A Successful Treatment Approach in 2 Patients With Type 1 Plasminogen Deficiency: Intratracheal Application of Fresh Frozen Plasma and Tissue Plasminogen Activator.

BACKGROUND: Respiratory system involvement is common in congenital plasminogen deficiency. Although many treatment approaches have been tried, there is still no definitive treatment for respiratory system involvement. OBSERVATIONS: We report 2 congenital plasminogen deficiency cases, who presented with severe respiratory symptoms, for whom a novel treatment modality was tried. After intravenous administration of FFP (fresh frozen plasma), tissue plasminogen activator and FFP were administered intratracheally, and respiratory system findings improved. CONCLUSIONS: Intratracheal administration of tissue plasminogen activator and FFP is an available treatment modality for patients with lung involvement. Fibrin plaques should be carefully removed and new lesion formation should be prevented.

Ultrasound-guided hydrostatic enema reduction of intussusception and confirmation with single abdominal radiograph in children.

PURPOSE: To detect false-positive reduction results after ultrasound (US)-guided hydrostatic intussusception reduction, we have incorporated water-soluble contrast material to the enema fluid and confirmed the reduction with a single abdominal radiograph. We present the results of the combined imaging method for the reduction of intussusception in children. MATERIALS AND METHODS: The records of the patients who were treated for intussusception were analyzed retrospectively. Patients were divided into two groups: a US-guided reduction group and a US-guided reduction plus radiographic control group. The patient characteristics, symptoms, treatment methods, outcomes, and complications and follow-up were analyzed. RESULTS: A total of 164 intussusception episodes were treated in 153 patients. Hydrostatic reduction of intussusception was performed in 59 patients in the US-guided group and in 94 patients in the US-guided plus radiographic control group. Recurrence rate in the US-guided group was 15.7%, vs 3.5% in the US-guided plus radiography group (P = .029). In the US-guided plus radiographic control group, 5 (5.3%) false positive reductions under US guidance were determined by abdominal radiography. CONCLUSION: In order to decrease false positive reduction rate and early recurrence, US-guided intussusception reduction can be performed with saline plus water-soluble contrast material and confirmation of reduction obtained with a single direct abdominal radiograph.

Lung density analysis using quantitative computed tomography in children with pectus excavatum.

PURPOSE: To evaluate the mean lung density in children with pectus excavatum (PE) and to assess the correlation between the cardiac rotation angle, Haller index, pulmonary function test, and lung density. MATERIAL AND METHODS: This retrospective study included 33 children with PE and 31 healthy controls. The densities of lung parenchyma were evaluated by quantitative computed tomography (CT). Three lung levels were determined: T4 vertebra level, T10 vertebra level, and the level of the measurement of the cardiac rotation angle. The cardiac rotation angle and the Haller index were calculated. All measurements were done by 2 radiologists, independently. Student's t-test or the Mann-Whitney U test, intraclass correlation coefficients, Pearson or Spearman's rank correlation coefficient, and Kruskal-Wallis test were used for statistical analysis. A p-value less than 0.05 was considered as statistically significant. RESULTS: All the lung levels in the PE group had lower mean densities than healthy children, with statistical significance in the right lung at the T10 vertebra level (-818.60 ± 33.49 HU, -798.45 ± 40.24 HU; p = 0.028). There was a correlation between the cardiac rotation angle and the Haller index (r = 0.593; p < 0.001). There were no correlations between mean lung density and cardiac rotation angle, Haller index, and pulmonary function tests. CONCLUSIONS: The lower mean lung densities were found in PE, especially in the right lower lobe. The parenchymal aeration should be considered independently from the severity of PE.

The Density of Interstitial Cells of Cajal Is Diminished in Choledochal Cysts.

