Three-dimensional reconstruction of defects in congenital diaphragmatic hernia: a fetal MRI study.

OBJECTIVE: To assess the clinical feasibility and validity of fetal magnetic resonance imaging (MRI)-based three-dimensional (3D) reconstruction to locate, classify and quantify diaphragmatic defects in congenital diaphragmatic hernia (CDH). METHODS: This retrospective study included 46 cases of CDH which underwent a total of 69 fetal MRI scans (65 in-vivo and four postmortem) at the Medical University of Vienna during the period 1 January 2002 to 1 January 2017. Scans were performed between 16 and 38 gestational weeks using steady-state free precession, T2-weighted and T1-weighted sequences. MRI data were retrieved from the hospital database and manual segmentation of the diaphragm was performed with the open-source software, ITK-SNAP. The resulting 3D models of the fetal diaphragm and its defect(s) were validated by postmortem MRI segmentation and/or comparison of 3D model-based classification of the defect with a reference classification based on autopsy and/or surgery reports. Surface areas of the intact diaphragm and of the defect were measured and used to calculate defect-diaphragmatic ratios (DDR). The need for prosthetic patch repair and, in cases with repeated in-vivo fetal MRI scans, diaphragm growth dynamics, were analyzed based on DDR. RESULTS: Fetal MRI-based manual segmentation of the diaphragm in CDH was feasible for all 65 (100%) of the in-vivo fetal MRI scans. Based on the 3D diaphragmatic models, one bilateral and 45 unilateral defects (n = 47) were further classified as posterolateral (23/47, 48.9%), lateral (7/47, 14.9%) or hemidiaphragmatic (17/47, 36.2%) defects, and none (0%) was classified as anterolateral. This classification of defect location was correct in all 37 (100%) of the cases in which this information could be verified. Nineteen cases had a follow-up fetal MRI scan; in five (26.3%) of these, the initial CDH classification was altered by the results of the second scan. Thirty-three fetuses underwent postnatal diaphragmatic surgical repair; 20 fetuses (all of those with DDR ≥ 54 and 88% of those with DDR > 30) received a diaphragmatic patch, while the other 13 underwent primary surgical repair. Individual DDRs at initial and at follow-up in-vivo fetal MRI correlated significantly (P < 0.001). CONCLUSIONS: MRI-based 3D reconstruction of the fetal diaphragm in CDH has been validated to visualize, locate, classify and quantify the defect. Planning of postnatal surgery may be optimized by MRI-based prediction of the necessity for patch placement and the ability to personalize patch design based on 3D-printable templates. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Early exposure to latex products mediates latex sensitization in spina bifida but not in other diseases with comparable latex exposure rates.

BACKGROUND: The high prevalence of latex sensitization in patients with spina bifida (SB) has been attributed to repeated and early exposure to latex products. Other diseases such as gastroschisis/omphalocoele and post-haemorrhagic/congenital hydrocephalus are also associated with repeated and early latex exposure. OBJECTIVE: The aim of the study was to evaluate whether the high prevalence of latex sensitization in patients with SB is rather related to the underlying disease itself than to disease-associated known risk factors. METHODS: We compared children with SB (n=35), children with gastroschisis/omphalocoele (G/O, n=20) and children with post-haemorrhagic/congenital hydrocephalus (PH, n=45). All children with SB and PH had a ventriculo-peritoneal shunt since a very young age. Patients who underwent three or less surgical procedures matched in terms of age, number of operations, atopy and gender distribution, and were analysed for IgE sensitization rates to latex. RESULTS: In the SB group, 16 of 35 patients (46%) showed elevated latex-specific IgE antibodies in contrast to one of 20 patients (5%) in the G/O group and four of 45 patients (8.9%) in the PH group (P<0.0005 and P<0.005, Fisher's exact test). Comparing matched control groups (

Advancements in the diagnosis of acute appendicitis in children and adolescents.

