Vascular Anatomy in Congenital Lung Lesions-Description and Classification.

Background: Bronchopulmonary sequestration (BPS) and hybrid lesion of congenital pulmonary airway malformation (CPAM) are congenital lung lesions typically presenting with systemic vascular connection. We describe and categorize this atypical systemic vascular anatomy in congenital lung lesions. Methods: In a medical chart review from 2005 to 2020 patients with systemic vascular connection of congenital lung lesions were identified. Clinical and radiological data were collected and compared. Two experienced pediatric radiologists reviewed postnatal thoracic contrast-enhanced computed tomography scans to describe and categorize atypical vascular anatomy. We completed our findings with a review on vascular anatomy in congenital lung lesions. Results: A total of 21 patients with congenital lung lesions (nine extralobar BPS, five intralobar BPS, seven hybrid lesions) had systemic arterial supply; with seven of these additionally having systemic venous drainage. Origin of the feeding arteries from the aorta or aortic main branches was described as supra-diaphragmatic (descending thoracic aorta) in nine and infra-diaphragmatic in ten patients (abdominal aorta, celiac trunk). In two patients with hybrid lesions both supra- and infra-diaphragmatic arterial feeders were found. Additional systemic venous connection of supra-diaphragmatic type drains into the azygos-hemiazygos system (4/21) while the infra-diaphragmatic type (3/21) drains into caval vein, portal or splenic vein. Conclusion: Various variants of systemic arterial and venous connection of congenital lung lesions can be found. Classification of systemic arterial connection as well as venous drainage of congenital lung lesions as supra-diaphragmatic and infra-diaphragmatic types is intuitive, simple and may be important for the surgeon to avoid unanticipated situations and to perform safe resections.

Treatment Options for Scrotal Migration of Ventriculoperitoneal Shunts: Case Illustration and Systematic Review of 48 Cases.

BACKGROUND: Scrotal migration of intact or disconnected tubing is a rare complication of ventriculoperitoneal shunts. While some illustrative case reports can be found in the literature, a systematic review on treatment options is lacking. OBJECTIVE: To propose the first literature-based treatment algorithm on scrotal shunt migration. METHODS: We conducted a literature search using the keywords: "VP," "ventriculoperitoneal," "shunt," and "scrotum." We identified 36 publications with 48 cases reported including our index case. RESULTS: Median age at presentation was 13.5 mo (3 d to 65 yr) which was 4 mo (3 d to 72 mo) after last shunt-related surgery. All patients had scrotal swelling, 39 (81%) patients presented without other symptoms, 4 (8%) had additionally local pain, and 4 (8%) patients presented with symptoms of shunt dysfunction. Treatment was surgically in all but one case where spontaneous resolution without repeat migration occurred. In 3 of 4 patients who had either subcutaneous shortening or abdominal repositioning of the shunt without hernia repair, scrotal shunt migration recurred within the following month. Whereas the surgical treatment with reposition of the migrated catheter back into the peritoneal cavity via a groin incision plus hernia repair yielded a definite treatment in all 26 performed cases, the revision rate was significantly higher in the shunt revision without hernia repair cohort (P = .0009). CONCLUSION: Scrotal shunt migration is a rare shunt complication with good recovery when treated surgically. We recommend hernia repair in addition to either manual or surgical repositioning of migrated tubing.

Hereditary pancreatitis in childhood: course of disease and complications.

