Annekatrin Schulze, Jurek Schultz, Adrian Dragu, Guido Fitze
{"title":"Diagnostics and Treatment of Volkmann Ischemic Contracture in a Seven-Year-Old Child.","authors":"Annekatrin Schulze, Jurek Schultz, Adrian Dragu, Guido Fitze","doi":"10.1055/s-0042-1749210","DOIUrl":"https://doi.org/10.1055/s-0042-1749210","url":null,"abstract":"<p><p>A 7-year-old boy presented 6 weeks after open reduction and crossed Kirschner wire (K-wire) fixation of a supracondylar humerus fracture. Previous treatments had restored skeletal anatomy without documented complications. However, the patient would not move the entire arm, including his forearm and hand. Any passive movement led to anxious adverse reactions, and there was partial numbness of all fingers. After intensive physio- and occupational therapy supported by nerve stimulation and psychological counseling, anxiety-related functional deficits of the shoulder and elbow resolved to reveal the severe Volkmann contracture of the right hand developed fully. Electroneurography, X-ray, magnetic resonance imaging of the forearm, and ultrasonography showed nonfunctional ulnar and a partially disturbed radial motor nerve distal to the elbow along with damaged flexor muscles of the forearm after compartment syndrome. In addition, damage to the median nerve at the elbow level was diagnosed. After intense conservative therapy, we partially resected fibrotic fascia of the superficial flexor compartment, freed ulnar and median nerves, and performed staircase-like releases of tendons and tenotomies. We achieved a full range of motion of all fingers and markedly improved the range of motion of the wrist. The Disabilities of the Arm, Shoulder and Hand scores for function improved from 80 to 16 at the 2-year follow-up postoperatively, but some impairments of fine motor function persisted. Subtle symptoms of a developing compartment syndrome need to be recognized. Overlooked and untreated, a consecutive Volkmann contracture can turn the extremity nonfunctional. Intensive physical, psychological, and surgical therapy in a specialized center can restore function but requires endurance and perseverance throughout the lengthy recovery.</p>","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":" ","pages":"e68-e72"},"PeriodicalIF":0.6,"publicationDate":"2022-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9296264/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40529423","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Francesca Palmisani, Wilfried Krois, Janina Patsch, Martin Metzelder, Carlos A Reck-Burneo
{"title":"High-Resolution Transperineal Ultrasound in Anorectal Malformations-Can We Replace the Distal Colostogram?","authors":"Francesca Palmisani, Wilfried Krois, Janina Patsch, Martin Metzelder, Carlos A Reck-Burneo","doi":"10.1055/s-0042-1750027","DOIUrl":"https://doi.org/10.1055/s-0042-1750027","url":null,"abstract":"<p><p><b>Introduction</b> Anorectal malformations (ARM) affect 1 in 5,000 newborns with a wide range of defects. In the absence of a visible fistula, the diagnosis and classification of ARM require an augmented pressure distal colostogram. This procedure can be done after a diverting colostomy has been performed and implies exposing the child to radiation. We hypothesized that high-resolution transperineal ultrasound could correctly diagnose the type of ARM, thus sparing radiation exposure. <b>Case Description</b> Four full-term male newborns with ARM and no visible anal opening were referred to our center for further management. A diverting descendostomy was performed in the first 48 hours of life in all cases. Prior to the reconstructive surgery, we performed a high-resolution transperineal ultrasound with 3D tomographic reconstruction of the perineal region to assess the urethra, the rectum, and a possible fistula. Findings were compared with a conventional augmented pressure distal colostogram. The image acquisition was fast and did not cause any additional distress to the children. <b>Conclusion</b> In all cases the results of the distal colostogram nicely correlated with the high-resolution transperineal ultrasound with 3D tomographic reconstruction. In the future, we envision a time when it can potentially replace the distal colostogram in preoperative assessment of ARM with no distress and exposure to radiation.</p>","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":" ","pages":"e84-e88"},"PeriodicalIF":0.6,"publicationDate":"2022-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9296266/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40529425","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sara Fernandes, Carolina Soares-Aquino, Joana Monteiro, Norberto Estevinho, Mariana Borges-Dias