The aim of our study was to further improve the preoperative diagnosis of acute appendicitis in children and adolescents. All diagnostic parameters from the patients' medical history (duration and quality of abdominal pain, stool behaviour), the laboratory (leukocytes, C-reactive protein), the clinic (defense, tenderness on percussion, nausea, vomiting, dry tongue) and repeated ultrasound investigations (visualisation of the appendix, indirect signs of an inflammatory process in the appendix region) were documented prospectively and were re-assessed with regard to their diagnostic value. As an additional parameter, procalcitonin was determined. 1156 patients (593 male/563 female) with a mean age of 9.51 years (+/- 1.2 yrs) (max. 15 yrs/min. 2.3 yrs), referred to the department with acute abdominal pain, were examined. 233 (141 male/92 female; 20.1 %) of these patients with a mean age of 10.47 years (+/- 1.1 yr) had appendicitis. Based on the patients' medical history, laboratory findings, the initial clinical investigation and the initial ultrasound investigation, 173 patients (74.3 % of the later operated 233 children with appendicitis) were diagnosed with certainty. The diagnosis of 60 patients (25.7 %) of this group remained uncertain. These patients received a saline enema (Clysmol, Pharmacia & Upjohn Company) and were subjected to a second clinical and sonographic investigation after approximately four hours of parenteral fluid substitution (Ringer's lactate, Mayrhofer Pharmazeutika Company, 4 ml/kg/h). The other 923 patients (79.83 %) were discharged and were followed up as outpatients in the following days. Based on this stepwise procedure, the percentage of correctly diagnosed appendicitis could be increased to 97.4 %. The measurement of procalcitonin proved to be of no value in the diagnosis of acute appendicitis. It may be concluded that in children with abdominal pain, high diagnostic accuracy can only be achieved by a carefully combined evaluation of all individual diagnostic parameters and repeated investigations.

Neovascularisation of the testes by experimental omentotesticulopexy.

With the currently used surgical procedures, surgical correction of extreme forms of high undescended testes is associated with an atrophy rate of 10% to 30%. In this experimental study in two groups of Sprague-Dawley rats of pubescent age, we examined whether fixation (pexy) of an omental flap on the testis (omentotesticulopexy = OMTX) permits preservation of the viability of the parenchyma. A left-sided OMTX was performed in group 1 with the animals under general anaesthesia. An omental flap was prepared. The tunica albuginea of the testis planned for OMTX was pierced with a needle (26 x 23 G) within an area with a diameter of 1 cm; a puncture distance of 2 mm was used. The omental flap was then fixed using absorbable monofilament 6-0 interrupted sutures. Six weeks later the spermatic vessel was transected at this site. The right testis was left untouched throughout the procedure; it remained in situ. In group 2 only the spermatic vessel on the left side was severed. The right testis was not manipulated and remained in situ. After a total of 10 weeks both testes were removed from all rats and fixed in formalin. Standardised haematoxylin and eosin staining was performed and histological sections were obtained. The size of the testicles, Leydig-cell count, viability, and calcified areas within the tissue were determined for all testicles. Student's t-test was used for statistical evaluation. The study showed that OMTX leads to neovascularisation in the area of fixation and that viability can be preserved within this area.

Umbilicoplasty in neonates with primary omphalocele closure.

A new technique of umbilicoplasty in neonates who underwent primary omphalocele repair is described and illustrated. The procedure resulted in a nearly-normal appearance of the umbilicus.

Sonographic management of infantile clavicular fractures.

A new method for radiation-free management of infantile clavicular fractures is presented. Forty-nine infants with clavicular fractures were examined radiologically and sonographically by independent examiners. The two imaging techniques were compared with regard to practicability, diagnostic results, and assessment of the healing process. The paired t-test showed no significant difference between X-ray and ultrasound (US) for the criteria of practicability and primary diagnostic results. With regard to assessment of the healing process, a highly significant advantage in favor of US was registered. One week before radiographs revealed any sign of bone healing, stress-resistant, stable healing of tissue is seen on US. Even consolidation disorders such as early signs of pseudarthrosis are detected very early by US. This enormous diagnostic advantage reduces the duration of immobilization, the frequency and number of follow-up examinations, and last but not least, provides a cost-effective method of treatment without exposure to ionizing radiation.

