表现为脚间池占位,CT为等密度,MRI为等或稍长T1等或稍长T2信号影,增强扫描病变无强化。临床表现与病灶的大小以及是否有蒂无明显关系。结论：儿童出现性早熟和/或痴笑样癫癎,影像表现为脚间池占位,CT等密度,MRI等或稍长T1等或稍长T2信号影,增强扫描病变无强化,此时应考虑到下丘脑错构瘤。［中国当代儿科杂志,2009,11（5）：364-366］     

Iloe-ileocolic intussusception: Radiological features and reducibility

This paper concerns ileo-ileocolic intussusception rather than the commoner ileocolic intussusception. diagnostic criteria for ileo-ileocolic intussusceptions are established by retrospective study of radiological findings in 28 surgically proven cases. Using these criteria, 11 successfully reduced cases were found in the corresponding period (28.2%). All were apparently cured after one reduction: the evidence suggests that a small but significant number of ileoileocolic intussusceptions may be safely reduced by normal methods during barium enema.     

Radiological features in a case of Münchausen syndrome by proxy

Munchausen syndrome by proxy is a type of child abuse which is usually diagnosed on clinical grounds. In occasional instances, radiological diagnosis plays a role in the recognition of this disorder. We present the radiological findings in a child with an unusual osteomyelitis owing to Münchausen syndrome by proxy.     

Radiological features of ingested metallic mesh earphone pieces

Foreign body ingestion remains a frequent cause of morbidity in infants and children. Symptoms vary with size, shape, location and composition of the foreign body. Flat objects tend to rotate to the greatest diameter of the esophageal lumen and become oriented in the coronal plane. We report two cases of ingested metallic mesh earphone pieces found in the upper esophagus. The foreign bodies were difficult to identify on posteroanterior images, but were more easily visualized on the lateral images. The presentation of this clinical entity and the recognition of its distinctive radiological findings may prevent a delayed diagnosis in the future and avoid potential complications.     

Radiological features of late-onset lymphoedema in Noonan's syndrome

Noonan's syndrome is a multiple congenital anomaly syndrome with diverse manifestations. Lymphatic abnormalities occur in less than 20% of patients. We report a 14-year-old boy who presented with swollen lower limbs and dysmorphic features characteristic of Noonan's syndrome. The radiological features of this unusual case of late-onset lymphoedema in association with Noonan's syndrome are presented.     

Nephrogenic diabetes insipidus. Radiological and clinical features

Two brothers with nephrogenic diabetes insipidus are presented. The importance of early recognition and treatment to reverse the consequential complications are underscored. The disorder has a sex-linked recessive transmission, where all male offspring of an affected mother show the extreme thirst characteristic of the disease; in whom hyposthenuria persisted despite arginine vasopressin infusion, and in whom the serum arginine vasopressin concentrations correlate with the serum osmolality which serves to discriminate nephrogenic diabetes insipidus from the pituitary diabetes insipidus. The radiological features of "non-obstructive" hydronephrosis and hydroureters, the combined use of hydrochlorothiazide and the potassium-sparing diuretic amiloride in comparison to the prostaglandin inhibitor are discussed.     

Radiological features of fungal typhlitis complicating acute lymphoblastic leukaemia

We report the radiological features of fungal typhlitis, a complication of acute lymphoblastic leukaemia, in a child. Colonic dilatation, intramural gas and a tumour-like filling defect in the caecum were demonstrated on ultrasound and contrast enema. Received: 8 January 1996 Accepted: 15 January 1996     

Radiological features of focal nodular hyperplasia of the liver in children

Background. Focal nodular hyperplasia (FNH) is an unusual hepatic tumour in children and should be distinguished from other hepatic lesions. Objective. To describe the imaging characteristics of FNH in children. Materials and methods. We examined five patients (three boys and two girls, mean age 9.4 years) with pathologically confirmed FNH. The diagnosis was obtained by tumour resection (n = 4) and percutaneous needle biopsy (n = 1). One patient with multiple FNHs showed recurrent lesions after tumour resection. All patients were studied with US (including colour and power Doppler US [n = 3]) and CT. Dynamic enhanced CT scans were available in three patients. MRI (n = 2) or coeliac angiography (n = 1) was performed in three patients. Results. Seven of eight FNH lesions in five patients were demonstrated by imaging. The average size of the lesions was 6.5 cm. Six lesions detected on US showed variable echogenicity with a central hyperechoic scar (n = 2). On Doppler examination, central or peripheral hypervascular areas were seen (n = 3). Six lesions detected on contrast-enhanced CT showed high attenuation (n = 4) or iso-attenuation (n = 2). On early phase scans, all the lesions (n = 3) showed high attenuation. Irregular linear or ovoid central scars were detected in two patients on CT. MR demonstrated three lesions in two patients, one of which had not been detected by US or CT. A central low signal intensity scar (n = 1) was seen on T2-weighted MRI. Coeliac angiography performed in one patient showed a hypervascular mass with homogeneous staining. Conclusion. FNH in children shows a wide spectrum of imaging findings on various radiological examinations and the typical central scar was not always seen on imaging studies. Dynamic enhanced CT obtained in the early phase and colour Doppler studies may be helpful in the diagnosis of FNH by allowing characterisation of tumour vascularity. FNH should be included in the differential diagnosis of liver mass in children. Received: 10 September 1997 Accepted: 17 April 1998     

Radiological features in congenital erythropoietic porphyria (Gunther's disease)

Gunther's disease or congenital erythropoïetic porphyria is a rare and severe disorder comprising cutaneous and haemolytic symptoms. Photocutaneous lesions are responsible for scleroderma-like calcifications and deformities of the extremities visible on X-rays. Hemolytic manifestations lead to diffuse major osteopenia. Soft tissue calcifications of the fingers can be seen even in young patients. One case reported here is the first illustration of intracranial calcifications located on dura-mater and calvarium.