Five Years Follow-up of Opsoclonus-Myoclonus-Ataxia Syndrome-Associated Neurogenic Tumors in Children.

AIM: Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare autoimmune disorder. Approximately half of the cases are associated with neuroblastoma in children. This study's aim is to review management of our cases with OMAS-associated neuroblastoma for treatment approach as well as long-term follow-up. METHODS: Age at onset of symptoms and tumor diagnosis, tumor location, histopathology, stage, chemotherapy, OMAS protocol, surgery, and follow-up period were evaluated retrospectively in six patients between 2007 and 2022. RESULTS: Mean age of onset of OMAS findings was 13.5 months and mean age at tumor diagnosis was 15.1 months. Tumor was located at thorax in three patients and surrenal in others. Four patients underwent primary surgery. Histopathological diagnosis was ganglioneuroblastoma in three, neuroblastoma in two, and undifferentiated neuroblastoma in one. One patient was considered as stage 1 and rest of them as stage 2. Chemotherapy was provided in five cases. The OMAS protocol was applied to five patients. Our protocol is intravenous immunoglobulin (IVIG) 1 g/kg/d for 2 consecutive days once a month and dexamethasone for 5 days (20 mg/m2/d for 1-2 days, 10 mg/m2/d for 3-4 days, and 5 mg/m2/d for the fifth day) once a month, alternatively by 2-week intervals. Patients were followed up for a mean of 8.1 years. Neuropsychiatric sequelae were detected in two patients. CONCLUSION: In tumor-related cases, alternating use of corticosteroid and IVIG for suppression of autoimmunity as the OMAS protocol, total excision of the tumor as soon as possible, and chemotherapeutics in selected patients seem to be related to resolution of acute problems, long-term sequelae, and severity.

Treatment of young patients with pilonidal sinus disease with the original (unmodified) Limberg flap standardized for the first time.

BACKGROUND: Pilonidal sinus disease (PSD) is commonly seen in young adults but may also affect adolescents. Our goal was to present results from operated patients, with a focus on the original Limberg flap, which we standardized for the first time. METHODS: This study was a retrospective review of 60 teenage patients who underwent surgery in a single pediatric surgery center over approximately 15 years. RESULTS: Of the 60 patients, 33 (55%) were male and the mean age was 14.20 ± 1.31 (9-17) years. After pilonidal sinus excision, primary repair and Limberg flap were performed in 45 (75%) and 15 (25%) patients respectively. There were no complications in the patients who underwent the Limberg flap technique, whereas 49% of the patients who underwent primary repair exhibited complications. The mean follow-up was 6.7 ± 4.8 years. CONCLUSION: We standardized the Limberg flap for the first time in children with measurements and consider that it meets expectations: it is not difficult to perform, it has low complication and recurrence rates, it requires an acceptable period to return to normal activities, and gives satisfactory cosmetic results. We did not identify any complications in patients treated with the Limberg flap excision and post-operative epilation. We consider that findings achieved by the treatment of original, standardized Limberg flap excision is comparable with all of the treatment methods reported in the literature related with children.

Management of traumatic bile duct injuries in children.

PURPOSE: Pediatric experience with biliary tract injuries (BTI) is limited and mostly consists of case presentations. The purpose of this study is to evaluate clinical and radiological findings of possible BTI, treatment strategies, and results. METHODS: The records of nine patients with the diagnosis of BTI between July 2009 and November 2017 were reviewed retrospectively. RESULTS: There were seven boys and two girls (mean 8.05 ± 4.39 years). The mechanisms were motor vehicle occupant, fall, crush and gunshot wound. Hepatic laceration routes that extended into the porta hepatis and contracted the gall bladder were demonstrated on computerized tomography (CT). Bile duct injury was diagnosed with bile leakage from the thoracic tube (n = 2), from the abdominal drain (n = 2) and by paracentesis (n = 5). Extrahepatic (n = 8) and intrahepatic (n = 1) bile duct injuries were diagnosed by cholangiography. Endoscopic retrograde cholangiography, sphincterotomy, and stent placement were successfully completed in five patients. Peritoneal drainage stopped after 3-17 days of procedure in four patients. The fifth patient was operated with the diagnosis of cystic duct avulsion. Cholecystectomies, primary repair of laceration, cystic duct ligation, and Roux-en-Y hepatoportoenterostomy were performed in the remaining four patients. All patients presented with clinically normal findings, normal liver functions, and normal ultrasonographic findings in the follow-up period. CONCLUSIONS: The presentation of the parenchymal injury extending to the porta hepatis with contracted gall bladder on CT and diffuse homogenous abdominal fluid should be considered as signs of BTI. We suggest a multi-disciplinary approach for the diagnosis and treatment of BTIs. Surgery may be indicated according to the patient's clinical condition, radiological findings and failure of non-operative treatment.

