Pub Date : 2024-09-16DOI: 10.1016/j.epsc.2024.102885
Emily J. Zolfaghari , Matthew Shaughnessy , Hao Wu , Michael Caty , Emily Christison-Lagay , Matthew A. Hornick
Introduction
Pediatric massive thymic hyperplasia is a rare condition that can masquerade as a respiratory illness, making diagnosis challenging.
Case presentation
An 18-month-old female with a 3-month history of multiple admissions for suspected pneumonia warranting antibiotics presented with recurrent respiratory distress, fevers, and fatigue. Work-up for possible parapneumonic effusion, including six chest x-rays and an ultrasound, eventually led to a computed tomography (CT) of the chest, which revealed a large homogeneous mass occupying much of the right-hemithorax. Percutaneous core biopsy showed thymocytes suggestive of either a massive thymic hyperplasia or a thymoma. Consensus was to proceed with upfront surgical resection of the mass, which was performed via right thoracotomy. Pathology was consistent with true thymic hyperplasia. The post-operative course was uncomplicated, and no issues were identified at 3-month follow-up. We plan to follow the patient at 6 months, 1 year, and then annually with surveillance chest x-rays to monitor for recurrence case-report.
Conclusion
Thymic hyperplasia should be included in the differential diagnosis of pediatric patients with persistent respiratory symptoms and abnormal findings on chest x-ray.
导言小儿胸腺大量增生是一种罕见疾病，可伪装成呼吸道疾病，因此诊断具有挑战性。病例介绍一名 18 个月大的女性患者，曾因疑似肺炎多次入院治疗，并使用抗生素，病史长达 3 个月，反复出现呼吸困难、发热和乏力。检查结果显示可能存在肺旁积液，包括六次胸部 X 光检查和一次超声波检查，最终进行了胸部计算机断层扫描（CT），发现一个巨大的均匀肿块占据了右侧胸腔的大部分。经皮核心活检显示，胸腺细胞提示大块胸腺增生或胸腺瘤。大家一致同意对肿块进行前期手术切除，手术是通过右胸廓切开术进行的。病理结果与真正的胸腺增生一致。术后过程并不复杂，3 个月随访时未发现任何问题。我们计划对患者进行 6 个月、1 年的随访，然后每年进行一次胸部 X 光检查，以监测复发情况。
{"title":"Massive thymic hyperplasia in a toddler masquerading as a loculated pleural effusion: A case report","authors":"Emily J. Zolfaghari , Matthew Shaughnessy , Hao Wu , Michael Caty , Emily Christison-Lagay , Matthew A. Hornick","doi":"10.1016/j.epsc.2024.102885","DOIUrl":"10.1016/j.epsc.2024.102885","url":null,"abstract":"<div><h3>Introduction</h3><p>Pediatric massive thymic hyperplasia is a rare condition that can masquerade as a respiratory illness, making diagnosis challenging.</p></div><div><h3>Case presentation</h3><p>An 18-month-old female with a 3-month history of multiple admissions for suspected pneumonia warranting antibiotics presented with recurrent respiratory distress, fevers, and fatigue. Work-up for possible parapneumonic effusion, including six chest x-rays and an ultrasound, eventually led to a computed tomography (CT) of the chest, which revealed a large homogeneous mass occupying much of the right-hemithorax. Percutaneous core biopsy showed thymocytes suggestive of either a massive thymic hyperplasia or a thymoma. Consensus was to proceed with upfront surgical resection of the mass, which was performed via right thoracotomy. Pathology was consistent with true thymic hyperplasia. The post-operative course was uncomplicated, and no issues were identified at 3-month follow-up. We plan to follow the patient at 6 months, 1 year, and then annually with surveillance chest x-rays to monitor for recurrence case-report.</p></div><div><h3>Conclusion</h3><p>Thymic hyperplasia should be included in the differential diagnosis of pediatric patients with persistent respiratory symptoms and abnormal findings on chest x-ray.</p></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"110 ","pages":"Article 102885"},"PeriodicalIF":0.2,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2213576624001131/pdfft?md5=45ed11365993c746183d75987cd2ea86&pid=1-s2.0-S2213576624001131-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142239888","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}
Intramuscular hemangiomas (IMH) are rare benign vascular tumors. They can occur anywhere in the body but are more common in the head and neck. Their location in the muscles of the abdominal wall is uncommon.