Special compression sutures: a new surgical technique to achieve a quick decrease in shunt volume caused by diffuse hemangiomatosis of the liver.

A new surgical technique for the management of shunting liver hemangiomas that otherwise would rapidly lead to intractable cardiac failure is presented. Rapid, significant reduction of shunt volume was achieved surgically by transhepatic compression sutures using PTFE pledgets and selective ligation of a large feeding vessel from the right hepatic artery.

Accessory scrotum or perineal collision-hamartoma. A case report to illustrate a misnomer.

A case of a congenital perineal tumor in a male infant is described, which consisted of separated fat and smooth muscle tissue in nodular arrangement. According to its clinical presentation, this lesion would be grouped into the category of so called "accessory scrotum," as part of the entity of ectopic scrotum. Based on our case and on a review of the literature, the possibility is raised that this labelling may be inappropriate on grounds of etiology and pathomorphology. It is suggested to consider similar lesions as the result of a perineal hamartomatous fat tissue overgrowth with transposed scrotal skin, which may or may not show additional hamartomatous proliferation of smooth muscle bundles, and to label them "perineal lipoma/lipoblastoma with ectopic scrotal skin" or "perineal collision-hamartoma" in the case of a prominent isolated smooth muscle component.

Influence of different conditioning methods on force and fatigue resistance in chronically stimulated skeletal muscles.

A study was undertaken to compare different conditioning methods for the transformation of latissimus dorsi muscle into a fatigue resistant one for application in circulatory assist. In ten sheep four electrodes were sutured to the epineurium of the left thoracodorsal nerve for indirect electrical stimulation of the latissimus dorsi muscle. In six sheep a "carousel stimulation," a special multichannel stimulation, in combination with a recently developed conditioning protocol was used for muscle conditioning (multichannel method). In four sheep, a conventional stimulation protocol using single channel stimulation was applied for transformation of the muscle (single channel method). The final experiments were carried out when fatigue resistance was obtained. The maximum tetanic forces at different preloads were determined and fatigue resistance was tested during 20 minutes of continuous stimulation. Both conditioning patterns led to fatigue-free chronic stimulation. Muscles conditioned by multichannel stimulation exhibited between 20% and 33% less force than the contralateral unconditioned muscles, whereas in the single channel group this loss was between 32% and 43%. Thus, the multichannel method revealed relatively superior in preserving muscle force for chronic stimulation.

Diaphragmatic paresis in newborns due to phrenic nerve injury.

Phrenic nerve lesions as a result of birth trauma have been reported as a cause of acute respiratory distress infrequently. We report recent diagnostic and therapeutic experiences in four newborns with birth-traumatic phrenic nerve injury: one bilaterally, and three unilaterally, all right-sided. In each case, mechanical ventilation was required for at least 16 days. Ultrasound examination of the diaphragm and phrenic nerve conduction studies turned out to be the diagnostic methods of choice. Spontaneous recovery occurred in two children and two became asymptomatic after operative treatment. One improved after plication of diaphragm and one after autologous nerve transplantation.

[Meconium peritonitis: intrauterine follow-up--postnatal outcome]. / Mekoniumperitonitis: Intrauteriner Verlauf--postnatales Outcome.

In nine patients with meconium peritonitis prenatal ultrasonographic findings were correlated with the clinical course and outcome. Ultrasound findings included polyhydramnion (n = 4), ascites (n = 4), disseminated (n = 3) and solitary echogenic areas (n = 1), echopoor cystic areas (n = 3) and echogenic-echopoor solitary areas (n = 1). Intra-abdominal calcifications were found in five patients before delivery. Eight neonates survived and were subsequently followed up, one fetus died in utero. Four of the eight survivors required surgery, namely for meconium ileus (n = 1), perforation secondary to intestinal volvulus (n = 2) and inguinal hernia associated with prenatal rubella infection (n = 1). Three patients were healthy, one patient required drainage of pleural effusion and respirator therapy but recovered without further problems. Cystic fibrosis was diagnosed in the patient with meconium ileus. Postnatal outcome could not be predicted from the prenatal sonographic findings.

