Cholelithiasis in children with CHD: is it a problem?

BACKGROUND: An association of heart disease and its treatment with biliary calculi is popularly accepted. We sought determine the prevalence and risk factors of paediatric gallstone disease in the presence of CHD and analyse the treatment options. We evaluated the role of open-heart surgery in the development of gallstones in patients with CHD. Patients and methods In a 10-year, retrospective, chart review (2005-2014), patients with CHD and cholelithiasis were identified and reviewed. RESULTS: In all, 19 of 4729 children with CHD had cholelithiasis (0.4%); eight patients underwent cardiac surgery before diagnosis of cholelithiasis (group 1), whereas 11 of them had not (group 2). The prevalence was 0.3% in group 1 and 0.5% in group 2. In nine asymptomatic patients, gallstones were found incidentally. Children with cholecystolithiasis (n=17) received ursodeoxycholic acid. A resolution of gallstones was found in four cases; two patients underwent biliary surgery, and the others (15/17) were successfully managed non-operatively. CONCLUSION: Despite an accumulation of risk factors, prevalence of gallstones is not as high as expected in children with CHD. Open-heart surgery with a heart-lung machine plays a minor role as an aetiological factor. In about half of the cases, cholelithiasis is an incidental finding and patients stay asymptomatic. Prophylactic administration of ursodeoxycholic acid is not indicated in children undergoing open-heart surgery for CHDs. Biliary surgery is reserved for patients with recurrent symptoms or cholestasis. In children with CHD, cholelithiasis is a minor and manageable co-morbid condition.

Volvulus without malposition--a single-center experience.

BACKGROUND: This is a single-center case series about the rare condition of volvulus without malposition and/or malrotation (VWM) in preterm babies. We focus on diagnostic difficulties, and our results should help to distinguish VWM as a distinct entity different from classical volvulus and segmental volvulus. MATERIALS AND METHODS: Medical chart review of infants with VWM from 2003-2012 was used. RESULTS: A total of 15 patients were identified. All of them had volvulus in the absence of intestinal malposition or other associated intestinal pathologies. All patients were born prematurely. Emergency laparotomy was necessary in all 15 patients. Two groups were identified. Group 1 includes four patients with typical signs of meconium obstruction of prematurity (MOP). Small bowel resection was only necessary in one of these four patients, all survived without residual intestinal lesions. Group 2 consists of 11 patients without signs of MOP-small bowel resection and temporary enterostomy were necessary in all these children. Four patients presented with pneumatosis intestinalis on the abdominal plain film, suggesting necrotizing enterocolitis. Although two infants died, the survivors showed complete recovery. CONCLUSIONS: VWM is a distinct disease of prematurity. When associated with MOP, VWM has a favorable outcome of treatment. In contrast, VWM occurring in the absence of signs of meconium obstruction requires small bowel resection. VWM primarily affects the top of the midgut (ileum). Because of absent malposition, presentation of VWM may be uncharacteristic. Pneumatosis intestinalis in advanced VWM may lead to diagnostic difficulties and a delay in treatment.

Sinus sternoclavicularis: a congenital cervical sinus.

We try to characterize a previously rather neglected congenital cervical sinus located in the sternoclavicular area in five children. This sinus showed extension to the left sternoclavicular joint in all patients, so we call this congenital lesion "sinus sternoclavicularis." With knowledge of this congenital lesion, diagnosis can easily be established based on case history and clinical examination; no further radiological tests are required. Surgical excision is the treatment of choice. The chance of recurrence seems to be high because of misinterpretation of the lesion.

Status epilepticus, cardiac resuscitation, and posterior reversible encephalopathy syndrome after ingestion of viscous lidocaine: a plea for more childproof packaging of pharmaceuticals.

