N-Methyl-D-Aspartate Receptor Encephalitis with Psychiatric Symptoms and an Ovarian Teratoma Detected by MRI in a 17-Year-Old Girl.

N-methyl-D-aspartate receptor (NMDAR) encephalitis is a rare antibody-mediated autoimmune encephalitis often associated with an ovarian teratoma in adolescent females. Here we present a 17-year-old girl with only and unusual psychiatric symptoms as part of her NMDAR encephalitis in combination with a very small ovarian teratoma suspected by magnetic resonance (MR) imaging and finally histologically confirmed. We further review the literature of NMDAR encephalitis in combination with an ovarian teratoma and discuss the recommended radiological workup in children with a suspected ovarian tumor.

[Esophageal Atresia Repair - Can We Influence the Schedule?]. / Ösophagusatresie und tracheoösophageale Fistel - ist der Zeitpunkt der Versorgung steuerbar?

UNLABELLED: The treatment of newborns with esophageal atresia (EA) and tracheoesophageal fistula (TEF) is associated with a great logistic effort. The aim of the presented study was to analyse the possibility to influence the time of surgery. MATERIAL AND METHOD: Data from 30 neonates with EA and TEF regarding the date and mode of birth, biometric data and preoperative acid-base and blood gas values were collected retrospectively. The newborns were divided into two subgroups: birth between Monday and Thursday ("week"), and birth from Friday to Sunday ("weekend"). RESULTS: We observed a seasonal peak of births in November/December. The rate of prenatal ultrasound detection of polyhydramnions was 40%. In 14 of 16 cases with Caesarean section, maternal or foetal problems predicted the date and mode of delivery. In both groups, most newborns had an unimpaired postnatal adaptation. There were no significant differences regarding biometry. Delivery at the weekend was associated with later surgical repair (second vs. first day of life). Repeated estimations of acid-base and blood gas parameters over a median time span of 13 hours revealed a stable situation with a trend to normalisation. DISCUSSION: The time of birth is multifactorial and, in most cases, can neither be predicted nor influenced. Stable respiratory and metabolic parameters in the majority of patients allow a surgical intervention within a limited time frame during the first days of life. CONCLUSION: As it is hardly possible to plan the surgical procedure, an experienced team as well as neonatal intensive care facilities and operation room access must be available throughout the week.

Chondroepitrochlearis Muscle--A Phylogenetic Remnant with Clinical Importance.

We report on an infant, presenting with a cord-like accessory muscle crossing the anterior axillary fold. The accessory structure appeared as an axillary web and caused tethering of the humerus together with an abnormal shape of the shoulder. The chondroepitrochlearis muscle is thought to be of phylogenetic origin. In our patient the tendinous sling was resected at the age of 7 month with normalization of function and cosmetics.

Pediatric appendicitis may be co-associated with meteorologic factors: A case-crossover study.

BACKGROUND: The etiopathogenesis of appendicitis is still not fully understood. Few reports have researched the impact of weather as a cofactor for the development of appendicitis. METHODS: To investigate the potential association between the development of appendicitis and exposure to ambient meteorological conditions, a retrospective case-crossover study was conducted. Publicly available meteorologic data were matched with those from 1343 patients aged ≤19 years with histologically confirmed acute (AA; n = 996) or perforated appendicitis (PA; n = 347) treated at our pediatric surgical hospital between October 2001 and October 2018. RESULTS: A time-stratified conditional logistic regression analysis revealed increased odds ratios (ORs) for AA of 4-5% for each 1 hPa increase in vapor pressure during each 3-7 day cumulative moving averages (CMA) lag preceding appendectomy. This effect was stronger on stratification by age class >10 years (OR 1.05-1.08; 3-7-day CMA) and during spring (OR 1.08-1.09; 4-7-day CMA). Moreover, each 1% increase in relative humidity on the day of appendectomy was associated with a 2% decrease in the OR for PA, which was 4% during autumn under seasonal stratification. CONCLUSION: The role of meteorologic factors in the development of appendicitis remains largely unclear. In accordance with the literature, our results show that seasonal variations together with exposure to altered levels of humidity and vapor pressure may impact patients at different grades of appendiceal inflammation. Thus, transient fluctuations in meteorologic and seasonal variables may constitute cofactors that potentially influence the occurrence and course of pediatric appendicitis.