Analysis of 309 cases of esophageal atresia for associated congenital malformations.

Three hundred nine cases of esophageal atresia were retrospectively investigated. One hundred and sixty-two (52.4%) had associated congenital malformations. The total number of malformations was 357. The eight groups of malformations in decreasing order of frequency were as follows; cardiac, 72 cases (23.3%); musculoskeletal, 55 cases (17.8%); anointestinal, 50 cases (16.2%); genitourinary, 45 cases (14.6%); head and neck, 31 cases (10.0%); mediastinal, 26 cases (8.4%); chromosomal, 17 cases (5.5%); pulmonary, 6 cases (1.9%). Of the 162 cases with associated malformations, 83 (51.2%) had a single group of malformations, 41 (25.3%) had two groups of malformations, and 38 (23.5%) had three or more groups of malformations. More than 50% of pulmonary (83.3%), head and neck (77.4%), chromosomal (58.8%), and mediastinal malformations (53.8%) were found in association with cardiac malformations. The frequency of associated malformations in esophageal atresia cases was found to be increased significantly during the investigated interval. Early gestational age and lower birthweights were significantly correlated with higher rates of malformations. The survival rates of patients with esophageal atresia was also found to be significantly increasing over time. The mean survival rates over 32 years for groups with and without malformations were 38.3% and 70.7%, respectively, and were statistically significant. The mean survival rates of the same groups in the last 5 years were found to have changed dramatically, rising to 62.9 in the group with malformations and to 100% in the group without malformations; again, the difference is significant.

Sonographic detection of internal jugular vein thrombosis after central venous catheterization in the newborn period.

We sonographically investigated the internal jugular veins of 40 children who had undergone catheterization of the vein (group A: silastic catheter, n = 24; group B: polyurethane catheter, n = 16) in the neonatal period. The average age at catheter implantation was 43 +/- 73 days, the average birthweight 2414 +/- 1145 g, and the average gestational age 34.8 +/- 5.0 weeks. We performed follow-up longitudinal and transverse high resolution sonographic scans including routine examination of the contralateral jugular vein at a mean age of 3.7 +/- 1.5 years. In group A thrombotic alterations were detected in 8 aut of 24 patients. In three of these patients we found mild clinical symptoms. In group B thrombotic alterations were detected in 1 aut of 16 patients without clinical symptoms. Mean birthweight (1815 versus 3313 g) and mean gestational age (32.3 versus 38 weeks) were significantly lower and indwelling time of the catheters (18 versus 11 days) was significantly longer in group A. Our results indicate that jugular vein thrombosis is a frequent long-term complication in neonates after jugular vein catheterization. High resolution ultrasonography is an adequate method for detecting jugular vein thrombosis and should therefore routinely be performed for long-term follow-up.

Thiaproline reduces glomerular basement membrane thickness and collagen accumulation in the db/db mouse.

Glomerular basement membrane thickening and mesangial expansion are the main pathological features in diabetic nephropathy--glomerulosclerosis with the biochemical correlate of increased collagen accumulation. We studied a new principle to reduce collagen accumulation in the glomerulus: thiaproline, known to inhibit protein synthesis by blocking the elongation, led in our studies to a morphological reduction of the glomerular basement membrane thickening and to a decreased collagen content. As the thiaproline analogue is incorporated into collagen, the mechanism of increased degradation of the modified collagen is being discussed.

Recent evaluation of prognostic risk factors in esophageal atresia--a multicenter review of 223 cases.