Ingestion of viscous lidocaine in children can lead to potentially lethal neurologic and cardiac effects. We report the case of a 2-year-old boy who developed posterior reversible encephalopathy syndrome 2 days after unobserved ingestion of about 500 mg viscous lidocaine (40 mg/kg of bodyweight). Initially, the child presented with convulsive status epilepticus and subsequent cardiac arrest necessitating cardiopulmonary resuscitation for eight minutes. After 2 days of full recovery, the child presented with progressive disorientation, dizziness, and visual neglect. Lasting for 2 days, these symptoms finally disappeared completely. Combined with the findings on cerebral magnetic resonance imaging, this episode was interpreted as posterior reversible encephalopathy syndrome. Two weeks after the ingestion, no neurologic and visual abnormalities were found. Viscous lidocaine is prescribed routinely for dentition or other painful lesions in the oral cavity in children. Despite the potential hazardousness of the drug, packaging of viscous lidocaine is not childproof. Therefore, physicians have to instruct the parents carefully to minimize the risk of overuse or accidental ingestion. In general, the use of viscous lidocaine should be limited.

Recurrent pancreatitis due to an intraluminal duodenal diverticulum: report of a case.

We report the investigation and treatment of a 14-year-old girl in whom a detailed assessment of recurrent episodes of pancreatitis revealed a large intraluminal 'windsock'-like duodenal diverticulum. As the diverticulum was closely attached to the papilla of Vater, it was resected by a transduodenal approach. This report focuses on the significance of rare congenital anomalies of the duodenum (e.g., duplication cyst, intraluminal diverticulum) during the diagnostic workup of recurrent pancreatitis in children.

Perinatal neuroblastoma of the pancreas.

Neuroblastoma is the most common solid tumor in infancy. Arising from the neural crest these tumors are usually located along the sympathetic chain from the neck to the pelvis and in the adrenal medulla. We report the case of a 3-week-old boy presenting with recurrent episodes of colicky pain. After ultrasound examination, magnetic resonance imaging and laboratory data a pancreatic neuroblastoma was suspected. Tumor resection via distal pancreatectomy and histologic investigation confirmed the diagnosis. Surgery is the treatment of choice in children with pancreatic masses, and is usually well tolerated even in the neonatal period. Conclusive diagnosis can be frequently established only by tumor resection or biopsy. Pancreatic neuroblastoma is an extremely rare tumor with only a few cases described in literature.

Ileostomy Complications in Infants less than 1500 grams - Frequent but Manageable.

BACKGROUND: In very low birth weight infants abdominal emergency surgery may result in ileostomy formation. We observed a frequent stoma complications in these patients. This retrospective analysis put light on ileostomy-related problems and complications in very low birth weight (VLBW) infants. MATERIALS AND METHODS: In a seven-year retrospective chart review (2008 - 2014) infants with ileostomy formation weighing less than 1500 grams at time of operation were identified and reviewed. Data analysis included demographic data, complications and short term outcomes. RESULTS: Thirty patients were included. Ileostomy was formed for spontaneous intestinal perforation (SIP) (n=17), meconium obstruction of prematurity (MOP) (n=6), midgut volvulus (MV) (n=5), necrotizing enterocolitis (NEC) (n=1) and Hirschsprung's disease (HD) (n=1). Three patients died before ileostomy reversal was considered. In seven patients planned ileostomy reversal was done. Twenty infants had stoma related complications (stoma prolapse, prestomal obstruction, stoma retraction, high output stoma, peristomal skin excoriation, and stomal ischemia). Complications did not correlate with underlying diseases. Stomal complications necessitated earlier stoma reversal (mean 62 days). Postoperative complications after stoma reversal occurred in three children (wound dehiscence, adhesion ileus, anastomotic stricture). CONCLUSIONS: Although ileostomy related complications are frequent in very low birth weight infants, mortality is low. Morbidity is manageable.

Spontaneous localized intestinal perforation in very-low-birth-weight infants: a distinct clinical entity different from necrotizing enterocolitis.