Levels of C-Reactive Protein and Sodium May Differentiate a Perforated Appendix from a Nonperforated Appendix in Children.

BACKGROUND: Acute appendicitis (AA) might be amenable to conservative antibiotic treatment, whereas a perforated appendix (PA) necessitates surgery. We investigated the value of clinical-laboratory markers in distinguishing AA from a PA. METHODS: Retrospectively obtained preoperative parameters for 306 consecutive patients (<18 years) with histologically confirmed appendicitis (AA (n = 237) vs. PA (n = 69)), treated at our institution between January 2014 and December 2017. RESULTS: A PA was associated with male preponderance, younger age, decreased sodium level and increased white blood cell count, Tzanakis score, C-reactive protein (CRP) level, and CRP-to-lymphocyte ratio (CLR). Upon discrimination analysis, CLR and CRP displayed the highest accuracy in differentiating a PA from AA. Regression analysis identified levels of CRP, sodium, and the Tzanakis score as independent predictors for a PA. CONCLUSION: Levels of CLR, CRP, sodium, and Tzanakis score might support decision-making regarding treatment options for pediatric appendicitis.

Direct bladder hernia after indirect hernia repair in extremely low birth weight babies: two case reports and a review of the literature.

BACKGROUND: Inguinal hernia repair is the most common surgical procedure in babies. Despite a meticulous technique, relapses may occur. The occurrence of a direct bladder wall hernia in relapses has never before been reported in the literature. CASE PRESENTATION: Here, we report two cases of direct bladder herniation: a white baby boy born after 25 weeks of gestation and a white baby boy born after 26 weeks of gestation. Both of the formerly extremely low birth weight babies were affected after open bilateral hernia repair. Recurrent hernias developed on the right side, and direct bladder herniation was identified intraoperatively. In one case, laparoscopy was applied to identify a supravesical type of hernia. Immaturity and a difficult postnatal course might have contributed to hernia relapse in these cases. CONCLUSIONS: Misinterpretation of bladder herniation might have disastrous consequences. Laparoscopy is a helpful tool in comparable cases.

Single-port laparoscopic percutaneous extraperitoneal closure of femoral hernia in children and adolescents: case series.

BACKGROUND: Femoral hernias are an often unexpected groin pathology during childhood. However, the pediatric surgeon has to be aware to diagnose femoral hernia and to repair this condition. This is the first report on laparoscopic percutaneous extraperitoneal closure of femoral hernia (LPEF) in children and adolescents. METHODS: Over a 6-year period in a bi-institutional clinical study, we retrospectively identified six children and one young adult who underwent LPEF repair. RESULTS: Femoral hernia was laparoscopically confirmed in seven patients. Ages at surgery were 3, 5, 7, 7, 8, 8.5, and 18 years, respectively. In the first case, we combined laparoscopic diagnosis with open repair. In the consecutive six cases, hernias were repaired minimally invasively with the percutaneous extraperitoneal technique described below. During a follow-up between 6 years and 6 months, no recurrence was observed. SURGICAL TECHNIQUE: For LPEF, we percutaneously placed a peritoneal U-shape suture with integrated transfixation of the hernia sac, closed with an epifascial knot. We performed LPEF using two graspers. The peritoneum was percutaneously punctured with a venous cannula through which the suture was inserted. One grasper was inserted through the working channel of the laparoscope to invert the hernia sac into the abdominal cavity. A mini-grasping forceps inserted through the cannula retrieved the thread and completed LPEF. CONCLUSION: We demonstrate that single-port laparoscopic percutaneous extraperitoneal closure of femoral hernia is successful and quick in children and in adolescents.

Isolated tracheoesophageal fistula versus esophageal atresia - Early morbidity and short-term outcome. A single institution series.