BACKGROUND: Hereditary pancreatitis is rare. Pain therapy for juvenile symptom onset, child development and the risk of pancreatic carcinoma in adulthood must be considered. PATIENTS, MATERIAL AND METHODS: Data from a cohort of 11 patients with disease onset in childhood (< 16 years) were analyzed retrospectively. The gene encoding cationic trypsinogen (PRSS1), serine protease inhibitor Kazal type 1 (SPINK1) and cystic fibrosis transmembrane conductance regulator (CFTR) genes were investigated as genetic factors. Treatment concept and complications were registered. Prognosis, treatment success and quality of life were objectified using the chronic pancreatitis prognosis score and a standardized questionnaire (KIDSCREEN-10 index). RESULTS: The mean age of symptom onset was 7.5 ±4.2 years. The PRSS1 and SPINK1 mutations each occurred with 36.4%, 3 patients had a pancreas divisum and 2 a long common channel. The course of pancreatitis was obstructive in 90.9%. Exocrine pancreatic insufficiency occurred in seven patients so far (mean age 12.5 years). Stenting was performed in 72.7% and 18.2% needed pancreatic surgery. Currently the chronic prognosis score is on average 7.5 points, pain on numerical rating scale 0 (no pain). The mean KIDSCREEN­T score of 66.9 confirms a very good quality of life. CONCLUSION: Patients with genetically caused chronic pancreatitis are rare. Their care ranges from pain therapy in childhood and adolescence to questions concerning family planning and pancreatic cancer prevention from mid-adulthood onward. The disease is challenging for the interdisciplinary cooperation. We found the step-up strategy to be a good option for pain therapy. A national registry monitored by scientific societies with active recruitment for screening examinations will further improve and ensure care in the long term.

Torsion of an undescended testis - A surgical pediatric emergency.

BACKGROUND/PURPOSE: Torsion of an undescended testis is a surgical emergency whose frequency may be underestimated in the pediatric population. We describe this entity and focus on diagnostic challenges and optimal treatment of torsion of an undescended testis. METHODS: We present a two-center retrospective chart review of patients with torsion of an undescended testis treated between 2013 and 2018. Two instructive cases are used to depict characteristics of this rare entity. RESULTS: We identified 11 patients with previously diagnosed cryptorchidism undergoing surgery for torsion of an undescended testis, accounting for 9.7% (11/107) of all testicular torsions in the period. Mean age at diagnosis was 9.4 months (1-22 months). Mean duration from onset of symptoms to presentation was 19.3 h (8-48 h). At admission to hospital 10 patients presented with groin lump (10/11, 90.9%) with or without pain leading to a suspected diagnosis of inguinal testicular torsion (5/11, 45.5%), incarcerated inguinal hernia (4/11, 36.4%) and epididymitis (1/11, 9.1%). Ten patients had an ultrasound examination before surgery leading to the correct diagnosis in six patients. Ultrasound findings were misinterpreted as incarcerated inguinal hernia in three patients. In eight patients the testis had to be removed at time of surgery; one of the three salvaged testes atrophied, resulting in a salvage rate of 18%. CONCLUSION: Torsion of an inguinal testis is not as rare as it might be presumed. Presentation of these patients is often deferred owing to equivocal signs and symptoms. In addition age at presentation differs from typical testicular torsion. As this might negatively influence testicular salvage rate, we advocate for special attention to this differential diagnosis in children with groin pathologies. Even if the child is not in pain, a tender groin in boys with undescended testes must prompt a quick and thorough examination to rule out torsion of an undescended testis. Ultrasound examination is of limited value and must not delay acute surgical treatment. LEVEL OF EVIDENCE: IV.

Pediatric chylothorax-lymphatic imaging enables targeted surgical treatment.

Chylothorax-the collection of lymphatic fluid in the pleural space-is a rare finding in otherwise healthy adolescents. Initially, clinical signs and symptoms are often non-specific and a wide range of underlying causes necessitates extensive diagnostic workup. Treatment options include dietary measures, medical treatment, and various surgical procedures. We report about a 12-year-old boy with accidental diagnosis of chylothorax. Lymphatic imaging led to visualization of a leakage of an accessory left-sided thoracic duct and thoracoscopic clipping was successfully performed. Lymphatic imaging procedures depict underlying causes of chylothorax allowing targeted therapeutic management.

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.

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.

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.

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.

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.

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.