Pub Date : 2022-08-16eCollection Date: 2022-01-01DOI: 10.1055/s-0042-1755721
Balázs Fadgyas, Gábor István Garai, János Schnur, Viktória Irén Kiss, Viktória Vass, Enikő Mátyus, György Balázs, Tamas Cserni
Severe abdominal pain and vomiting are common symptoms in children with pediatric multisystem inflammatory syndrome (PIMS). Mesenteric lymphadenitis and aseptic peritonitis are predominantly reported in cases where acute surgical abdomen was suspected and laparotomy was performed at the early stage of the pandemic. These reports generally discouraged surgeons to perform exploration in COVID-19-related cases and medical management was prioritized. Only a few COVID-19-specific surgical cases with intestinal ischemia were published. Here, we report another case of COVID-19-related intestinal ischemia complicated with Meckel's diverticulitis in a non-immunocompromised child who clearly required surgical intervention. In our case, the combination of COVID-19-related vasculitis and low blood pressure episodes may have contributed to this severe outcome.
{"title":"COVID-19-Related Intestinal Ischemia in A 7-Year Old Boy.","authors":"Balázs Fadgyas, Gábor István Garai, János Schnur, Viktória Irén Kiss, Viktória Vass, Enikő Mátyus, György Balázs, Tamas Cserni","doi":"10.1055/s-0042-1755721","DOIUrl":"https://doi.org/10.1055/s-0042-1755721","url":null,"abstract":"<p><p>Severe abdominal pain and vomiting are common symptoms in children with pediatric multisystem inflammatory syndrome (PIMS). Mesenteric lymphadenitis and aseptic peritonitis are predominantly reported in cases where acute surgical abdomen was suspected and laparotomy was performed at the early stage of the pandemic. These reports generally discouraged surgeons to perform exploration in COVID-19-related cases and medical management was prioritized. Only a few COVID-19-specific surgical cases with intestinal ischemia were published. Here, we report another case of COVID-19-related intestinal ischemia complicated with Meckel's diverticulitis in a non-immunocompromised child who clearly required surgical intervention. In our case, the combination of COVID-19-related vasculitis and low blood pressure episodes may have contributed to this severe outcome.</p>","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2022-08-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9381359/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40433129","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}
Laparoscopic surgery has been applied for Hirschsprung's disease (HD). We herein report our approach to mesenteric processing for laparoscopic-assisted transanal endorectal pull-through (L-TERPT). Following mucosectomy and entering the abdominal cavity, a vessel sealing system is transanally inserted into the abdominal cavity for mesenteric processing based on concept of Natural Orifice Translumenal Endoscopic Surgery. Since the transanal axis is parallel to the dissected mesentery, it makes easier to operate in comparison to when the procedure is performed through the abdominal working port and can reduce the additional abdominal trocar wound. We also use indocyanine green (ICG) fluorescence navigation. Fluorescing the vessels with ICG allows intraoperative visualization of the blood flow in the retrieved intestine. With these innovative combined techniques, L-TERPT for HD can be safely performed, even in infants with small intraabdominal cavities.
{"title":"Transanal Mesenteric Resection in Hirschsprung's Disease Using ICG under Concept of NOTES Technique.","authors":"Mitsuru Muto, Shun Onishi, Masakazu Murakami, Keisuke Yano, Toshio Harumatsu, Satoshi Ieiri","doi":"10.1055/s-0042-1751051","DOIUrl":"https://doi.org/10.1055/s-0042-1751051","url":null,"abstract":"<p><p>Laparoscopic surgery has been applied for Hirschsprung's disease (HD). We herein report our approach to mesenteric processing for laparoscopic-assisted transanal endorectal pull-through (L-TERPT). Following mucosectomy and entering the abdominal cavity, a vessel sealing system is transanally inserted into the abdominal cavity for mesenteric processing based on concept of Natural Orifice Translumenal Endoscopic Surgery. Since the transanal axis is parallel to the dissected mesentery, it makes easier to operate in comparison to when the procedure is performed through the abdominal working port and can reduce the additional abdominal trocar wound. We also use indocyanine green (ICG) fluorescence navigation. Fluorescing the vessels with ICG allows intraoperative visualization of the blood flow in the retrieved intestine. With these innovative combined techniques, L-TERPT for HD can be safely performed, even in infants with small intraabdominal cavities.</p>","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2022-08-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9381358/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40433130","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}
Pub Date : 2022-07-27eCollection Date: 2022-01-01DOI: 10.1055/s-0042-1751241
Anthony Mancuso, Amy Singleton, Hadeel Abaza, Michael Albert, Jeffrey Mikutis
A 10-year-old male presented with symptoms in his right shoulder indicative of adhesive capsulitis. Radiographic films did not demonstrate any osseous abnormalities. Magnetic resonance imaging demonstrated the presence of an eccentric lesion within the coracoid process consistent with an osteoid osteoma. Six months after surgical removal the patient is back to full activities. For the pediatric population, surgeons must always consider diagnoses that could alter a patient's growth or result in long-term disability. In particular, an atypical presentation of musculoskeletal disease in a pediatric patient presenting with a disease that typically is seen in the older population warrants further workup.