Intestinal malrotation needs immediate consideration and investigation.

BACKGROUND: The aim of this study was to evaluate clinical presentation, diagnostic studies, and volvulus rate and to describe the unusual clinical clues of intestinal malrotation. METHODS: A retrospective descriptive review was carried out of all patients diagnosed with intestinal malrotation between 2002 and 2014. Patients were divided into two groups: infants (≤1 year, n = 16; group 1); and children (>1 year, n = 12; group 2). Patient demographics, clinical history, symptoms, physical examination, diagnostic work-up, operative findings and early outcome were evaluated. RESULTS: Bilious vomiting was the cardinal complaint in both groups. Unusual symptoms such as respiratory insufficiency, dehydration, afebrile convulsion, and lethargy were prominent symptoms in six patients in group 1, whereas history of frequent hospitalization due to recurrent abdominal pain and feeding intolerance were prominent in six patients in group 2. Midgut volvulus was identified in 15 patients, four of whom were in group 2. Standard Ladd's procedure was done in addition to correction of volvulus. CONCLUSION: Malrotation with or without midgut volvulus is not a rare condition and should be kept in mind for any age group. Specific signs of diagnosis are not easily identified. In the case of unusual clinical presentation, diagnosis may be delayed and can result in catastrophic consequences if intestinal perfusion occurs. Although midgut volvulus is seen most frequently in infants, risk and complication rate are high beyond 1 year of age as well, and can manifest as failure to thrive, food intolerance, and abdominal pain needing recurrent hospitalization. Diagnostic suspicion and interdisciplinary coordination are essential for timely diagnosis and surgical treatment.

Mortality Factors in Crush Syndrome.

BACKGROUND: Crush Syndrome is a major cause of morbidity and mortality following large-scale catastrophic earthquakes. Since there are no randomized controlled studies on Crush Syndrome, knowledge on this subject is limited to expert experience. The primary objective is to analyze the epidemiological and demographic characteristics, clinical outcomes, and mortality factors of earthquake victims after the Pazarcik and Elbistan earthquakes on February 6, 2023. METHODS: This cross-sectional and observational retrospective study evaluated 610 earthquake victims who presented to our center between February 6 and April 30, 2023. Among these patients, 128 with Crush Syndrome were included in the study. Patient information was gathered from hospital records during their stay and from national registries upon referral. The primary outcome was to identify risk factors for mortality. Demographic and laboratory data were analyzed by acute kidney injury (AKI) stages; mortality-affecting factors were identified through regression analysis. RESULTS: Of the 128 Crush Syndrome patients (100 adults, 28 children), 64 were female. The AKI rate was 32.8%. Among patients with AKI, the frequency of hemodialysis requirement was 69%, and the mortality rate was 14.2%. The overall mortality rate for patients with Crush Syndrome was 4.6%, compared to 3.9% (19/482) in earthquake victims without Crush Syndrome (p=0.705). Notably, low systolic blood pressure at admission was the only factor significantly affecting mortality in Crush Syndrome patients (Hazard Ratio [HR]: 1.088, p=0.021, 95% Confidence Interval [CI]). CONCLUSION: Our study highlights low systolic blood pressure upon admission as a significant risk factor for increased mortality in Crush Syndrome patients. This finding may contribute to the literature by emphasizing the importance of monitoring blood pressure under rubble and administering more aggressive fluid therapy to patients with low systolic blood pressure.

Management of pyloric stricture in children: endoscopic balloon dilatation and surgery.