Case presentation
A 14-year-old girl presented with 5 months of progressive swelling on the right side of her abdomen followed by intermittent pain. On examination, a solitary mass with ill-defined borders was noted fixed to the subcutaneous tissue in the right lumbar quadrant. After elevating the right leg, the swelling became more prominent. Doppler ultrasonography revealed a mixed echogenic mass in the subcutaneous plane of the anterior abdominal wall of the right lumbar region. Magnetic resonance imaging of abdomen was suggestive of hemangioma, and the patient underwent surgical excision. Histopathological examination confirmed the diagnosis of capillary hemangioma. During follow-up at 3 and 6 months, the patient did not present with recurrence and was healthy.
Conclusion
Although IMH is a rare soft-tissue tumor, it should be considered when diagnosing symptomatic intramuscular swelling.
{"title":"Capillary hemangioma of the external oblique muscle: A case report","authors":"K.K. Athish , Venkat Hariharan Marimuthu , Vamsi Krishna Reddy Kurri , Suggula Vamsi Krishna , Anwadevi Arun , Dinesh Kumar Sathanantham","doi":"10.1016/j.epsc.2024.102881","DOIUrl":"10.1016/j.epsc.2024.102881","url":null,"abstract":"<div><h3>Introduction</h3><p>Intramuscular hemangiomas (IMH) are rare benign vascular tumors. They can occur anywhere in the body but are more common in the head and neck. Their location in the muscles of the abdominal wall is uncommon.</p></div><div><h3>Case presentation</h3><p>A 14-year-old girl presented with 5 months of progressive swelling on the right side of her abdomen followed by intermittent pain. On examination, a solitary mass with ill-defined borders was noted fixed to the subcutaneous tissue in the right lumbar quadrant. After elevating the right leg, the swelling became more prominent. Doppler ultrasonography revealed a mixed echogenic mass in the subcutaneous plane of the anterior abdominal wall of the right lumbar region. Magnetic resonance imaging of abdomen was suggestive of hemangioma, and the patient underwent surgical excision. Histopathological examination confirmed the diagnosis of capillary hemangioma. During follow-up at 3 and 6 months, the patient did not present with recurrence and was healthy.</p></div><div><h3>Conclusion</h3><p>Although IMH is a rare soft-tissue tumor, it should be considered when diagnosing symptomatic intramuscular swelling.</p></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"110 ","pages":"Article 102881"},"PeriodicalIF":0.2,"publicationDate":"2024-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S221357662400109X/pdfft?md5=9ec8de4d6c3107a0df91527188b423de&pid=1-s2.0-S221357662400109X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142274387","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 : 2024-09-12DOI: 10.1016/j.epsc.2024.102883
Paul McClure , Kate McNevin , Giri Shivaram , Caitlin Smith
Introduction
While most lymphatic malformations (LMs) are treated by percutaneous sclerotherapy, intra-abdominal LMs may be difficult to access percutaneously due to their anatomic location.
Case presentation
An 11-year-old otherwise healthy boy presented with abdominal pain and cross-sectional imaging (including CT scan and MRI) identified an intra-abdominal LM located in the lesser sac, bordered by the stomach, spleen, and pancreas. An attempt to access the lesion percutaneously was made, however this was unsuccessful due to overlying viscera. Given the morbidity associated with an open surgical approach, the patient underwent laparoscopic-guided doxycycline sclerosant injection. After the LM was exposed laparoscopically, a pigtail catheter was placed into the lesion using the Seldinger technique. Doxycycline sclerosant was instilled three times over the course of three days and the pigtail catheter was removed after the third instillation. The LM completed resolved in addition to the abdominal pain the patient was experiencing. After more than one year of follow up, the LM has not recurred.
