Non­syndromic first case of pediatric rhabdomyosarcoma originating from the umbilical left medial ligament: A case report.

Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children. The present study reports the case of a 2-year-old female who presented with abdominal pain and a palpable abdominal mass. Radiological investigations failed to reveal the tissue origin of the mass and a tru-cut biopsy confirmed the diagnosis of embryonal RMS. Surgical excision was performed after neo-adjuvant chemotherapy. The pelvic end of the mass was observed to continue with the left medial umbilical ligament. The patient's postoperative course was uneventful, and follow-up imaging showed no evidence of recurrence. Τhe present case report is the first non-syndromic case with left umbilical medial ligament-originated RMS.

A rare cause of intestinal obstruction in children trichobezoar: How to diagnose?

BACKGROUND: Trichobezoar is a rare clinical condition in children, which is caused by the accumulation of swallowed hair mass in the digestive tract. This condition is most common in young women with psychiatric histories who suffer from trichotillomania (TTM), where they have an irresistible urge to pull out their hair. Diagnosis and treatment of this pathology, which is already extremely rare, and its variable clinical presentations are challenging. The aim of this study was to increase awareness of trichobezoar in the differential diagnosis of signs of intestinal obstruction in children and to evaluate the diagnosis and management of this rare pathology. METHODS: The clinical data of six patients who were treated for trichobezoars in the pediatric surgery department of our hospital between 2009 and 2022 were retrospectively analyzed. RESULTS: Six female patients were treated with the diagnosis of trichobezoar during this period. Patients were diagnosed with the help of anamnesis, physical examination, abdominal ultrasonography (USG), and finally, endoscopy. USG can predict the intestinal wall infiltration and the tail extended to the duodenum through pylorus in the series. All patients were evaluated with contrast-enhanced abdominal radiography. Five surgical interventions were performed in four of the cases. In a case who underwent surgery twice, the distal intestinal satellite bezoar was not noticed in the first operation. Two patients were diagnosed to have trichobezoar, but surgery was not required. These patients were younger and had early-onset TTM (before 10 years old). The patients were followed for an average of 10.8 years and no recurrence was detected. CONCLUSION: Trichobezoar is a rare cause of intestinal obstruction in children with fatal complications when diagnosed late. Failure to follow an algorithm for the management of the disease causes difficulties in the diagnosis and treatment. Especially in pa-tients with a known psychiatric history, whole abdominal USG and laparoscopy performed with awareness can prevent unnecessary examinations.

A rare cause of intestinal obstruction in children: signet-ring cell adenocarcinoma of the colon.

BACKGROUND: Signet-ring cell adenocarcinoma of the colon is well-recognized in adult patients who are extremely rare and not well-documented in children. Our study aims to raise awareness about this rare disease and its long-term outcomes. METHODS: We retrospectively evaluated patients with signet-ring cell colon adenocarcinoma. RESULTS: Six patients, three boys and three girls, with a mean age of 14.83 (range, 13-17 years), presented with signs of intesti-nal obstruction and were diagnosed with signet-ring cell colon adenocarcinoma. All patients had air-fluid levels on abdominal X-ray. Abdominal ultrasonography of all patients revealed subileus. Abdominal computed tomography was performed in five patients, and pre-operative colonoscopy was conducted in two patients before the emergency intervention. All of the patients underwent emergent exploratory laparotomy with the preliminary diagnosis of acute abdomen. In two patients, debulking surgery followed by a stoma was performed. The remaining four patients were treated with anastomosis following intestinal resection. All girls had metastases on the ovary. One of the patients died due to the burden of multiple metastases in the early period, and three died in the sixth post-operative year. We have been following the remaining two patients since then. CONCLUSION: Although signet-ring cell carcinomas (SRCCs) are rare, they should be considered in the differential diagnosis of acute abdomen and intestinal obstruction in pediatric patients. Despite early diagnosis and treatment, SRCC has a poor prognosis in the pediatric population.

Use of procalcitonin as a biomarker for sepsis in pediatric burns.