BACKGROUND AND AIMS: Interstitial cells of Cajal (ICC) have been shown to be present in the extrahepatic biliary tract of animals and humans. However, ICC distribution in choledochal cysts (CC) has not been investigated. A study was conducted to investigate the distribution of ICC in the extrahepatic biliary tract, including CC, in pediatric human specimens. METHOD: The specimens were divided into two main groups as gallbladders and common bile ducts. Gallbladders were obtained from the cholelithiasis, CC operations and autopsies. Common bile ducts were obtained from autopsies. Tissues were stained using c-kit immunohistochemical staining. ICC were assessed semi-quantitatively by applying morphological criteria and were counted as the number of cells/0.24 mm(2) in each area under light microscopy. RESULTS: A total of 35 gallbladders and 14 CC were obtained from operations. Ten gallbladders plus common bile ducts were obtained from autopsies. The mean numbers of ICC in the gallbladders of cholelithiasis and the gallbladders of CC were 12.2 ± 4.9 and 5.3 ± 1.2, respectively (p = 0.003). The mean numbers of ICC in the common bile ducts and CC were 9.8 ± 2.9 and 3.4 ± 1.4, respectively (p = 0.001). CONCLUSION: The scarcity of ICC in the extrahepatic biliary tract may be responsible for the etiopathogenesis of the CC.

Conventional single-port laparoscopic appendectomy for complicated appendicitis in children: Efficient and cost-effective.

BACKGROUND: Laparoscopic appendectomy (LA) is gradually gaining popularity among paediatric surgeons for complicated appendicitis. A retrospective study was conducted to compare conventional single port LA, multiport LA and open appendectomy (OA) for complicated appendicitis in children. PATIENTS AND METHODS: From January 1995 from December 2014, 1,408 patients (604 girls, 804 boys) underwent surgery for uncomplicated and complicated appendicitis. The patient characteristics, operation times, duration of hospitalization, operative costs, and postoperative complications were recorded. A 10-mm 0(°) scope with a parallel eye piece and an integrated 6 mm working channel were inserted through an 11-mm "conventional umbilical port" for single port LA. RESULTS: A total of 314 patients with complicated appendicitis (128 girls, 186 boys) underwent appendectomy. Among these, 102 patients (32.4%) underwent single port LA, 17 patients (5.4%) underwent multiport LA and 195 patients (62.1%) underwent OA. The hospital stay of the single port LA group was significantly less (3.88 ± 1.1) compared with multiport LA (5.41 ± 1.2) and OA groups (6.14 ± 1.1) (P < 0.001). Drain usage, wound infection and adhesive intestinal obstruction rates were significantly high in the OA group. There was no significant difference between the groups in postoperative intraabdominal abscess formation. Single-port LA performed for complicated appendicitis was cheaper compared with the other groups. CONCLUSIONS: The present study has shown that single-port LA for complicated appendicitis can be conducted in a reasonable operative time; it shortens the hospitalization period, markedly reduces postoperative wound infection and adhesive intestinal obstruction rates and does not increase the operative cost.

Developing the pediatric surgery patient satisfaction scale and assessment of its validity and reliability.

Aim of this study was to develop a patient satisfaction scale for needs and expectations of pediatric surgery patients and to propose a new measurement tool in this field. Population of study consisted of all patients between May 2018 and February 2020 at a pediatric surgery service of a university hospital in Turkey. A pool of 70 items was prepared for scale. Two items were removed in line with expert opinions and suggestions. As a result of content validity and test application, 36 items were removed, and scale was revised. Data were transferred to SPSS Statistics 23 and AMOS 22 program. After evaluating scope validity of scale, Content Validity, Structural Validity, Exploratory Factor Analysis, and finally Reliability Analysis were examined. As a result of the analyses, 32 items with eight sub-dimensions were obtained from scale. Eight-factor scale explained 60.42% of total variance. Cronbach Alpha internal consistency of scale was found to be 0.88. Item factor loads of scale were created and the reliability of scale were obtained at desired level. The scale is suitable for patients aged 6 to 18 years old.