{"title":"Osteoid Osteoma of the Coracoid Process Presenting as Adhesive Capsulitis in a 10-Year-Old Male: A Case Report.","authors":"Anthony Mancuso, Amy Singleton, Hadeel Abaza, Michael Albert, Jeffrey Mikutis","doi":"10.1055/s-0042-1751241","DOIUrl":"https://doi.org/10.1055/s-0042-1751241","url":null,"abstract":"<p><p>A 10-year-old male presented with symptoms in his right shoulder indicative of adhesive capsulitis. Radiographic films did not demonstrate any osseous abnormalities. Magnetic resonance imaging demonstrated the presence of an eccentric lesion within the coracoid process consistent with an osteoid osteoma. Six months after surgical removal the patient is back to full activities. For the pediatric population, surgeons must always consider diagnoses that could alter a patient's growth or result in long-term disability. In particular, an atypical presentation of musculoskeletal disease in a pediatric patient presenting with a disease that typically is seen in the older population warrants further workup.</p>","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2022-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9329140/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40573384","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}
Pub Date : 2022-07-27eCollection Date: 2022-01-01DOI: 10.1055/s-0042-1750409
Amr AbdelHamid AbouZeid, Shaimaa Abdelsattar Mohammad, Marco Rady Sos, Nader Nassef Guirguis, Heba A Mahmoud, Manal El-Mahdy
"Cloaca" is a term used to describe an anomaly in the female where a single orifice is located in the perineum draining both urogenital and gastrointestinal tracts. Few reports used the same term "cloaca" to describe the counterpart anomaly in the male. We present two "male" cases of anorectal anomalies associated with significant penile deformity (caudally displaced penis) that were managed during the period between January 2010 and September 2021. Characteristically, both cases had a single "central" perineal orifice. The latter was located anterior to the predestined site of the normal anus and just beneath a caudally positioned hypospadiac phallus. The caudal displacement of the penis was strikingly obvious by the presence of severe form of penoscrotal transposition. Both cases were associated with a perineal swelling (hamartoma) just beside the central perineal orifice. The urethra was very short (like that in the female), besides the single perineal orifice, which makes the presentation very similar to cloacal anomalies.
{"title":"Cloaca-Like Anomalies in the Male: A Report on Two Cases.","authors":"Amr AbdelHamid AbouZeid, Shaimaa Abdelsattar Mohammad, Marco Rady Sos, Nader Nassef Guirguis, Heba A Mahmoud, Manal El-Mahdy","doi":"10.1055/s-0042-1750409","DOIUrl":"https://doi.org/10.1055/s-0042-1750409","url":null,"abstract":"<p><p>\"Cloaca\" is a term used to describe an anomaly in the female where a single orifice is located in the perineum draining both urogenital and gastrointestinal tracts. Few reports used the same term \"cloaca\" to describe the counterpart anomaly in the male. We present two \"male\" cases of anorectal anomalies associated with significant penile deformity (caudally displaced penis) that were managed during the period between January 2010 and September 2021. Characteristically, both cases had a single \"central\" perineal orifice. The latter was located anterior to the predestined site of the normal anus and just beneath a caudally positioned hypospadiac phallus. The caudal displacement of the penis was strikingly obvious by the presence of severe form of penoscrotal transposition. Both cases were associated with a perineal swelling (hamartoma) just beside the central perineal orifice. The urethra was very short (like that in the female), besides the single perineal orifice, which makes the presentation very similar to cloacal anomalies.</p>","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2022-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9329137/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40573385","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}
Pub Date : 2022-07-19eCollection Date: 2022-01-01DOI: 10.1055/s-0042-1749210
Annekatrin Schulze, Jurek Schultz, Adrian Dragu, Guido Fitze
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.
{"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":null,"pages":null},"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}
Pub Date : 2022-07-19eCollection Date: 2022-01-01DOI: 10.1055/s-0042-1750027
Francesca Palmisani, Wilfried Krois, Janina Patsch, Martin Metzelder, Carlos A Reck-Burneo
Introduction 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. Case Description 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. Conclusion 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.
{"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":null,"pages":null},"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}
Pub Date : 2022-07-19eCollection Date: 2022-01-01DOI: 10.1055/s-0042-1748316
Sara Fernandes, Carolina Soares-Aquino, Joana Monteiro, Norberto Estevinho, Mariana Borges-Dias
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.
{"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":null,"pages":null},"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}
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.
{"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":null,"pages":null},"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}
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.
{"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":null,"pages":null},"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}
Pub Date : 2022-06-25eCollection Date: 2022-01-01DOI: 10.1055/s-0042-1748866
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
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.
{"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":null,"pages":null},"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}