Conclusion
Abdominal LMs may be safely treated with a laparoscopic-guided doxycycline sclerosant injection when surgical excision would be exceedingly morbid and there is no safe percutaneous entry site.
{"title":"Laparoscopic-guided sclerotherapy of an abdominal lymphatic malformation: A case report","authors":"Paul McClure , Kate McNevin , Giri Shivaram , Caitlin Smith","doi":"10.1016/j.epsc.2024.102883","DOIUrl":"10.1016/j.epsc.2024.102883","url":null,"abstract":"<div><h3>Introduction</h3><p>While most lymphatic malformations (LMs) are treated by percutaneous sclerotherapy, intra-abdominal LMs may be difficult to access percutaneously due to their anatomic location.</p></div><div><h3>Case presentation</h3><p>An 11-year-old otherwise healthy boy presented with abdominal pain and cross-sectional imaging (including CT scan and MRI) identified an intra-abdominal LM located in the lesser sac, bordered by the stomach, spleen, and pancreas. An attempt to access the lesion percutaneously was made, however this was unsuccessful due to overlying viscera. Given the morbidity associated with an open surgical approach, the patient underwent laparoscopic-guided doxycycline sclerosant injection. After the LM was exposed laparoscopically, a pigtail catheter was placed into the lesion using the Seldinger technique. Doxycycline sclerosant was instilled three times over the course of three days and the pigtail catheter was removed after the third instillation. The LM completed resolved in addition to the abdominal pain the patient was experiencing. After more than one year of follow up, the LM has not recurred.</p></div><div><h3>Conclusion</h3><p>Abdominal LMs may be safely treated with a laparoscopic-guided doxycycline sclerosant injection when surgical excision would be exceedingly morbid and there is no safe percutaneous entry site.</p></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"110 ","pages":"Article 102883"},"PeriodicalIF":0.2,"publicationDate":"2024-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2213576624001118/pdfft?md5=41430dd8736a686d574082953ab93bd7&pid=1-s2.0-S2213576624001118-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142239887","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 : 2024-09-12DOI: 10.1016/j.epsc.2024.102884
Zachary Ballinger , Erin Scott , Alanna Hickey , Jonathan Green , Jeremy Aidlen , Muriel Cleary
Introduction
Although patients with Rapunzel syndrome are often asymptomatic, rare complications such as isolated gastric or duodenal perforation, pancreaticobiliary or intestinal obstruction, and intussusception have been described.
Case presentation
A 5-year-old female with no known medical history presented after nearly two weeks of worsening constipation, abdominal distension, and abdominal pain. A CT scan demonstrated a large gastric bezoar, pneumoperitoneum, and large volume complex free fluid. The patient was taken to the operating room for an abdominal exploration. We found a large gastric trichobezoar with hair extending into the small bowel, consistent with Rapunzel syndrome. The gastric trichobezoar was connected to a mid-ileal trichobezoar through interdigitating hair fibers. At multiple locations along the small bowel, garroted linear perforations had occurred due to tension created by the intestinal peristalsis along the interconnecting hair fibers, creating mucosal ulcerations and transmural pressure necrosis that required an extensive small bowel resection. Ultimately, we did one end-end small bowel anastomosis and placed a gastrostomy tube. Gastrostomy tube feeds were started on postoperative day 6 after confirming with a contrast study that there were no intestinal leaks. She developed a superficial wound infection that was managed with antibiotics and negative pressure wound therapy. She was discharged tolerating regular diet on post-operative day 23, and is undergoing psychiatric therapy.
Conclusion
Although rare, Rapunzel syndrome can lead to severe complications. When two trichobezoars are present, interconnecting hair fibers can cause small bowel perforations.