Clues for the early loss of renal function in congenital hydronephrosis: Analysis of renal pelvis collagen ratio, diuresis renography and upper urinary tract morphology.

INTRODUCTION: Diagnosing real urinary obstruction and surgical decision making for the operative correction of urinary obstruction, are still problematic in congenital hydronephrosis (CH). Compliance of the renal pelvis is one of the important defense mechanisms of renal parenchyma against urinary obstruction. We observed early loss of function in some cases of CH with low and moderate anteroposterior diameter of the renal pelvis (APDRP). OBJECTIVE: To evaluate structural properties of the renal pelvic tissue of patients with CH and the relation of these structural properties with renal function and Anteroposterior diameter of the renal pelvis. STUDY DESIGN: Ureteropelvic junction (UPJ) excised during UPJ obstruction operations from 2013 to 2019 were evaluated histopathologically. The patients were divided into the two groups according to initial renal function, i.e. group with initial preoperative differential renal function (DRF) less than 35% and group with initial DRF greater than 35%. The percentage of collagen to whole tissue area were analyzed using image processing program. The relationship between DRF and tissue collagen ratio, SFU and APDRP was evaluated. RESULTS: There were 5 patients in the DRF <35% group and 16 patients in the DRF >35% group. However, APDRP's of the DRF <35% group were also significantly lower than the DRF >35% group. The collagen distribution in the muscle layer was more prominent in the DRF <35% group. Proportionally, percentage of collagen stained surface was significantly higher in DRF <35% group. DISCUSSION: There are numerous histopathological studies evaluating the cause of UPJ obstruction. Besides these studies that are oriented to etiology, there are many studies comparing the histopathological changes at UPJ with surgical outcome and prognosis. In the present study, we found that renal pelvis collagen ratio was significantly increased in patients with lower APDRP and with severe renal function loss. This increase in the collagen content in the renal pelvis have been shown to affect the compliance negatively and decrease APDRP, which leads to a faster loss of renal function. Thus, pelvic structural changes accompanying UPJ obstruction may aggravate urinary obstructive process. CONCLUSION: Increased renal pelvis collagen ratio negatively affects the expansion of the renal pelvis, which is one of the protective mechanisms of the renal parenchyma, and may be one of the triggering mechanisms of early loss of renal function.

Addition of Transfixation Suture to Purse String Suture During Intraperitoneal Inguinal Hernia Repair Increases Peri-Hernia Sac Neck Collagen Formation.

BACKGROUND: The worldwide accepted repair for indirect inguinal hernia in children is high ligation of the hernia sac with open herniotomy. However, laparoscopic pediatric inguinal hernia repair (IHR) has been gaining popularity in the last two decades. An experimental study was conducted to investigate the effects of different intraperitoneal IHR suture techniques on the collagen formation at the hernia sac neck. METHODS: Present study was conducted on thirty-five male adult (3-6 months old) Wistar-Albino rats (260-300 g). Intraperitoneal IHR with different hernia sac neck suturing techniques (purse string suture only, transfixation suture only and purse string suture plus transfixation suture) were performed through median laparotomy using open operative techniques. Non-absorbable 2/0 braided polyester suture with 16 mm 1/2 curved round needle (Ti-cron, Covidien, MN) was used as suture material. RESULTS: The highest collagen thickness around the suture was detected in intraperitoneal IHR with purse-string plus transfixation suture group. The collagen thickness of the intraperitoneal IHR with purse string suture only and IHR with tranfixation suture only groups were not statistically significantly different. The collagen thickness of the intraperitoneal IHR with purse string suture plus transfixation suture group was statistically significantly higher compared with the intraperitoneal IHR with purse string suture only and intraperitoneal IHR with transfixation suture only groups. CONCLUSIONS: The combined usage of purse string suture and transfixation suture during laparoscopic intraperitoneal inguinal hernia repair further stimulates mesothelial fibrosis at the hernia sac neck compared with mesothelial fibrosis induced by purse string suture only or transfixation suture only.