{"title":"Thoracic Outlet Syndrome after Minimally Invasive Repair of Pectus Excavatum in a 15-Year-Old Boy: A Case Report.","authors":"Sara Fernandes, Carolina Soares-Aquino, Joana Monteiro, Norberto Estevinho, Mariana Borges-Dias","doi":"10.1055/s-0042-1748316","DOIUrl":"https://doi.org/10.1055/s-0042-1748316","url":null,"abstract":"<p><p>Nuss procedure has become the treatment of choice in pectus excavatum mainly because of the excellent functional and cosmetic results. Despite the good results, several complications have been reported. The aim of this study is to describe a case of thoracic outlet syndrome (TOS) after Nuss procedure and review the management of such rare complication. A 15-year-old boy otherwise healthy was submitted to Nuss procedure, with no perioperative complications. Two-weeks later, the patient complained of right-hand paresthesia, progressive weakness of the right arm and coldness. After imaging and electromyography, TOS diagnosis was established. Removal of the bar was proposed but refused by the patient. Conservative management with rehabilitation exercising and nerve nourishing was initiated. At 7 months, the patient recovered arm and hand function. Abrupt structural changes of thoracic cavity with marked elevation of the upper chest induce nerve and vascular compression arousing a TOS and should be acknowledged as one potential complication of Nuss procedure. Conservative management can be an alternative treatment to bar removal, showing good results on functional recovery in early stages of compression.</p>","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":" ","pages":"e89-e92"},"PeriodicalIF":0.6,"publicationDate":"2022-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9296261/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40529424","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Emergency Separation of Extreme VLBW Omphalopagus Twins: Case Report.","authors":"Waleed Burhamah, Amar Alnaqi, Yaqoub Jafar, Esmaeel Taqi","doi":"10.1055/s-0042-1750134","DOIUrl":"https://doi.org/10.1055/s-0042-1750134","url":null,"abstract":"<p><p>The classification of conjoint twins is based according to the site of attachment. The challenges in management of such anomalies span the entire continuum of care from delivery to resuscitation to separation and finally discharge. Scheduled separation is ideal, occasionally the caring team is faced with no option but to perform an emergent separation. Omphalopagus is a type of conjoined twinning characterized by union of the peritoneal cavities through an infraumbilical abdominal wall defect. In this report we describe our experience with a successful emergency separation of extremely preterm omphalopagus twins. This is the first case of conjoint twins in Kuwait, we highlight the challenges faced, stressing the importance of adhering to antenatal care as well as management by a multidisciplinary team.</p>","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":" ","pages":"e84-e87"},"PeriodicalIF":0.6,"publicationDate":"2022-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9252611/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40590121","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Continuous Hepatogonodal and Splenogonogal Fusion: A Rare Cause of Bilateral Intra-Abdominal Testis in an 18-Month-Old Boy.","authors":"Gül Durmuş, Ozlem Boybeyi-Turer, Hatice Nursun Özcan, Onur Gözmen, Hüseyin Demirbilek, Tutku Soyer","doi":"10.1055/s-0042-1747671","DOIUrl":"https://doi.org/10.1055/s-0042-1747671","url":null,"abstract":"<p><p>The fusion of gonadal structures with internal organs is very rare. The close proximity between the left gonad and spleen during embryogenesis may result in splenogonadal fusion (SGF). Moreover, the trapping of hepatocyte-destined mesenchyme cells in gonads is defined as hepatogonadal fusion (HGF). The fusion of gonads with intra-abdominal organs may be continuous and may impair testicular descent during the prenatal period. We herein report an 18-month-old boy presented with bilateral nonpalpable testis due to concomitant continuous HGF and SGF. To our knowledge, this is the first case of concomitant HGF and SGF in a boy with bilateral intra-abdominal testis. Laparoscopic excision of fibrous cords and orchidopexy can be achieved despite continuous fusions.</p>","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":" ","pages":"e80-e83"},"PeriodicalIF":0.6,"publicationDate":"2022-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9233567/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40402799","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Lucas Moratilla Lapeña, Maria Carmen Sarmiento Caldas, Carla Ramírez, María San Basilio, Paloma Triana Junco, Lara Rodríguez-Laguna, Victor Martínez-González, Elena Marín-Manzano, Antonio Perez-Martinez, Juan Carlos Lopez-Gutierrez