{"title":"Rapunzel syndrome leading to multiple bowel perforations: A case report","authors":"Zachary Ballinger , Erin Scott , Alanna Hickey , Jonathan Green , Jeremy Aidlen , Muriel Cleary","doi":"10.1016/j.epsc.2024.102884","DOIUrl":"10.1016/j.epsc.2024.102884","url":null,"abstract":"<div><h3>Introduction</h3><p>Although patients with Rapunzel syndrome are often asymptomatic, rare complications such as isolated gastric or duodenal perforation, pancreaticobiliary or intestinal obstruction, and intussusception have been described.</p></div><div><h3>Case presentation</h3><p>A 5-year-old female with no known medical history presented after nearly two weeks of worsening constipation, abdominal distension, and abdominal pain. A CT scan demonstrated a large gastric bezoar, pneumoperitoneum, and large volume complex free fluid. The patient was taken to the operating room for an abdominal exploration. We found a large gastric trichobezoar with hair extending into the small bowel, consistent with Rapunzel syndrome. The gastric trichobezoar was connected to a mid-ileal trichobezoar through interdigitating hair fibers. At multiple locations along the small bowel, garroted linear perforations had occurred due to tension created by the intestinal peristalsis along the interconnecting hair fibers, creating mucosal ulcerations and transmural pressure necrosis that required an extensive small bowel resection. Ultimately, we did one end-end small bowel anastomosis and placed a gastrostomy tube. Gastrostomy tube feeds were started on postoperative day 6 after confirming with a contrast study that there were no intestinal leaks. She developed a superficial wound infection that was managed with antibiotics and negative pressure wound therapy. She was discharged tolerating regular diet on post-operative day 23, and is undergoing psychiatric therapy.</p></div><div><h3>Conclusion</h3><p>Although rare, Rapunzel syndrome can lead to severe complications. When two trichobezoars are present, interconnecting hair fibers can cause small bowel perforations.</p></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"110 ","pages":"Article 102884"},"PeriodicalIF":0.2,"publicationDate":"2024-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S221357662400112X/pdfft?md5=e70dd972dbf25f5b0036274726829134&pid=1-s2.0-S221357662400112X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142230769","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 : 2024-09-11DOI: 10.1016/j.epsc.2024.102880
Abel Girma Demessie , Suleiman Ayalew , Michael A. Negussie , Leaynadis Kassa , Asratu Getnet Amare , Mesfin Tesera Wassie
Introduction
Dog bites commonly result in soft tissue injuries and infections, but rarely cause intestinal perforation.
Case presentation
A 9-year-old male presented with abdominal distension and vomiting three days after being bitten in the abdomen by a stray dog. He was not evaluated immediately after the injury. One day later he developed abdominal distension, difficulty passing stool and gas, non-projectile vomiting, and intermittent high-grade fever. Two days after these symptoms began, he visited a local health center where he was started on antibiotics and was referred to our hospital for further care. On arrival, he was febrile, tachycardic, tachypneic, irritable, had a normal oxygen saturation and a Glasgow Coma Scale of 15. Abdominal examination revealed distension, diffuse tenderness, absent bowel sounds, and visible bite marks in the left lumbar and iliac regions. Blood tests were normal. A plain abdominal X-ray showed pneumoperitoneum. Suspecting a bowel perforation, the patient was taken to the operating room for an exploratory laparotomy. Approximately 200 ml of pus were found in the peritoneal cavity, along with a pinpoint jejunal perforation 30 cm from the ligament of Treitz, and a serosal tear 3 cm distal to it. The involved jejunal segment was resected, followed by a primary end-to-end jejunal anastomosis. Oral feedings were initiated on postoperative day four. The recovery was uneventful and at the two-week follow-up, he was in good health.
Conclusion
Intestinal perforation following a dog bite, although rare, should be considered in patients presenting with abdominal symptoms after such injuries.