Urinary continence after high urogenital sinus repair conducted with posterior prone approach: electromyography-uroflowmetric assessment.

PURPOSE: We aimed to present the results of urinary continence assessment objectively with electromyography (EMG)-uroflowmetry after high urogenital sinus (HUGS) repair with posterior prone approach without division of rectum. METHODS: The records of patients who underwent HUGS repair via posterior prone approach between January 2005 and July 2018 were reviewed retrospectively. Incontinence, dysuria, hesitation, and straining during urination were evaluated during the clinical follow-up. Dysfunctional voiding scoring system was used as a questionnaire. Patients were re-evaluated with EMG-uroflowmetry in terms of voiding volume and pattern, voiding time, maximum flow rate, average flow rate, maximum flow time, and post-voiding residual volume. RESULTS: Seven patients with HUGS were treated with a posterior prone approach. The median age of the patients was 18 months (8-21 months). The median UGS length was 4.4 cm (3.6-5.5 cm), urethral length was 1.1 cm (1.0-1.5 cm), and vaginal length was 4.9 cm (4.1-5.1 cm). No urination or defecation problems were described by the patients or their parents. When the results of the dysfunctional voiding scoring systems questionnaire were analyzed, results scored 7 (range 5-8). EMG-uroflowmetric test graphics of the patients showed normal flow curves without plateau, intermittency or irregularity. Pelvic EMG assessment was normal in all patients. CONCLUSION: EMG-uroflowmetry has shown objectively that urinary continence and normal voiding pattern are preserved after HUGS repair with posterior prone approach without division of rectum.

Successful outcomes in adolescent varicocele treatment with high-level laparoscopic varicocelectomy.

PURPOSE: In this study, we aimed to compare the effects of testicular vein ligation level on complications encountered; i.e. high-level ligation cranial to the linea terminalis vs ligation caudal to the linea terminalis. METHODS: A total of 47 unilateral adolescent patients, treated with laparoscopic varicocelectomy between January 2004 and December 2017, were reviewed retrospectively. Patients were divided into two groups in terms of ligation level: caudal to the linea terminalis as group 1 and cranial to the linea terminalis as group 2. Symptoms, varicocele grades, preoperative testicular growth arrest, operative method, hydrocele formation, postoperative recurrence and testicular catch-up growth were recorded. RESULTS: The mean operation time was 38.6 ±â€¯10.2 min (34-53 min) in group 1 and was 33.6 ±â€¯6.4 min (29-42 min) in group 2. Single hydrocele occurred in the laparoscopic nonselective varicocelectomy in group 1 (4.5%) and was successfully treated with open hydrocelectomy. Single varicocele recurrence was observed in the laparoscopic selective varicocelectomy in group 1 (4.5%) and treated with laparoscopic nonselective varicocelectomy cranial to the linea terminalis. CONCLUSIONS: The high-level ligation of the spermatic veins cranial to the linea terminalis during laparoscopic varicocelectomy, independent of the technique applied, may contribute to reasonable low hydrocele and recurrence rates. LEVEL OF EVIDENCE: Level III.

Laparoscopic Excision of the Retroperitoneal Mullerian Cyst in a Different Location.

BACKGROUND: Herein, we report laparoscopic excision of a retroperitoneal Mullerian cyst, which is uniquely located medial to the left adrenal gland, mimicking adrenal adenoma in an adolescent girl. CASE: A healthy 13-year-old adolescent girl presented with secondary amenorrhea. Ultrasound and magnetic resonance imaging showed a solid-cystic mass medial to the left adrenal gland. Laparoscopic cyst excision was performed. The histopathological findings were consistent with a benign cyst of Mullerian origin. SUMMARY AND CONCLUSION: To the best of our knowledge, this is the first case report of Mullerian cyst located medial to the adrenal gland in all age groups.