Infection and sepsis continue to be the leading cause of morbidity and death in burn injuries. Diagnosing sepsis in burns is challenging as signs and symptoms of sepsis are not specific and overlap with those related to the burn injury. While the use of procalcitonin (PCT) as a biomarker is recommended for diagnosing sepsis in burns, evidence for children with burns is scarce. In this study, we aimed to investigate the role of PCT in distinguishing sepsis in pediatric burns. A prospective observational study was conducted in a single pediatric burn unit. Children hospitalized with burns ≤ 30% of total body surface area were included while patients with chemical burn, inhalation injury, or concomitant chronic diseases were excluded. Patients were classified into three groups for sepsis, systemic inflammatory response syndrome (SIRS), or controls using the American Burn Association (ABA) criteria. The predictive role of C-reactive protein (CRP) and PCT was investigated for distinguishing sepsis. Seventy-two patients were included in the study. The median total body surface area (TBSA) size was 12% (2.0-28.5%), and the median abbreviated burn severity index (ABSI) score was 3 (2-7). The median length of burn unit stay was 9.5 days (1-59 days). Sepsis was diagnosed in 11 patients (15.2%), and SIRS was present in 23 patients (40.0%), whereas 38 patients (52.8%) had neither SIRS nor sepsis (control group). Receiver operating characteristic analysis revealed that CRP and PCT levels distinguished sepsis patients from non-sepsis patients while PCT had a higher positive predictive value (50.0% vs. 45.0%). Optimal cutoff values of CRP and PCT for distinguishing sepsis were 66.75 mg/L and 0.95 ng/mL. CONCLUSIONS: PCT levels could distinguish sepsis in children with burn injuries, performing better than CRP levels. Confirmatory studies are needed to evaluate the development of sepsis and the role of PCT in diagnosing sepsis in pediatric burn patients. WHAT IS KNOWN: • Even though there are excellent criteria for the diagnosis of infection and sepsis in children and several clinical parameters and biomarkers are being studied, it's difficult to diagnose burn wound sepsis in children. WHAT IS NEW: • Data from this study showed that procalcitonin levels performed better than CRP levels as a biomarker for distinguishing sepsis from systemic inflammatory response syndrome (SIRS) in children with burn injuries.

Laparoscopic Repair of Morgagni Hernia in Children.

PURPOSE: This study aimed to evaluate our patients who underwent laparoscopic-assisted transabdominal repair for Morgagni hernia (MH). METHODS: We retrospectively reviewed patients who underwent laparoscopy-assisted transabdominal repair using loop sutures for MH between March 2010 and April 2021. Demographic data, symptoms, operative findings, operation methods, and postoperative complications of the patients were reviewed. RESULTS: A total of 22 patients with MH were treated with laparoscopy-assisted transabdominal repair using loop suture. There were 6 girls (27.2%) and 16 boys (72.7%). Two patients had Down syndrome, and two patients had cardiac defects (secundum atrial septal defect, patent foramen ovale). One patient had a V-P shunt due to hydrocephalus. One patient had cerebral palsy. The mean operation time was 45 min (30-86 min). The hernia sac was not removed, and a patch was not used in any of the patients. The mean hospitalization time was 1.7 days (1-5 days). One patient's defect was very large, and another patient's liver was densely attached to the liver sac, causing bleeding during dissection. In total, two patients were converted to open surgery. There was no recurrence during the follow-up. CONCLUSION: Laparoscopy-assisted transabdominal repair is an efficient and safe choice for the repair of MH. Leaving the hernia sac does not increase the recurrence, so there is no need to dissect the sac.

Our Experience with Laparoscopic Pyloromyotomy in Patients with Infantile Hypertrophic Pyloric Stenosis.

OBJECTIVES: In this study, we aim to discuss our experience with laparoscopic pyloromyotomy in patients with infantile hypertrophic pyloric stenosis (IHPS) and skills development throughout our learning curve. METHODS: We retrospectively collected data from 15 patients with IHPS who underwent laparoscopic pyloromyotomy between 2016 and 2019 in our clinic. Evolution in operation techniques, peroperative and postoperative surgical complications were analysed. RESULTS: In this research, 15 patients (male-to-female ratio:2.7/1) were studied. The median age at presentation was 36.5 days (25-100 days). Non-bilious projectile vomiting was seen in all of the patients, and in eight cases, marked failure to thrive was seen. Situs inversus totalis was seen in one of the cases as an associated anomaly, no other anomalies were noted. A palpable olive-shaped mass was found in only 33% of infants (five cases). A patient was detected to have no IHPS peroperatively. One of the cases was converted to open technique due to peroperative technical difficulties. A patient underwent 2nd operation due to incomplete pyloromyotomy. The duration of the first and last cases was 110 mins and 35 mins, respectively. CONCLUSION: The laparoscopic approach in patients with infantile hypertrophic pyloric stenosis can result in good postoperative outcomes and satisfying surgery in the hands of surgeons who perform minimally invasive surgery routinely.