BACKGROUND: Idiopathic spontaneous intestinal perforation (SIP), a distinct clinical entity different from necrotizing enterocolitis (NEC), has an increasing prevalence in very-low-birth-weight infants. The aims of our study were to define patient characteristics and potential risk factors for premature infants with SIP compared with infants subjected to surgical treatment for NEC. STUDY DESIGN: The medical records of 29 premature infants with either SIP (n = 13) or NEC (n = 16) were reviewed retrospectively. RESULTS: Infants who experienced SIP were smaller at birth, had lower Apgar scores, and required more intensive neonatal resuscitation. An increased rate of premature rupture of membranes in infants with SIP (8/13 versus 6/16) was not associated with a higher rate of infection in mothers or infants. The onset of illness in SIP was significantly earlier than in NEC (p = 0.022). In contrast to patients with NEC (7/16), 11 of 13 patients in the SIP group had received indomethacin (p = 0.02). Bluish discoloration of the abdomen (8/13), a gasless abdomen (8/13), and the absence of pneumatosis intestinalis (0/13) were further significant markers in infants with SIP. At operation, SIP was always located in the terminal ileum in an antimesenteric position (13/13), and the remaining bowel appeared grossly normal. In most cases of SIP (10/13), the histologic investigation revealed an area of hemorrhagic necrosis without the typical coagulation necrosis seen predominantly in NEC. CONCLUSIONS: Based on clinical presentation and radiologic and intraoperative findings, SIP is a distinct pathologic entity in very-low-birth-weight infants and can be differentiated from classic NEC. Detected early, SIP can be treated by simple procedures (sutures, or resection and primary anastomosis) with a low rate of morbidity and mortality.

Longitudinal observation of antenatally detected congenital lung malformations (CLM): natural history, clinical outcome and long-term follow-up.

OBJECTIVE: The objective of the study is to present longitudinal observations in antenatally detected congenital lung malformations (CLM), particularly pulmonary sequestration (PS) and cystic adenomatoid malformation (CAM). METHODS: Fetuses found to have a CLM on prenatal ultrasound (US) were included in this study and followed up until delivery. In all newborns radiographs and computerized tomography (CT) studies of the thorax were performed. Surgical procedures included sequesterectomy, lobectomy, segmentectomy, and non-anatomic resection. Based on prenatal US findings, intrauterine course, postpartum chest radiographs and CT scans, as well as clinical signs and surgical findings patients were divided into six groups. RESULTS: Over a period of 6 years, routine prenatal US revealed suggestion of CLM in a series of 35 consecutive fetuses. In six cases pregnancy was terminated or the fetuses suffered fetal demise. Another four fetuses became symptomatic in utero when sequential scanning revealed hydrops, hydrothorax, and enlargement of cysts or polyhydramnios. Three cases in this group received serial therapeutic amniocentesis and serial puncture of either the hydrothorax or intrapulmonary cysts. After postpartum treatment in the intensive care unit surgical procedures were performed uneventfully and confirmed the diagnosis of CAM, PS or hybrid type lesions. In 11 patients US findings were considered to demonstrate spontaneous resolution of the lesion, but disappearance without sequelae could be confirmed only in six infants. Five infants were shown to have persistent CLM on postpartum CT scans. These infants underwent resection of the lesion within the first year of life. In 11 fetuses CLM were continuously demonstrated during pregnancy with only slight changes in size and structure. Postpartum the infants were asymptomatic and were subjected to a systematic plan of diagnostic work-up and treatment. Surgery in these infants revealed a large number of hybrid type lesions (n=5). In three infants, the primary diagnosis of PS or CAM had to be corrected during the diagnostic and therapeutic work-up. CONCLUSION: CLM are diagnosed antenatally with an increasing frequency and are shown to be quite different from previously applied concepts. The expected clinical outcome is far better than thought to be possible.

Haemoptysis in a teenager: late diagnosis of unnoticed foreign body aspiration.