In this study, 223 cases of esophageal atresia (Type IIIb: 85.7%; Type II: 5.8%; Type IIIc: 4.0%; Type IIIa: 2.2%; Type IV: 2.2%) from 6 pediatric surgery centers of Austria, were retrospectively examined for the following parameters and their influence on the prognosis: Birth weight (2494.7 +/- 702.0 g), gestation week (range 27-42 weeks; mean 37.3 +/- 3.1 weeks), sex (male: n = 128; female: n = 95), long-gap atresia (> or = 2 cm: n = 33), Tracheomalacia (n = 16), associated malformations (n = 122; cardiac 27.4%, renal 17.9%, skeletal 17.0%, anal: 10.3%, intestinal 9.9%, mediastinal 7.6%, chromosomal 2.2%), preoperative aspiration (n = 92), pneumonia (n = 96), anastomotic insufficiency (n = 45), empyema (n = 5), mediastinitis (n = 8), sepsis (n = 32), other medical complications (n = 122, in 80 infants), other surgical complications (n = 57). The mortality rate was 41.3% overall, from 1975 to 1991; however, it was 25% from 1987 to 1991 and 0% in 1991. A statistically significant correlation was found between prognosis and the following factors: Cardiac malformations (p = 0.0001), medical complications except aspiration and pneumonia (p = 0.0001), empyema (p = 0.0081), mediastinitis (p = 0.0214), and sepsis (p = 0.0295). These 5 significant factors were given different points and a prognostic score was calculated by the addition of these points. This score was predictive for survival in 90.6% of cases and for mortality in 94% of cases.(ABSTRACT TRUNCATED AT 250 WORDS)

Mechanical bridge to transplantation with the Vienna heart in TAH and LVAD configuration.

The Vienna heart uses a vacuum formed, pellethane pulsatile ventricle and is available in left ventricular assist (LVAD) and total artificial heart (TAH) configurations. This device was used as mechanical support of the failing heart in nine patients intended for heart transplantation. In two patients with cardiomyopathy an orthotopic TAH was implanted; one survived despite severe preoperative ischemic liver damage, and the other died of sepsis. In seven patients an atrio-aortic LVAD was implanted; six had suffered an acute myocardial infarction with cardiogenic shock, and one could not be weaned off bypass. Three patients survived. These included one 65-year-old with incipient ARDS at operation, and a 40-year-old with preoperative liver and kidney insufficiency who was transplanted in septicemia. In this patient the septic focus, natural and artificial heart, were removed at transplantation. Four patients died. In one we were unable to establish satisfactory circulation, one died after failure of the transplanted heart, one suffered a lethal cerebral embolism and one developed multi-organ failure after repeated attacks of ventricular fibrillation. With the Vienna heart sufficient circulatory support could be established with cardiac outputs between 6 and 8 l/min for the TAH and 3.5 to 4.5 l/min for the LVAD. With this type of support an overall survival rate of 44% could be achieved. Mechanical hemolysis was not a clinical problem and no device failure occurred.

Influence of valve surgery on female fertility.

Pregnancies following cardiac valve surgery represent a considerable maternal and fetal risk. The present paper is a report of 163 women in child-bearing age who underwent cardiac valve surgery. Congenital (14.7%) and acquired (85.3%) cardiac defects required a total of 72 alloplastic and 16 bioprosthetic valve implantations. In 69 patients, purely instrumental correction was performed, in 6 patients conduits were placed. Thirty-four pregnancies were observed and led to the delivery of 18 healthy infants. The birth weight was always in the 2-sigma normal range or slightly above. Three cesarean sections were performed due to the cardiac defect, in 6 patients vacuum extraction or forceps delivery was done, and in 9 patients delivery was spontaneous. We observed 4 cases of spontaneous abortion, and in 12 cases interruption of pregnancy. 75.1% women never became pregnant. Potential risks in terms of endocarditis, repeated valve implantation and anticoagulation are emphasized, methods to decrease the physical strain during delivery are discussed.

[Significance of surgically corrected heart valve diseases on fertility]. / Aspekte zur Bedeutung operativ korrigierter Herzklappenvitien auf die Fertilität.

We report on 111 patients, who were operated for heart valve failure. The influence of the operation on fertility was examined. Only 24 women became pregnant. From 34 pregnancies, 4 resulted in early abortion; in 12 cases abortion was performed (8 times for medical reasons), therefore 18 children were born. The remaining 87 operated patients (78.4%) never became pregnant, although only 29 patients were advised against pregnancy. 27 patients decided on various contraceptive methods by themselves.