Late presentation of cystic adenomatoid malformation type 4 of lung in an asymptomatic child.

Congenital cystic adenomatoid malformation of lung is an uncommon embryonic developmental anomaly usually diagnosed perinatally. Rarely the presentation is delayed until childhood and adulthood. Herein we present a 10-years-old girl with a late presentation of congenital cystic adenomatoid malformation type 4 of lung who was diagnosed coincidentally.

A rare presentation of hydatid cyst in a child.

Cyst hydatid is the most widespread, serious cestode infection in the world. The most common organ affected by hydatid disease is the liver followed by the lungs and the two organs are affected simultaneously in about 5-13% of cases. The involvement of kidneys are rarely reported. Here we reported a patient with hydatid cysts in lung and kidney which the surgery was effective for both organs. The rarity of this case is the unusual combination of the cyst development in these organs without the involvement of liver.

The role of water-soluble meconium subfraction and lipid-soluble meconium subfraction on the superior mesenteric artery vasoconstriction in chick embryos.

OBJECTIVE: Intraamniotic meconium has been responsible for intestinal damage in gastroschisis and meconium-dependent intestinal ischemia has been proposed to induce additional intestinal damage in gastroschisis. This study is aimed to determine the effects of lipid and water-soluble meconium subfractions on the contractility of the superior mesenteric artery (SMA). MATERIALS AND METHODS: The study was conducted on 18-day fertilized chick embryos (Gallus Domesticus). Meconium is fractioned into water and lipid-soluble components. Only one SMA tissue was prepared from each embryo and suspended in the organ bath. Isometric contraction responses (ICR) were created in SMA tissues by one hour of incubation in Krebs-Henseleit solution for each group. Groups consisted of control, meconium, water-soluble meconium subfraction and lipid-soluble meconium subfraction. ICR of the SMA specimens were evaluated with a transducer-amplifier system on a computer. The data were expressed (mean±1SD) as milliNewton (mN). RESULTS: The ICR of the meconium, water-soluble meconium subfraction and lipid-soluble meconium subfraction groups were significantly high when compared to the control group (p<0.01). The meconium and water-soluble meconium subfraction created more contraction response than the lipid-soluble meconium subfraction (p<0.01). The ICR of the meconium group was not different from the ICR of the water-soluble meconium subfraction group (p>0.05). CONCLUSION: Water-soluble meconium subfraction has a profound vasoconstrictor effect on the SMA compared to the lipid-soluble meconium subfraction.

The etiology of tracheal mucosal injury in proximal esophageal atresia plus distal tracheoesophageal fistula: an animal study.

Tracheal mucosal damage has been reported in autopsy specimens of cases with proximal esophageal atresia and distal tracheoesophageal fistula (EA-TEF) (Gross classification type C). Such changes have not been reported for isolated EA (Gross classification type A). Our hypothesis is that passage of amniotic fluid (AF) through fistula via larynx and trachea may damage tracheal mucosa. An experimental study was conducted to investigate this hypothesis in the Adriamycin-induced EA-TEF model. In the first stage of the study, we tested whether the fetuses with EA-TEF associated with pyloric atresia cannot swallow AF whereas the fetuses EA-TEF without intestinal atresia can swallow AF. Carbon solution was injected into the AF for this purpose. In the second stage of study, at the 21st day of their gestation, fetuses were extirpated and dissected under microscope. In both stages, fetuses were divided into four groups as control, fetuses without tracheoesophageal abnormalities, fetuses with EA-TEF only, fetuses with pyloric atresia associated with EA-TEF. Lungs and tracheas of the all fetuses were removed for histopathological examination. While carbon particles were present in the trachea, stomachs of the fetuses without tracheoesophageal anomalies, with EA-TEF only and control fetuses, carbon particles were absent in both trachea and stomachs of the fetuses with pyloric atresia associated with EA-TEF. Histopatological examination of the tracheal mucosa showed damage throughout the trachea in the fetuses with EA-TEF only group. Tracheal mucosa was found to be normal in other groups. Bronchial mucosa and lung tissues were found to be normal in all groups. Amniotic fluid swallowed through the TEF causes histopathological changes in the tracheal mucosa of the fetuses with EA-TEF only group. These findings may also contribute to the development of new fetal treatment modalities.