Assessment of Patients with Hirschsprung Disease and the Use of Laparoscopy.

OBJECTIVES: This study aims to evaluate patients who were diagnosed and treated due to Hirschsprung disease (HD) in our clinic. METHODS: We retrospectively evaluated the demographic and clinical findings of the patients with HD, who were operated in our clinic between January 2010 and December 2015. RESULTS: During study period, 28 patients (19 male 9 female) were found to be operated due to HD in our clinic. Mean age was 16.8 months (1-168). "Transanal Endorectal Pull-through (TERPT)" was performed to 20 of them, Duhamell procedure to five and Soave procedure to three of them. TERPT was applied as laparoscopy assisted in four of them and biopsies were taken laparoscopically preoperatively from one of the patients from each group. Soave procedure was performed in three patients; one had anal stenosis and history of recurrent enterocolitis after TERPT procedure and pathologic analysis revealed neuronal intestinal dysplasia and the other one had total colonic HD and performed Soave procedure with colonic patch. Seven (25%) patients had enterocolitis. Frequencies of enterocolitis were three in two patients, two in two patients and one in three patients. Broad spectrum anibiotics and rectal washouts were supplied to these patients. Five of the seven patients with enterocolitis were operated with TERPT; two patients were operated with Duhamell procedure. Only one of them had total colonic HD. Three patients had total colonic HD diagnosis. Two of them were operated with Duhamell-Martin procedure and one was with Soave procedure with colonic patch according to Kimura technique. Anal stenosis developed in two patients after TERPT and treated with dilatations. Soiling rate was 3% (1/28) and this single patient was treated with laxatives and toilet training. Mean duration of hospitalization was 8.75 (2-14) days. Mean length of the removed intestinal segment was 23.6 (5-38) cm. Mean follow-up was for 35.5 (2-56) months. Neither of the patients was followed in the intensive care unit postoperatively nor died. CONCLUSION: TERPT procedure win priority in HD, but other procedures keep importance. Recently, laparoscopy-assisted TERPT is preferred in our clinic in HD therapy due to easy biopsy, full exposure to the transitional zone, the advantage of meso preparation of colon and prevention of strained anastomosis.

Thoracoscopic Resection in the Treatment of Spontaneous Pneumothorax.

OBJECTIVES: We retrospectively evaluated the patients with primer spontaneous pneumothorax (PSP) who were treated with thoracoscopic resection. METHODS: We retrospectively collected the data of the patients with a spontaneous pneumothorax who were operated with video-assisted thoracoscopic surgery (VATS) between 2010 and 2016. RESULTS: During the study period, 10 patients applied to our hospital with spontaneous pneumothorax. Five children (three boys, two girls) with a mean age of 16.6 (16-17) were selected with VATS. Three of the patients had bleb, one of the patients had Congenital Cystic Adenomatoid Malformation (CCAM) type 2, and the last one had chronic emphysematous tissue on pathological analyses. Post-operative follow-up time was 2.2 (1-4) years without any complication. CONCLUSION: Spontaneous pneumothorax is a disease especially seen in puberty. The main reasons are apical segment bullae formation and blebs. VATS is especially advantageous to reach apical segments and for easy resections. Blebs, CCAM and emphysematous lung tissue may cause spontaneous pneumothorax.

Can serum soluble urokinase plasminogen activator receptor be an effective marker in the diagnosis of appendicitis and differentiation of complicated cases?