BACKGROUND: Surgical correction is the most preferred treatment modality in pyloric stricture (PS). Recently a few studies reported the experience of balloon dilation in children with PS. This study was designed to present our experiences of the management of the patients with PS with balloon dilation and corrective surgery. METHODS: The records of 14 patients who were treated with the diagnosis of PS between August 2003 and August 2011 were reviewed retrospectively. RESULTS: There were nine boys and five girls (mean age, 3.4 ± 1.7 years). The history of caustic ingestion was detected in eight patients; six of them were admitted on the day of ingestion. Two patients were admitted with nonbilious vomiting more than 2 weeks after ingestion. Four patients did not have a remarkable medical history, including caustic ingestion. They admitted with the complaint of nonbilious vomiting. PS was detected during endoscopy in two patients who had a diagnosis of peptic ulcer disease. PS was shown by barium meal study in all patients. Endoscopy was performed in all patients. Endoscopic balloon dilation was tried in 12 patients. Overall eight patients required surgical procedures for PS. The complaints were resolved by endoscopic balloon dilation of pylorus in the remaining six patients. CONCLUSIONS: Although endoscopic balloon dilatation for benign PS in adults is a generally accepted method of treatment, there is less experience with endoscopic balloon dilatation for PS in children. PS due to benign disorders can be effectively and successfully treated through endoscopic balloon dilatation in suitable patients. In patients with successful pyloric balloon dilatation, surgery can be avoided.

Ingested foreign bodies in children: Do they really pass spontaneously from the gastrointestinal tract? A single-centre experience with 1000 cases.

BACKGROUND: Foreign body (FB) ingestion is frequently encountered in all departments that treat children. FB may bring about significant anxiety for parents and physicians. The present study aims to determine the appropriate approach for FB ingestion in children. METHODS: The records of 1000 children with a history of FB ingestion between the years 2005 and 2017 were reviewed retrospectively in this study. RESULTS: Of 1000 children, 53.8% were male. The most common types of FBs were coins (35%). X-ray was negative in 49% of the patients, and 86% of these patients received no intervention. Of the 504 (51%) X-ray-positive patients, the oesophagus (68%) was the most common location. Life-threatening complications were tracheo-oesophageal fistula (1), Meckel's diverticulum perforation (1), and perforation due to rigid endoscopy (1). CONCLUSION: We demonstrated that coins, which are the most commonly ingested FBs, have various types and sizes according to their countries of origin, and this affects spontaneous passage. We found that only 48% (quite low compared to the literature) of the coins passed spontaneously. In asymptomatic patients with a gastric button battery, we suggest a "watchful waiting" approach. The patients should be observed and managed at home. In our study, we found that 85% of the button batteries that reached the stomach passed spontaneously.

Boix-Ochoa (Partial Fundoplication) Treats Reflux, Even in Neurologically Impaired Patients. Can it Take the Title of "Gold Standard" from Total Fundoplication?

BACKGROUND: In 4-5% of cases of gastroesophageal reflux disease (GERD), surgical treatment is required. The aim of the study was to evaluate the success of Boix-Ochoa antireflux surgery, which is considered more physiologic with a higher failure rate (need for reoperation) than Nissen fundoplication, which is believed to be the gold standard operation. METHOD: In the 13 years from 2005 to 2018, the medical records of all children who underwent Boix-Ochoa in a single institution by pediatric surgeons were reviewed retrospectively. RESULTS: A total of 133 fundoplications were performed, of which patients were divided into four groups: neurologically impaired, structurally impaired, neurologically and structurally impaired, and neurologically and structurally normal; there were 64, 8, 34, and 27 patients in each group, respectively. Structural impairments included hiatal hernia and esophagus atresia, having previously had a gastrostomy and esophageal stenosis. The most common short-term complication was distal esophageal stenosis (13%), which caused vomiting and dysphagia, and was treated by dilatations. There were six (4.5%) recurrences of GER, one in the neurologically and structurally impaired group with a hiatal hernia and five in the structurally impaired group (three esophagus atresias, two caustic esophageal strictures). The mean follow-up period was 5.27 ± 3.43 years. Neurological impairment did not affect the success rate. CONCLUSION: Although there has not any literature demonstrating significant benefits of one procedure, we detected with this largest study in the pediatric literature about Boix-Ochoa fundoplication (more physiologic and easily performed) that it was successful (95%) in protecting reflux even in neurologically impaired patients (98%). We consider Boix-Ochoa (partial fundoplication) to be an alternative method to Nissen (complete fundoplication), and it can be done safely with a high success rate.

Huge Neck Masses Causing Respiratory Distress in Neonates: Two Cases of Congenital Cervical Teratoma.