Complex urogenital malformation associated with female pseudohermaphroditism: caudal dysgenesis syndrome.

Caudal dysgenesis syndrome is a rare cause of female pseudohermaphroditism. This syndrome consists of absent perineal and anal openings in association with ambiguous genitalia, urogenital, colonic, and lumbosacral anomalies. We report a case of caudal dysgenesis syndrome in an infant who had non-palpable testes, bifid scrotum, a phallus-like structure and urethral atresia. Radiological evaluation revealed bilateral hydronephrosis, bifid uterus, cervix and vagina. Caudal dysgenesis syndrome should be considered in any female infant presenting with bilateral streak ovaries, and Müllerian and genito-urinary anomalies.

The intestinal damage induced by lipid soluble meconium subfraction is profound compared to the intestinal damage induced by water soluble meconium subfraction.

BACKGROUND: It is unclear which substances in meconium are responsible for intestinal damage in gastroschisis. An experimental study was designed to investigate the effects of the lipid or water soluble subfractions of meconium on the intestines of gastroschisis in a chick model. METHODS: Meconium was pooled, homogenated, rota-evaporated dry and diluted. Meconium subfractions were obtained from water soluble and lipid soluble extracts of the meconium. Five days old fertilized chick embryos were used and divided into 5 groups: control, sham, water soluble meconium subfraction, lipid soluble meconium subfraction and whole meconium. All embryos were extirpated on the 18days and the intestines were harvested for histopathological examination. Serosal thickness was measured under light microscopy. RESULTS: Serosal thickness of the meconium (36.36±2.8µm), the water soluble meconium (14.15±0.93µm) and the lipid soluble meconium (23.88±1.69µm) subfractions groups were significantly increased compared with the control (7.47±0.68µm) and the sham (7.48±0.71µm) groups (p<0.001). Serosal thickness of the lipid soluble meconium subfraction group was significantly increased compared with the water soluble meconium subfraction group (p<0.001). Serosal thickness of the meconium group was significantly increased compared to both the water and the lipid soluble meconium subfraction groups (p<0.001). CONCLUSION: Lipid soluble meconium subfraction induces more intestinal damage compared to water soluble meconium subfraction.

Nuss procedure: Technical modifications to ease bending of the support bar and lateral stabilizer placement.

BACKGROUND: Modifications defined to ease bending of the support bar and lateral stabilizer placement during minimal invasive repair of pectus excavatum (MIRPE) have not been reported. We herein report our experience with MIRPE including several technical modifications. METHODS: A total of 87 patients who underwent MIRPE were evaluated retrospectively. Technical modifications are (1) a template drawn preoperatively according to the anthropometric measurements, (2) more laterally placed thoracal incisions, (3) single existing incision for multiple support bars, (4) to secure lateral stabilizers to support bar in inverted position. RESULTS: The mean patient age was 11.2 ± 3.8 years. The mean operating time was 63.7 ± 18.7 min. The mean Haller index was 5.4 ± 2.1. Eight patients necessitated two support bars. The support bars were removed in 69 patients after the completion of treatment. Support bars were left in place 26.8 ± 4.3 months. Final chest contours of the 56 patients were evaluated as 12 months passed after support bar removal and excellent repair results were determined in 84.2%. CONCLUSION: Preoperative bending of the support bar according to anthropometric measurements and fixation of the lateral stabilizers to the support bar in inverted position facilitates bar shaping and lateral stabilizer placement.