PURPOSE: We compared the postnatal course, morbidity and early results after repair for cases of isolated or "pure" TEF with those for cases of esophageal atresia (EA) with distal tracheoesophageal fistula (TEF). METHODS: Twenty-four consecutive infants were divided into two groups: isolated TEF [TEF group] (n = 5) and EA with distal TEF [EA group] (n = 19). RESULTS: A high rate of prematurity (29%) and major cardiac and other surgically-relevant malformations (0.8 vs. 0.7 per infant) was found in both groups. The median age at surgery was 8 days for the TEF group vs. 1 day for the EA group (p < 0.01). Most infants of both cohorts had stable acid-base and respiratory parameters at admission. Generally, tracheoscopy provided valuable information regarding the position of the TEF. Surgery for isolated TEF was performed via right cervicotomy in 4 cases and via thoracotomy in one. Postoperative thoracostomy tubes were inserted in 3 cases and one emergency gastrostomy was created for acute gastric overextension (exclusively in patients with EA). The duration of postoperative mechanical ventilation (49 vs. 113 h, p = 0.045) and the median length of stay in the pediatric surgery unit (10 vs. 20.5 days, p = 0.003) were shorter for the isolated TEF group. Four EA patients experienced severe events. Total mortality was 8% (0 out of 5 with TEF vs. 2 out of 19 with EA). CONCLUSION: Developmental delay and a high rate of morbidity were found in both groups. More complex surgery increased perioperative morbidity in cases of EA. With early recognition of isolated TEF, a less complicated course can be expected in comparison with esophageal atresia.

Congenital oesophageal atresia discordant for tracheo-oesophageal fistula occurring in a set of dizygotic twins.

The authors present a set of female diamnionic and dichorionic twins with different blood types and congenital oesophageal atresia (EA) in both. Surgical management was successful. It can be assumed that EA with tracheo-oesophageal fistula in twin B occurred during an early embryological stage whereas the isolated EA in twin A was the result of a later event. To our knowledge, this is the first published set of dizygotic twins with different types of EA.

Renal function and urine drainage after conservative or operative treatment of primary (obstructive) megaureter in infants and children.

UNLABELLED: We examined renal function and urinary drainage of children with primary megaureter (PMU) in dependence on conservative or operative treatment. MATERIAL AND METHODS: The retrospective analysis covering the years 1994 to 2000 comprised children at an age of 0-7 years with 35 PMU. Sonography, dynamic MAG3 renography as well as endogenic creatinine clearance (GFR) were used to assess drainage and the renal function. Temporary urinary diversion was established in fourteen patients of both groups. In 14 children with 16 PMU a ureteroneocystostomy (UNC) was performed. The average observation period was 30 months (11-108). RESULTS: The children of the UNC group differed from the non-neoimplanted group in the age at diagnosis (10.5 vs. < 1 months), higher degrees of hydronephrosis on average, a more distinct dilatation of the ureter as well as renographically significant obstruction. Children of the non-UNC group, including four children with a type B drainage curve (O'Reilly), had an unimpaired differential renal function or improved during the observation period (initially 51% vs. 50.5% at the end). In neoimplantation group the differential function improved from 32.5% to 38.5% (p < 0.05) and obstruction resolved with one exception. CONCLUSION: Given a higher-grade PMU with a reduced function of the kidneys and a significant impaired drainage pattern and/or symptoms, neoimplantation without temporary diversion has proved to be an efficient renoprotective method. Furthermore, data clearly justify a conservative approach without urinary diversion in infants with large asymptomatic PMU.

[Diagnostically Approach to Pediatric Carpal Fractures: a Retrospective Analysis]. / Diagnostisches Vorgehen bei Handwurzelknochenfrakturen im Kindesalter: eine retrospektive Analyse.