Chest X-ray in a 17-year-old boy, presenting with haemoptysis, revealed a radiopaque foreign body (FB) in the right lower lobe. There was no history of aspiration. CT located the needle-shaped FB in the right posterobasal lower lobe segment bronchus. In bronchoscopy, the FB turned out to be a pin, of which the radiolucent plastic head was embedded in the peribronchial tissue. Extraction by flexible and rigid bronchoscopy failed; finally, thoracotomy and bronchotomy had to be performed to remove the pin. In delayed diagnosis of a tracheobronchial FB, CT scan is not only necessary to localise the FB but also to depict or rule out secondary pulmonary changes. Nevertheless, radiolucent components of a metallic FB might be invisible even in CT, leading to underestimation of its size and extension. Late diagnosis complicates removal of tracheobronchial foreign bodies and may even necessitate open surgery, including pulmonary resections.

Altered consciousness and lethargy from compromised intestinal blood flow in children.

Altered state of consciousness and lethargy could be early and cardinal complaints in diseases that actually originate within the abdomen. Thirteen children were seen at our departments in whom impairment of the individual's mental state preceded the appearance of common gastrointestinal symptoms. Whereas in infants with intussusception of childhood neurological signs and symptoms have been rarely mentioned, references to an altered state of consciousness and lethargy in volvulus, strangulation or bowel incarceration have not been previously reported. Recognition of this possibility, however, should be taken into account in the diagnostic work-up of children presenting with inexplicable alterations of their mental state.

Ureteropelvic junction disruption following blunt abdominal trauma.

We report here on a case of avulsion of the ureteropelvic junction in a 7-year-old boy who was injured in a car accident. Severe brain trauma took precedence over signs and symptoms of blunt abdominal trauma, but 24 hours after the accident, progressive distension of the abdomen required further evaluation. Ultrasound examination and a computed tomography scan revealed disruption of the ureteropelvic junction. Simple primary anastomosis was performed. We discuss the characteristics of this unusual injury and include a review of the literature.

Recurrent respiratory tract infections and dysphagia in a child with an aortic vascular ring.

Recurrent respiratory tract infections and dysphagia after the first years of life are rarely caused by vascular rings, and only a high index of clinical suspicion helps to avoid diagnostic delay and inappropriate treatment. Diagnostic workup in a 2-year-old girl with acute foreign body impaction into the esophagus and frequent respiratory tract infections revealed right descending aortic arch with ligamentum arteriosum as the cause of extrinsic esophageal-tracheal compression. Dividing the ligament gave release to the encircled esophagus and trachea. Relief of symptoms was achieved immediately after surgery.

Imaging of the scrotum in children.

Clinical examination of the scrotum is difficult due to the small size of the testes and the epididymis in infants and young children, and eliciting patients' history is challenging. Therefore imaging of the scrotum in childhood bears great importance. Ultrasound is the standard imaging technique of choice providing the clinicians with a definitive diagnosis in most cases. However, in conditions of testicular torsion and epididymoorchitis--the most common differential diagnosis of scrotal pain--ultrasound findings can be inconclusive and further evaluation is required. Since there is a large overlap between paediatric and adult pathology, differences from adults in anatomy and pathology must be considered when evaluating the paediatric scrotum.

Cervicofacial lymphadenitis due to atypical mycobacteria: a surgical disease.

Despite the increasing prevalence of cervicofacial lymphadenitis due to atypical mycobacteria (AMB) in children, the true nature of AMB infection in clinical practice is poorly understood. The purpose of our study was to define the most common signs and symptoms, and to establish a workable scheme of diagnosis and treatment. Patients fulfilling the criteria of AMB infection (i.e., clinical signs, positive cultures or polymerase chain reaction, histologic features) were included in the study. All children underwent a standard surgical procedure, depending on pretreatment and the course of the disease. Sixteen infants presented with characteristic unilateral lymphadenopathy predominantly involving the submandibular area (13/16). Eight children had been initially treated at various institutions by fine-needle puncture or incision, and 7 of the 16 patients had received antituberculous multidrug treatment for a varying length of time. Complete excision of the affected lymph nodes was the definitive treatment in all patients. Three children had transient marginal mandibular nerve paralysis that resolved within a few months in all cases. Recognition of the characteristic features of AMB adenitis may permit early diagnosis and appropriate surgical treatment.