BACKGROUND: Soluble urokinase plasminogen activator receptor (suPAR) is a new biomarker of inflammation level. The aim of the study was to evaluate whether suPAR levels could be useful to detect acute appendicitis and to differentiate uncomplicated appendicitis (UA) from complicated appendicitis (CA). METHODS: We prospectively studied 105 patients consisting of 40 UA cases, 40 CA cases, and 25 control patients. Blood samples were collected to measure suPAR level, C-reactive protein level, leukocyte counts, neutrophil counts, and neutrophil percentages preoperatively. RESULTS: Median values of suPAR level, C-reactive protein level, leukocyte counts, neutrophil counts, and neutrophil percentages in UA and CA were significantly higher than control patients. suPAR levels of the UA and CA groups showed a statistically significant difference (p=0.016). CONCLUSION: The current study demonstrated that serum suPAR concentrations can be helpful in differentiating CA from UA and in diagnosing acute appendicitis.

Laparoscopic repair in children with traumatic bladder perforation.

Here, we report two patients with a traumatic intraperitoneal bladder dome rupture repaired by laparoscopic intracorporeal sutures. The first patient was a 3-year old boy was admitted with a history of road accident. He had a traumatic lesion on his lower abdomen and a pelvic fracture. Computed tomography (CT) scan revealed free intraabdominal fluid. The urethragram showed spreading contrast material into the abdominal cavity. Laparoscopic exploration revealed a 3-cm-length perforation at the top of the bladder. The injury was repaired in a two fold fashion. Post-operative follow-up was uneventful. The second case was a 3-year-old boy fell from the second floor of his house on the ground. He had traumatic lesion on his lower abdomen and a pelvic fracture. Due to bloody urine drainage, a cystography was performed and an extravasation from the dome of the bladder into the peritoneum was detected. On laparoscopy, a 3-cm long vertical perforation at the dome of the bladder was found. The perforation was repaired in two layers with intracorporeal suture technique. The post-operative course was uneventful. Laparoscopic repair of traumatic perforation of the bladder dome is a safe, effective and minimally invasive method. The cosmetic outcome is superior.

Percutaneous internal ring suturing as a first choice laparoscopic inguinal hernia repair method in girls: a single-center study in 148 patients.

INTRODUCTION: We sought to retrospectively assess the operative findings and clinical outcomes of 148 girls who underwent laparoscopic inguinal hernia repair with the percutaneous internal ring suturing (PIRS) technique. METHODS: Between 2010 and 2014, girls with inguinal hernia underwent surgery using the laparoscopic PIRS technique described by Patkowski. Demographic and perioperative findings, complications, and recurrences were evaluated. RESULTS: A total of 205 inguinal hernia repairs were performed in 148 children with a mean age of 5.83 years (1 month-16 years). In 57 girls (38.5 %), the hernias were bilaterally repaired, while in 91 girls (61.5 %) hernias were unilaterally repaired. The mean follow-up time was 3.6 years (range 2.5-6.1 years). No serious complications or recurrence were noted. Granuloma occurred in one patient. CONCLUSION: The PIRS technique is a safe, simple and effective procedure for girls. Excellent cosmetic results and reduced recurrence rates are associated with this method. This procedure is particularly suitable for girls because they lack a spermatic cord and vascular structures that can cause complications with this technique in boys. Based on our experience and others in the literature, we suggest that the PIRS procedure might be considered a gold standard for inguinal hernia operations in girls.

Amyand's hernia: 11years of experience.

PURPOSE: Presence of the vermiform appendix in an inguinal hernia sac is known as Amyand's hernia. This may present as a tender inguinal swelling and is often misdiagnosed as irreducible or strangulated hernia. METHODS: Between January 2003 and December 2013 we treated 4498 patients with inguinal hernias and performed 3267 appendectomies. Among these; 46 had an Amyand's hernia. Age, sex, presenting symptoms, treatment modality, histopathological findings, duration of hospitalization, and post-surgical outcomes were analyzed retrospectively. RESULTS: All patients were boys. The mean age was 16.7months (15days-8years). 37 (80.4%) right, 2 (4.3%) left and 7 (15.2%) bilateral hernioplasties were performed. Nine patients underwent emergency surgery with an initial diagnosis of incarcerated hernia; Amyand's hernia was an incidental finding in the remaining 37 patients. Operative findings included 33 normal appendices, 9 inflamed appendices, one perforated appendix, and three appendices adherent to the hernia sac. Eighteen patients had appendectomy during hernia repair, and the other 33 had hernia repair without appendectomy. None of the patients developed recurrent hernia or appendicitis within the follow-up period. CONCLUSION: In Amyand's hernia the appendix should be examined carefully. A classification of Amyand's hernia according to the presenting symptoms and inflammatory status of the appendix may help to determine whether or not to proceed with appendectomy.