INTRODUCTION: Carpal fractures in children are rare, but can be missed, as their clinical symptoms are unspecific and discrete. Even X-ray diagnosis is difficult. Timely diagnosis and consistent therapy are especially important for scaphoid fractures, as they can help to avoid complications such as non-union or avascular necrosis. A diagnostic approach to paediatric carpal fractures will be discussed on the basis of the following group of patients. METHODS: Retrospective analysis of children under 14 years treated in our institution between 09/2010 and 02/2012 for clinically suspected carpal fracture. In the primary evaluation, all children underwent standard X-rays of the hand and/or wrist. All patients were treated by cast immobilisation until complete clinical recovery. All patients with clinical signs of carpal fracture were treated by cast immobilization, even with normal X-rays. The clinical follow-up examination was after 10 to 14 days. In patients with persistent complaints, MRI was performed. We retrospectively evaluated the records of all patients: the fractured carpal bone, and X-ray and MRI-diagnosis were stated. We calculated the mean difference between first presentation and MRI and the mean period for total recovery, in patients with fracture or non-fracture. RESULTS: 61 children (27 boys and 34 girls, mean age 11.5 y) were included in our study. The mean delay between accident and time of first presentation to our paediatric ED was 0.6 days. In primary X-rays, a carpal fracture was demonstrated in only in 2 (3.3 %) patients, but was suspected in only 6 (9.8 %) of patients. In 53 (87.9 %) patients, there was no radiographic evidence of carpal fracture. 14 patients underwent additional scaphoid views, but scaphoid fracture was confirmed in only 1 (7 %) of these patients. In 3 (21.4 %) patients, a scaphoid fracture was suspected and in 10 patients a carpal fracture could be excluded. After a mean time of 11.8 days, all patients underwent a clinical follow-up examination. 32 (54 %) patients had persistent symptoms and MRI was done after a mean time of 17 days. Carpal fracture was then excluded in 12/32 (37 %) patients and was diagnosed in another 20/32 (63 %) children. There were 14 scaphoid fractures, including 3× bone bruise lesions, 4 capitate fractures, 3 triquetral fractures, including 1× bone bruise lesion and 1 bone bruise lesion of the trapezoid. In patients with proven carpal fracture, it took a mean time of 56 days for complete recovery, in comparison with 15 days in patients with excluded carpal fracture. Surgical therapy was unnecessary in any of the patients, and there were no complications. CONCLUSION: In children with clinical and radiographic carpal fracture, diagnosis is difficult and often unsuccessful at first. Even in discrete clinical complaints, generous cast immobilization is essential and clinical follow up is recommended not later then 14 days. In patients with persistent clinical symptoms, MRI is the imaging method of choice, as it is capable of detecting carpal fractures and even bone bruise lesions with high sensitivity, thereby avoiding unnecessary diagnostic or therapeutic stress for the patients.

A new heterozygous mutation of the FOXL2 gene is associated with a large ovarian cyst and ovarian dysfunction in an adolescent girl with blepharophimosis/ptosis/epicanthus inversus syndrome.

Blepharophimosis/ptosis/epicanthus inversus syndrome (BPES), an autosomal dominant syndrome in which eyelid malformation is associated with (type I BPES) or without premature ovarian failure (type II BPES). Mutations of a putative winged helix/forkhead transcription factor FOXL2 account for both types of BPES. We report on a 16-year-old adolescent girl with blepharophimosis and ptosis. Subsequently she developed oligomenorrhea, secondary amenorrhea for 6 months, and an extremely large cyst of one ovary. The cyst contained 8 l of cyst fluid and histopathology displayed a large corpus luteum cyst. Following laparotomy, gonadotropin levels were elevated (LH 17.2 U/l, FSH 29.4 U/l) and estradiol levels decreased (67 pmol/l). Because of clinical aspects of BPES and abnormal ovarian function we suspected a mutation of her FOXL2 gene and found a new in-frame mutation (904_939dup36) on one allele, leading to a 12 alanine expansion within the polyalanine domain. We conclude that the FOXL2 mutation 904_939dup36 may account not only for blepharophimosis and ptosis but also for ovarian dysfunction and growth of the large corpus luteum cyst. In contrast to known FOXL2 mutations with polyalanine expansions and association with BPES type II, clinical aspects of our girl may indicate some degree of ovarian dysfunction that might finally lead to BPES type I with premature ovarian failure.

Pediatric MR-guided interventions.