{"title":"Jejunal perforation secondary to dog bite: A case report","authors":"Abel Girma Demessie , Suleiman Ayalew , Michael A. Negussie , Leaynadis Kassa , Asratu Getnet Amare , Mesfin Tesera Wassie","doi":"10.1016/j.epsc.2024.102880","DOIUrl":"10.1016/j.epsc.2024.102880","url":null,"abstract":"<div><h3>Introduction</h3><p>Dog bites commonly result in soft tissue injuries and infections, but rarely cause intestinal perforation.</p></div><div><h3>Case presentation</h3><p>A 9-year-old male presented with abdominal distension and vomiting three days after being bitten in the abdomen by a stray dog. He was not evaluated immediately after the injury. One day later he developed abdominal distension, difficulty passing stool and gas, non-projectile vomiting, and intermittent high-grade fever. Two days after these symptoms began, he visited a local health center where he was started on antibiotics and was referred to our hospital for further care. On arrival, he was febrile, tachycardic, tachypneic, irritable, had a normal oxygen saturation and a Glasgow Coma Scale of 15. Abdominal examination revealed distension, diffuse tenderness, absent bowel sounds, and visible bite marks in the left lumbar and iliac regions. Blood tests were normal. A plain abdominal X-ray showed pneumoperitoneum. Suspecting a bowel perforation, the patient was taken to the operating room for an exploratory laparotomy. Approximately 200 ml of pus were found in the peritoneal cavity, along with a pinpoint jejunal perforation 30 cm from the ligament of Treitz, and a serosal tear 3 cm distal to it. The involved jejunal segment was resected, followed by a primary end-to-end jejunal anastomosis. Oral feedings were initiated on postoperative day four. The recovery was uneventful and at the two-week follow-up, he was in good health.</p></div><div><h3>Conclusion</h3><p>Intestinal perforation following a dog bite, although rare, should be considered in patients presenting with abdominal symptoms after such injuries.</p></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"110 ","pages":"Article 102880"},"PeriodicalIF":0.2,"publicationDate":"2024-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2213576624001088/pdfft?md5=f0620379358ac48d00a37432db7dda78&pid=1-s2.0-S2213576624001088-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142230774","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 : 2024-09-11DOI: 10.1016/j.epsc.2024.102879
Parker T. Evans , Laura Stafman , Hernan Correa , Maren Shipe , Harold Lovvorn III , Jamie Robinson
Introduction
Traction-induced growth (Foker procedure) is a method for esophageal lengthening when primary anastomosis is not feasible in long-gap type A esophageal atresia (EA). We sought to identify the source of esophageal vascularization after thoracoscopic internal traction by analyzing markers of angiogenesis in resected esophageal pouch tips.
Case report
A child with Type A EA underwent esophageal lengthening with internal traction. In addition to EA, the patient also had trisomy 21, duodenal atresia, intestinal malrotation, and congenital heart disease. Operations for EA included thoracoscopic esophageal traction system placement at 7 months of age, and 5 subsequent tightening procedures over the following month. Baseline esophageal gap length was 5.5 cm without tension and 4 cm at maximal tension. Gap length was 3.6 cm, 1.4 cm, 1.3 cm, 7 mm, and 5 mm after each tightening procedure, respectively. Final esophageal anastomosis was done at 8 months of age. At the time of the anastomosis, angiogenic vessels were grossly visible at the ends of each esophageal pouch and indocyanine green fluorescence angiography demonstrated vascularization. Resected samples of the esophageal pouches were analyzed with immunohistochemistry for expression of ERG, a marker of angiogenesis. The patient who underwent traction demonstrated increased submucosal ERG expression (average 6.30 % cells) compared to 5 control patients who did not require traction-based lengthening to achieve anastomosis (average 1.34 % cells).
Conclusion
Thoracoscopic traction with serial tightening achieves adequate growth to restore esophageal continuity in patients with long-gap EA. Our findings suggest that anastomotic blood supply relies upon submucosal angiogenesis.