Oesophageal duplication cyst mimicking hydatid cyst in endemic areas.

The cystic appearance of both oesophageal duplications and pulmonary hydatid cysts can cause a misdiagnosis very easily due to rarity of cystic oesophageal duplications beside the higher incidence of hydatid cyst, especially in endemic areas. Here we report a 7-year-old girl with an oesophageal duplication cyst on the left side misdiagnosed as a hydatid cyst. The aim of the study is to report rare oesophageal duplications in the differential diagnosis of intrathoracic cysts.

Ultrasound-guided hydrostatic reduction of intussusception with saline: Safe and effective.

PURPOSE: The study was undertaken to assess the efficacy of ultrasound-guided saline enema in reducing intussusception and to determine the role of age and duration of symptoms on this event. METHODS: The case records of patients who were treated for intussusception at our institutions over the past 10 years were retrospectively analyzed. A total of 419 patients were treated for intussusception and 375 of them were included into the study. Patients were excluded if they had symptoms and signs of acute abdominal disease and required surgery as an initial treatment. RESULTS: Hydrostatic reduction was successful in 313 of the 375 patients (83.46%). The procedure-related complication rate was nil. There were 29 episodes of recurrences in 23 patients, and recurrence rates did not differ between patients who responded to hydrostatic reduction and those who required surgery. Younger age [median (range); 11 months (3-108 months) vs. 20 months (1-180 months); p<0.05], rectal bleeding (p<0.01) and long duration of symptoms [mean (range); 1.95 days (1-7 days) vs. 1.44 days (1-10 days); p<0.01] were significantly associated with failed hydrostatic reduction. CONCLUSION: Ultrasound-guided hydrostatic reduction is an easy, safe and effective method for the treatment of intussusception in the absence of acute abdominal findings.

Does percutaneous internal ring suturing contain risk of ilioinguinal nerve entrapment?

PURPOSE: In this study, we aim to assess the alteration of IIN functions in children with inguinal hernias operated on using open or 'percutaneous internal ring suturing' (PIRS). METHODS: This study was based on a prospective clinical trial of 60 pediatric patients. They were operated on using PIRS or conventional open hernia repair technique. Group 1 included 35 patients who were treated with PIRS technique. Group 2 included 22 patients who underwent a conventional open hernia repair. The ilioinguinal nerve stimuli in both the operational and non-operational areas were evaluated in patients with peripheral EMG for possible ilioinguinal nerve damage on the hernia side before the operation and to reevaluate ilioinguinal nerve function in the third postoperative week. RESULT: In Group 1, 19 cases underwent a preoperative EMG examination and in 35 cases, EMG examination was obtained postoperatively. Pre- and postoperative EMG results were normal in all cases in Group 1 on both the operational and non-operational sides. In Group 2, 15 preoperative and 25 postoperative EMG examinations were obtained. In Group 2, only one case with a right inguinal hernia who had normal preoperative EMG results showed no IIN response in a postoperative EMG evaluation obtained in the third postoperative week, with a normal left-side response. The EMG was repeated at the three-month postoperative third mark and revealed the same result. In a six-year-old female case, there was a negative EMG response on the non-operative side both pre- and postoperatively.

A retrospective review of the adnexal outcome after detorsion in premenarchal girls.

BACKGROUND: The aim of this study was to report our results on premenarchal girls with adnexal torsion who were treated with different approaches. MATERIALS AND METHODS: Twenty-six adnexal torsions in children were analysed retrospectively. Group 1 included cases of oophorectomy for the twisted adnexa. Group 2 contained the patients with adnexal torsion who untwisted either with a laparoscopic or open approach. Postoperative restoration of ovarian function was evaluated by Doppler ultrasound at the 6 th month. All oophorectomy and biopsy specimens were also evaluated. RESULTS: Group 1 consisted of eleven cases that underwent oophorectomy due to gangrenous change and haemorrhagic infarction. Histology was of a mature teratoma in two cases and haemorrhagic necrosis due to torsion in seven. Group 2 consisted of 15 patients. In 10 out of 15 patients, preoperative biopsy is performed in which their histology revealed haemorrhagic necrosis in eight cases, and simple cyst with a benign nature in two cases. In all of the 10 untwisted adnexas, postoperative radiological imaging showed complete recovery with normal follicular development. No malignancy or increased tumour markers were noted in both groups. CONCLUSION: Adnexas can be left in place regardless of the preoperative degree of necrosis. Biopsy can be added to the procedure to rule out malignancy.