Congenital cervical teratomas are rare and usually large enough to cause respiratory distress in the neonatal period. We present two cases of congenital huge cystic neck masses in which distinguishing cervical cystic hygroma and congenital cystic teratoma was not possible through radiologic imaging techniques. Experience with the first case, which was initially diagnosed and treated as cystic hygroma by injection sclerotherapy, led to early suspicion and surgery in the second case. The masses were excised completely and histopathologic diagnoses were congenital teratoma in both patients. Our aim is to review congenital huge neck masses causing respiratory distress in early neonatal life to highlight this dilemma briefly with these interesting cases.

Meckel diverticulum in children: Evaluation of macroscopic appearance for guidance in subsequent surgery.

BACKGROUND: The treatment of incidentally encountered asymptomatic Meckel diverticulum (MD) is controversial. We evaluated whether the macroscopic appearance correlates with clinical features, histopathological findings, future complications, and management decisions. METHODS: Patients who underwent MD resection at a single institution from 2000 to 2012 were retrospectively analyzed in terms of age, sex, clinical features, laboratory data, perioperative findings (diverticulum length, diameter, depth, thickening, and height-to diameter ratio [HDR]), pathology, and postoperative follow-up. RESULTS: Fifty children were enrolled. Sixteen percent of the resected MDs were found incidentally. Of 42 complicated MDs, 17 (40%) were long (HDR ≥2), 14 (33%) were thickened, and 29 (70%) exhibited base widening. Histopathologically, ectopic mucosa was found in 32 (64%) of all MDs and in 5 (62%) of incidentally removed MDs. There was no statistical difference between the macroscopic appearance and clinical signs, sex, or presence of ectopic tissue based on palpation. CONCLUSION: The macroscopic appearance of MD does not indicate the presence or absence of HGM and cannot be used to guide subsequent surgery. Additionally, 40% of symptomatic patients in our study had life-threatening complications requiring prompt fluid resuscitation. We consider that incidentally detected MD should be removed regardless of its macroscopic appearance.

Choledochal cysts in children: Intrahepatic ductal dilatation does not indicate true intrahepatic biliary duct disease.

BACKGROUND/AIMS: Choledochal cysts (CCs) are rare abnormalities of the biliary tract. Presenting our clinical experience with CCs herein, we aimed to identify if intrahepatic ductal dilatation indicates true intrahepatic biliary duct disease. MATERIALS AND METHODS: We retrospectively reviewed all cases of CCs in children diagnosed at a single center (Baskent University Fac-ulty of Medicine, Department of Pediatric Surgery) institution from 2005 to 2015. RESULTS: Of 18 patients with CCs, 7 were males (39%). The age range was 3 months to 17 years (mean age, 6.2±3.8 years). Intrahepatic bile duct dilatation was detected in 13 (72%) patients by preoperative scanning. Type I, II, III, and IVA cysts were diagnosed in 13, 1, 1, and 3 patients, respectively. In all patients, total cyst excision and Roux-en-Y hepaticoenterostomy were performed. CONCLUSION: In this study, most intrahepatic ductal dilatations seen on preoperative imaging were thought to be caused by a distal obstruction, not true intrahepatic biliary duct disease. This study supports the hypothesis that preoperatively distinguishing between type I and type IVA CCs is not necessary; it does not affect the initial treatment. We consider that complete cyst excision with Roux-en-Y hepaticojejunostomy is safe and should be performed soon after diagnosis, irrespective of symptom severity to avoid future complications.

Spleen Salvaging Treatment Approaches in Non-parasitic Splenic Cysts in Childhood.

The aim of this study was to evaluate our experience with primary non-parasitic splenic cysts (NPSC) which are relatively rare in children and consist almost exclusively of single case reports or small case series in the literature. The medical records of all patients who presented to our clinic with NPSC between 2005 and 2015 were evaluated retrospectively. There were 22 children whose ages ranged from 2 months to 14 years (mean 9.2 ± 4.7 years). The size of the cysts was in the range of 5 to 200 mm (mean 55.4 ± 48.2 mm). Ten patients underwent surgery for splenic cysts. Partial splenectomy (n = 2), total cyst excision (either open n = 4 or laparoscopically n = 1), and total splenectomy (n = 3) were performed. The non-operated patients were asymptomatic and followed with ultrasound (US). The follow-up period in non-operated patients ranged from 6 months to 5 years (mean 2.27 ± 1.29 years). Complete regression was observed in four (33 %) non-operated patients. The regressed cyst measurements were 10, 16, 30, and 40 mm, respectively. Approximately half of the NPSC is diagnosed incidentally. Small (<5 cm) asymptomatic cysts should be under regular follow-up with US/physical examination for regression. If surgery is required, we prefer open cyst excision as it gives excellent results and preserves splenic immune function.