MRI guided interventions are a relatively new but steadily growing field within surgery in pediatric age. Besides the advantages of MRI, such as multiplanar capability and excellent soft tissue contrast and spatial resolution, particularly relevant for the pediatric population is the lack of ionizing radiation. There is meanwhile a group of well defined diagnostic or therapeutic indications for applying MR imaging during pediatric interventions. Aim of this review is to give an overview about indications of MR-guided procedures in children as well as the advantages and disadvantages of MR-guided interventions. We also briefly discuss interventional MR-systems and MR-compatible devices. It is our opinion that MR-guidance for pediatric interventions is a promising technique at the beginning of its development.

Acute portal vein thrombosis after splenic embolization and splenectomy for autoimmune pancytopenia.

Postsplenectomy portal vein thrombosis for hematological diseases is uncommon in the pediatric population. The case summarized is, to our knowledge, the first manifestation of portal vein thrombosis in a child after preoperative splenic artery embolization and subsequent splenectomy for severe hypersplenism. We suggest that early routine diagnosis by Doppler ultrasonography and subcutaneous low molecular weight heparin therapy are useful steps for a successful outcome.

Mechanisms of hyperthermia- and 4-hydroperoxy-ifosfamide-induced cytotoxicity in T cell leukemia.

The prognosis of patients with early ALL (acute lymphoblastic leukaemia) relapse is poor with conventional chemotherapy alone. Thus, intensified chemotherapy strategies are required. The application of hyperthermia enhances the efficacy of certain antineoplastic drugs such as ifosfamide. In this study, the effects and molecular mechanisms of ifosfamide (4hydroperoxy-ifosfamide = 4OOH-IFA)- and/or hyperthermia-induced cell death are investigated in CEM cells. Hyperthermia enhanced the efficacy of 4OOH-IFA in a subaddictive manner. Analysis of caspase activation revealed an early hyperthermia-induced stimulation of caspase-3 and -6 directly after the heating pulse, while maximum activation following stimulation with 4OOH-IFA was obtained after 24 hours of culture. The combination of 4OOH-IFA and hyperthermia mediated an overaddictive caspase stimulation directly following the heating phase. At this time also an overaddictive cytotoxic effect was noticed, being mainly responsible for the enhancing effects of hyperthermia on 4OOH-IFA cytotoxicity. In conclusion, hyperthermia enhanced the cytotoxic effect of 4OOH-IFA on CEM cells by stimulation of an early 4OOH-IFA effect. Thus, thermochemotherapy might be considered as an intensifying treatment option in relapsed T cell leukemias.

[MRI-guided pediatric interventions]. / MRT-gestützte Interventionen bei Kindern - Retrospektive Auswertung einer Fallserie.

AIM: There are only a few diagnostic or therapeutic procedures where MR imaging is applied during pediatric interventions. This study will describe indications, complications as well as the advantages and disadvantages of MRI-guided interventions based on a case study in an open MRI scanner. MATERIALS AND METHODS: 14 procedures were performed in an open MRI scanner (Signa SP/i, GEMS) on 13 children (1 - 16 yr) with bone, soft tissue and brain lesions (1 - 4 cm diameter). Localization of the pathology, targeting and final control of the result were based on images acquired with SE-, 3D-, GE-sequences pre- and post-contrast as well as FSE-sequences. Interactive MRI-guided instrument navigation was performed using a multiplanar T1w GE-sequence. RESULTS: Eight biopsies (incl. 1 rebiopsy), two tumor resections, one removal of a free joint mouse and three tumor-markings were performed without complications. Five biopsies provided appropriate sample quality for a histological diagnosis while two samples were too fragmented requiring a second biopsy (one of them MRI-guided). Surgical progress during tumor resection and marking could be controlled during the intervention without repositioning the patient. DISCUSSION: MR image guided pediatric interventions combine the advantage of an imaging system without ionizing radiation and the high soft-tissue contrast. The low number of these kinds of procedures can be explained by the relatively high costs for the intervention, the increased duration for each procedure and the limited availability of open MRI systems. The integrated localization-system helps during planning of the access path when facing complex anatomical structures and provides safe navigation in sensitive regions like the epiphyseal cartilage. Recent and expected developments of the required MR-compatible biopsy instruments could provide higher efficiency for appropriate sample size and quality. Summarizing, MRI-guided pediatric interventions have shown to be a promising method at the beginning of its development.