Wilms' tumor: a 24-year retrospective study from a single center.

Medical records of 71 children with Wilms' tumor at Sisli Etfal Education and Research Hospital between 1990 and 2014 were reviewed. Mean age at diagnosis was 3.11 years (2 days-7 years). Male to female ratio was M/F = 6/10. The incidence of associated anomaly was 16.9%. Clinical manifestations included abdominal mass (89%), hematuria (30%), hypertansion (25%), abdominal pain (15%), fever (5%), restlessness (2%), weight loss (2%), varicocele (1%). Ultrasound (USG) was the most often initial study in a child presenting with abdominal mass. Doppler USG was also made to evaluate the inferior vena cava (IVC) for the presence of tumor extension in children with renal mass. The left kidney was affected in 33 patients (46.5%), the right was affected in 31 patients (43.7%). Two patients was extrarenal (2.8%). And 5 patients (7.04%) were bilateral on the presentation. Preoperative chemotheraphy was done in 14 cases. In 63 patients with unilateral Wilm tm, unilateral radical nefrectomy is performed. In one patient with solitary kidney, nephron sparing surgery (NSS) is performed. In 3 patients with bilateral tm NSS is performed and in 2 patients with bilateral Wilms' tm NSS is performed in one side and nefrectomy on the other side. Out of 71 Wilms tumor (WT) patients, 17 of them has been out of our follow. And 4 of them are died. Ten of them has metastases. Forty children are under follow with no metastases. Patients with WT needs a multimodal, multidisiplinary treatment with the cooperation of pediatric oncologist and pediatric surgeon and needs close follow-up.

Crossed testicular ectopia: Report of six cases.

Crossed testicular ectopia or transverse testicular ectopia is an extremely rare anomaly characterised by migration of one testis towards the opposite inguinal canal, usually associated with unilateral inguinal hernia. This report describes six cases of crossed ectopic testes, one of the largest series, and with unusual clinical histories.

A comparison of the double hydrodistention implantation technique (HIT) and the HIT with a polyacrylate/polyalcohol copolymer (PPC) for the endoscopic treatment of primary vesicoureteral reflux.

OBJECTIVES: We aimed to compare the success rates of the double hydrodistention implantation technique (HIT) and the HIT with a polyacrylate/polyalcohol copolymer (PPC) for the treatment of primary vesicoureteral reflux (VUR) with a new nonbiodegradable tissue-augmenting substance (Vantris, Promedon, Cordoba, Argentina). METHODS: Between January 2011 and December 2012, fifty-two children who underwent subureteric injection for primary VUR are included. The children were randomly separated into two groups, the HIT and the double HIT groups, according to the type of injection. Success was defined as no reflux on a follow-up voiding cystourethrogram (VCUG) after 6 months. The patients were evaluated according to sex, age, grade of reflux, number of injections, and injected volume, and the radiological success rates were compared. RESULTS: Fifty-two patients underwent an endoscopic injection for primary grade III-V VUR. The HIT group consisted of 26 patients with 33 ureters, and the double HIT group consisted of 26 patients with 35 ureters. There were no significant differences in terms of the sex, ages, VUR grades, bilaterality between the two groups. The mean injected volumes were ml 1.12 (1.02-1.22) in the HIT group and 1.24 ml (95 % CI 1.10-1.38) in the double HIT group. The reflux was resolved in 21/33 (63.6 %) ureters in the HIT group and in 30/35 (85.7 %) ureters in the double HIT group, (p < 0.05). We had only one complication. This patient in the double HIT group, developed bilateral hydronephrosis and oliguric renal failure requiring open reimplantation at the sixth month. CONCLUSION: We observed successful results double HIT method with PPC in Grade III-V reflux, but the long-term follow-up of patients is needed for hydronephrosis. As the double HIT treatment leads to a higher success rate, its use is preferable.