{"title":"Submucosal angiogenesis in traction-induced esophageal lengthening for long-gap esophageal atresia: A case report","authors":"Parker T. Evans , Laura Stafman , Hernan Correa , Maren Shipe , Harold Lovvorn III , Jamie Robinson","doi":"10.1016/j.epsc.2024.102879","DOIUrl":"10.1016/j.epsc.2024.102879","url":null,"abstract":"<div><h3>Introduction</h3><p>Traction-induced growth (Foker procedure) is a method for esophageal lengthening when primary anastomosis is not feasible in long-gap type A esophageal atresia (EA). We sought to identify the source of esophageal vascularization after thoracoscopic internal traction by analyzing markers of angiogenesis in resected esophageal pouch tips.</p></div><div><h3>Case report</h3><p>A child with Type A EA underwent esophageal lengthening with internal traction. In addition to EA, the patient also had trisomy 21, duodenal atresia, intestinal malrotation, and congenital heart disease. Operations for EA included thoracoscopic esophageal traction system placement at 7 months of age, and 5 subsequent tightening procedures over the following month. Baseline esophageal gap length was 5.5 cm without tension and 4 cm at maximal tension. Gap length was 3.6 cm, 1.4 cm, 1.3 cm, 7 mm, and 5 mm after each tightening procedure, respectively. Final esophageal anastomosis was done at 8 months of age. At the time of the anastomosis, angiogenic vessels were grossly visible at the ends of each esophageal pouch and indocyanine green fluorescence angiography demonstrated vascularization. Resected samples of the esophageal pouches were analyzed with immunohistochemistry for expression of ERG, a marker of angiogenesis. The patient who underwent traction demonstrated increased submucosal ERG expression (average 6.30 % cells) compared to 5 control patients who did not require traction-based lengthening to achieve anastomosis (average 1.34 % cells).</p></div><div><h3>Conclusion</h3><p>Thoracoscopic traction with serial tightening achieves adequate growth to restore esophageal continuity in patients with long-gap EA. Our findings suggest that anastomotic blood supply relies upon submucosal angiogenesis.</p></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"110 ","pages":"Article 102879"},"PeriodicalIF":0.2,"publicationDate":"2024-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2213576624001076/pdfft?md5=77f9c01184223acf920433b57300f555&pid=1-s2.0-S2213576624001076-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142233586","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}
Intestinal musculature defects leading to intestinal obstruction and perforation in children are rare and poorly understood.
Case presentation
Our case is of a 3-day-old term male infant who was brought to the emergency department with symptoms of bilious emesis and poor feeding, and not having passed meconium. Pre-natal assessment and antenatal assessments did not raise any concerns for serology abnormalities or dysmorphic features, and he had good APGAR scores. The patient had a patent anus with abdominal distension. Initially, a plain abdominal X-ray was suspicious for Hirschsprung's disease due to narrowing of the terminal descending colon. On day 7 of life, he developed spontaneous bowel perforation. A right hemicolectomy was performed on the same day, and subsequent gross pathological assessment demonstrated a focal muscularis propria defect. Histological examination confirmed the presence of ganglion cells, ruling out Hirschsprung's disease. Immunohistochemical stains confirmed the absence of muscularis mucosa in small foci, which lead to a diagnosis of segmental absence of intestinal musculature (SAIM). While a repeat laparotomy was required shortly after due to complications, he is now feeding well and gaining weight appropriately.
Conclusion
Segmental absence of intestinal musculature should be included in the differential diagnosis of newborns with symptoms of functional intestinal obstruction in whom Hirschsprung's disease has been ruled out.