Aspiration of an interesting foreign body: Myiasis.

Myiasis is a rare condition caused by the invasion of tissues by the larvae of flies. Many cases of myiasis involving various human organs have been reported. Tracheopulmonary or intratracheal myiasis is a very unusual and aberrant form of the disease in humans. We present a case of respiratory myiasis after aspiration of larvae by a healthy 8-month-old girl, which cannot be found in the English literature.

Solid variant of aneurysmal bone cyst of the rib presenting as a left intrathoracic mass without radiological bone destruction.

An aneurysmal bone cyst (ABC) is a benign but often rapidly expanding osteolytic multi-cystic osseous lesion that occurs as a primary, secondary, intra-osseous, extra-osseous, solid, or conventional lesion. A 15-year-old boy presented with a left-sided intrathoracic mass displacing the lung without bone destruction. The mass was totally resected without rib resection, and the pathological diagnosis was ABC. The clinical manifestations, etiology, management, and pathology are discussed, with a brief discussion regarding the difficulty in the preoperative differential diagnosis.

Complications of total implantable access ports and efficacy of Taurolidine-citrate lock solution against catheter-related infections.

BACKGROUND: Totally, implantable access ports (TIAPs) are used for long standing venous catheterization. This study was designed to present our experiences of the TIAPs applications and efficacy of Taurolidine-citrate lock solution (TCLS) against catheter-related infections. MATERIALS AND METHODS: We evaluated records of the 108 patients implanted with 112 TIAPs, which had been performed using heparin solution or TCLS between 2005 and 2013. RESULTS: Duration of exposure to TIAPs was 17-2051 days (median: 411 days). The primary diagnoses were solid tumours (n = 57), lymphoma (n = 23), haematologic diseases (n = 23), nephrotic syndrome (n = 4), Hirschsprung disease (n = 1). The right external jugular vein was most frequently used vascular access route (72.3%). Mechanical complications were observed in four cases. TIAPs were removed due to remission in 19 cases and infection in 19 cases. Median time from implantation and to the development of infection was 60 days. Heparin solution had been used for care in 33 ports, whereas heparin and TCLS had been used in 79 ports. Based on statistical comparison, use of TCLS was considered to be an important factor for preventing infection (P = 0.03). CONCLUSION: We consider that TCLS reduces infection prevalence so TIAPs would be used more extensively and effectively to prevent infections.

Different clinical presentations, diagnostic difficulties, and management of cecal duplication.

BACKGROUND: Cecal duplications (CDs) are very rare, representing 0.4% of all gastrointestinal duplications. This study evaluates the variable clinical presentations, imaging workup, and surgical management of CDs. METHODS: The records of 7 patients who underwent surgery between April 2001 and December 2011 for CD were retrospectively reviewed. Sex, age, duration of complaints, diagnostic studies, surgical intervention, and pathologic findings were recorded. RESULTS: The median age was 8 months, and mean age was 1.65 ± 2.88 years. Complaints were abdominal pain, rectal bleeding, vomiting, cough and dyspnea, and a prenatally detected cystic mass. The patients were referred with the diagnosis of appendicular abscess, necrotizing enterocolitis, gastrointestinal lymphoma, and intraabdominal cystic mass. Abdominal distention, signs of peritonitis, substernal retraction, fullness at right lower quadrant, and normal findings were detected on physical examination. Diagnostic imaging included plain abdominal radiography (7), ultrasonography (7), computed tomography (3), and magnetic resonance imaging (2). A cystic mass was reported on radiologic studies in 6 patients and appendicular abscess in 1. Cyst and cecum were resected, ileocolostomy was performed in 6 patients, and cyst excision was performed in 1. The diagnosis of duplication cyst was made by pathologic examination in all patients. CONCLUSIONS: Cecal duplications may be detected incidentally; however, they may mimic appendicular abscess, a tumor mass, or necrotizing enterocolitis. Whether cystic lesions are symptomatic, they should be excised when detected because of possible complications.