{"title":"Larotrectinib as an Effective Therapy in Congenital Infantile Fibrosarcoma: Report of Two Cases.","authors":"Lucas Moratilla Lapeña, Maria Carmen Sarmiento Caldas, Carla Ramírez, María San Basilio, Paloma Triana Junco, Lara Rodríguez-Laguna, Victor Martínez-González, Elena Marín-Manzano, Antonio Perez-Martinez, Juan Carlos Lopez-Gutierrez","doi":"10.1055/s-0042-1748866","DOIUrl":"https://doi.org/10.1055/s-0042-1748866","url":null,"abstract":"Congenital infantile fibrosarcoma (CIF) is a rare tumor in children that occurs in the first years of life. It usually arises in the extremities but some cases affect the trunk, neck, abdomen, or retroperitoneum. Surgical resection has been traditionally the treatment of choice but the development of genomic analysis and targeted therapies has shed light on new therapeutic options. We present two patients with a congenital mass, one in the abdominal cavity (1-month-old) and the second in the left lower extremity respectively (2-months-old). In both cases, the clinical and radiological findings showed heterogeneous masses with rapidly progressive growth. MRI in the first patient exhibited an abdominal mass surrounding the aorta and inferior vena cava associated with a giant infrarenal aortic aneurysm. CT-guided biopsy was performed with pathological findings of fibrosarcoma and ETV6-NTRK3 gene fusion. The second patient underwent open biopsy also with histopathological diagnosis of fibrosarcoma and the same mutation in the TRK gene ( NTRK3 ). Targeted therapy with a specific TRK inhibitor, larotrectinib, was started in both patients. Periodical controls were made by ultrasound or MRI, and after a few weeks of treatment, both children showed significant decrease in the mass. By the second and third months after starting the treatment, both tumors disappeared. The first patient is now 15-months-old and the second one is 8-months-old. Larotrectinib is a novel targeted therapy with excellent results in CIF but long-term outcomes are limited to establish it as a gold standard treatment.","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":" ","pages":"e76-e79"},"PeriodicalIF":0.6,"publicationDate":"2022-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9233566/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40402798","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Timothy F Tirrell, Farokh R Demehri, Prathima Nandivada, Erin R McNamara, Belinda Hsi Dickie
{"title":"Technical Considerations in Primary Repair of a Congenital Prostatic Rectourethral Fistula in an Adult-Sized Patient.","authors":"Timothy F Tirrell, Farokh R Demehri, Prathima Nandivada, Erin R McNamara, Belinda Hsi Dickie","doi":"10.1055/s-0041-1742155","DOIUrl":"https://doi.org/10.1055/s-0041-1742155","url":null,"abstract":"<p><p>Congenital anorectal malformations are generally diagnosed and repaired as a neonate or infant, but repair is sometimes delayed. Considerations for operative repair change as the patient approaches full stature. We recently encountered a 17-year-old male with an unrepaired congenital rectourethral fistula and detail our experience with his repair. We elected to utilize a combined abdominal and perineal approach, with robotic assistance for division of his rectourethral fistula and pullthrough anoplasty. Cystoscopy was used simultaneously to assure full dissection of the fistula and to minimize the risk of leaving a remnant of the original fistula (also known as a posterior urethral diverticulum). The procedure was well tolerated without complications. His anoplasty was evaluated 60 days postoperatively and was well healed without stricture. At 9 months of follow-up, he has good fecal and urinary continence. Robotic assistance in this procedure allowed minimal perineal dissection while ensuring precise rectourethral fistula dissection. The length of the intramural segment of the fistula was longer than anticipated. Simultaneous cystoscopy, in conjunction with the integrated robotic fluorescence system, helped reduce the risk of leaving a remnant of the original fistula.</p>","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":" ","pages":"e20-e24"},"PeriodicalIF":0.6,"publicationDate":"2022-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8840860/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39927676","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Xiaoyan Feng, Richard Wagner, Silvia Rogers, Martin Lacher, Ophelia Aubert
{"title":"Writing a Case Report in Pediatric Surgery: A Comprehensive Guideline.","authors":"Xiaoyan Feng, Richard Wagner, Silvia Rogers, Martin Lacher, Ophelia Aubert","doi":"10.1055/s-0041-1740935","DOIUrl":"https://doi.org/10.1055/s-0041-1740935","url":null,"abstract":"<p><p>An effective case report delivers clear and valuable clinical or surgical information to the medical community. Case reports dealing with pediatric surgical issues raise the medical community's awareness of rare diseases, unusual presentations of common disorders, or novel surgical or nonsurgical treatment approaches. Thus, case reports contribute substantially to medical advance by sharing remarkable or unexpected findings. For this reason, case reports should be prepared with vigilance, and current conventions on good medial writing practice should be observed. This guideline aims to assist clinicians and surgeons in the successful publishing of a compelling case report in pediatric surgery that is read and understood by the intended audience.</p>","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":" ","pages":"e13-e19"},"PeriodicalIF":0.6,"publicationDate":"2022-02-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8831094/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39915604","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Carlos Delgado-Miguel, Antonio Muñoz-Serrano, Lucas Moratilla, María Del Carmen Sarmiento, Miriam Miguel-Ferrero, Nuria Leal, Saturnino Barrena, Leopoldo Martínez
{"title":"Indocyanine Green (ICG)-Guided Identification of Hypermetabolic Pancreatic Nodules in Focal Congenital Hyperinsulinism: A Case Report in a 3-Month-Old Infant.","authors":"Carlos Delgado-Miguel, Antonio Muñoz-Serrano, Lucas Moratilla, María Del Carmen Sarmiento, Miriam Miguel-Ferrero, Nuria Leal, Saturnino Barrena, Leopoldo Martínez","doi":"10.1055/s-0042-1742780","DOIUrl":"https://doi.org/10.1055/s-0042-1742780","url":null,"abstract":"<p><p>Indocyanine green (ICG)-guided near-infrared fluorescence has been recently adopted in pediatric surgery, although its use in the treatment of congenital hyperinsulinism has not been reported. We present a case of focal congenital hyperinsulinism in which ICG-navigation with ICG was used during surgical treatment. A 3-month-old infant was referred to our institution from a peripheral hospital for episodes of persistent hypoglycemia since birth, with no response to intravenous treatment with diazoxide, octreotide, or hydrochlorothiazide. An abdominal positron emission tomography-computed tomography scan showed a hypermetabolic nodule in the proximal portion of the body of the pancreas, compatible with focal congenital hyperinsulinism. A heterozygous mutation in the ABCC gene (Ala1516Glyfs*19) frameshift type inherited from the father was identified, which supported this diagnosis. Laparoscopy-assisted surgery was performed with ICG-guided near-infrared fluorescence, with intravenous injection of 16 mg ICG (2 mg/mg), which allowed localization of the focal lesion in the body of the pancreas. The lesion was resected with bipolar electrocautery and intraoperative histological study confirmed complete resection. Plasma glucose values normalized 6 hours after surgery and the patient was discharged 5 days later. In conclusion, the use of ICG in the treatment of congenital hyperinsulinism helps to identify hypermetabolic pancreatic nodules, decreasing the likelihood of incomplete resection.</p>","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":" ","pages":"e9-e12"},"PeriodicalIF":0.6,"publicationDate":"2022-02-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8824696/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39915603","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Fabian Doktor, Jan-Hendrik Gosemann, Peter Zimmermann, Manuela Siekmeyer, Holger Stepan, Martin Lacher
{"title":"Umbilical Cord Teratoma Presenting as Ruptured Omphalocele.","authors":"Fabian Doktor, Jan-Hendrik Gosemann, Peter Zimmermann, Manuela Siekmeyer, Holger Stepan, Martin Lacher","doi":"10.1055/s-0041-1741509","DOIUrl":"https://doi.org/10.1055/s-0041-1741509","url":null,"abstract":"<p><p>Congenital mature teratomas of the umbilical cord are extremely rare. We report on a girl who presented with a ruptured omphalocele and a 7 cm mass connected to the umbilicus, which we resected on the first day of life. Histology revealed mature umbilical cord teratoma . On the 29th day of life, a secondary laparotomy was necessary to address the associated intestinal malformations (megaduodenum, stenotic small bowel with duplication and malrotation). After a prolonged hospital stay, we discharged the patient in age-appropriate conditions. Antenatal diagnosis of an umbilical cord tumor can be challenging in the presence of an omphalocele. Given the high prevalence of associated malformations, the finding of umbilical cord teratoma should be followed by a detailed and comprehensive neonatal workup for additional abnormalities.</p>","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":" ","pages":"e6-e8"},"PeriodicalIF":0.6,"publicationDate":"2022-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8813329/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39763951","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}