导言：肠道肌肉组织缺陷导致儿童肠梗阻和肠穿孔的病例非常罕见，人们对其了解甚少。我们的病例是一名出生 3 天的足月男婴，因胆汁淤积、喂养不畅和未排出胎粪等症状被送入急诊科。产前评估和产前检查均未发现血清学异常或畸形特征，APGAR 评分良好。患者肛门通畅，腹部膨胀。由于末端降结肠狭窄，腹部 X 光片初步怀疑是赫氏病。出生后第 7 天，他出现了自发性肠穿孔。当天对他进行了右半结肠切除术，随后的病理评估显示他有局灶性固有肌缺损。组织学检查证实了神经节细胞的存在，排除了赫氏病的可能性。免疫组化染色证实小灶内没有粘膜肌肉，因此诊断为节段性肠道肌肉缺失（SAIM）。结论对于有功能性肠梗阻症状且已排除赫氏病的新生儿，应将节段性肠肌缺失纳入鉴别诊断。
{"title":"Segmental absence of intestinal musculature in a newborn: A case report","authors":"Faiha Fareez , Daniella Rangira , Farah Abdulsatar , Miranda Schell , Sarab Hameed Mohamed","doi":"10.1016/j.epsc.2024.102878","DOIUrl":"10.1016/j.epsc.2024.102878","url":null,"abstract":"<div><h3>Introduction</h3><p>Intestinal musculature defects leading to intestinal obstruction and perforation in children are rare and poorly understood.</p></div><div><h3>Case presentation</h3><p>Our case is of a 3-day-old term male infant who was brought to the emergency department with symptoms of bilious emesis and poor feeding, and not having passed meconium. Pre-natal assessment and antenatal assessments did not raise any concerns for serology abnormalities or dysmorphic features, and he had good APGAR scores. The patient had a patent anus with abdominal distension. Initially, a plain abdominal X-ray was suspicious for Hirschsprung's disease due to narrowing of the terminal descending colon. On day 7 of life, he developed spontaneous bowel perforation. A right hemicolectomy was performed on the same day, and subsequent gross pathological assessment demonstrated a focal muscularis propria defect. Histological examination confirmed the presence of ganglion cells, ruling out Hirschsprung's disease. Immunohistochemical stains confirmed the absence of muscularis mucosa in small foci, which lead to a diagnosis of segmental absence of intestinal musculature (SAIM). While a repeat laparotomy was required shortly after due to complications, he is now feeding well and gaining weight appropriately.</p></div><div><h3>Conclusion</h3><p>Segmental absence of intestinal musculature should be included in the differential diagnosis of newborns with symptoms of functional intestinal obstruction in whom Hirschsprung's disease has been ruled out.</p></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"110 ","pages":"Article 102878"},"PeriodicalIF":0.2,"publicationDate":"2024-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2213576624001064/pdfft?md5=287b65789c6b98c5e05efd0f1d3b40ca&pid=1-s2.0-S2213576624001064-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142232568","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 : 2024-09-11DOI: 10.1016/j.epsc.2024.102882
Zachary Ballinger , Ezdean Alkurdi , Muriel Cleary , Jonathan Green , Kaitlyn Wong , Jeremy Aidlen
Introduction
Mediastinal teratomas present unique surgical challenges during resection. Here, we describe a novel use of Indocyanine Green (ICG) for real time identification of tumor borders and resection planes of a malignant mediastinal teratoma and right lung nodule.
Case presentation
A 13-year-old male with Klinefelter's Syndrome presented for right upper quadrant pain, difficulties walking and eating, and with elevated alpha-fetoprotein (AFP) and beta human chorionic gonadotropin (β-hCG). A CT scan demonstrated a lobulated right-sided mediastinal mass, as well as an isolated right middle lobe lung nodule. The patient underwent elective resection. He was injected with 3 mg/kg ICG one day prior to his planned operation. After median sternotomy, ICG imaging aided in the identification of the mediastinal mass, tumor borders and resection planes, particularly where the tumor was continuous with the right lobe of the thymus and along the deeper structures of the mediastinum. The right lung nodule was similarly visualized and resected. Pathologic examination of the mass revealed components of immature teratoma, choriocarcinoma, yolk sac tumor, and seminoma. The lung nodule demonstrated only chronic inflammation and compressive atelectasis from the tumor's mass effect. The patient recovered well and was discharged on post operative day 6. He follows with the oncology team for outpatient chemotherapy.
Conclusion
Though rare, teratomas in the mediastinum can occasionally be malignant and have the potential to involve adjacent structures, thereby creating a technical challenge at surgery. This case demonstrates the usefulness of ICG for intraoperative visualization of tumors to facilitate safe resection and improved outcomes.
{"title":"Indocyanine green guided resection of a mediastinal teratoma: A case report","authors":"Zachary Ballinger , Ezdean Alkurdi , Muriel Cleary , Jonathan Green , Kaitlyn Wong , Jeremy Aidlen","doi":"10.1016/j.epsc.2024.102882","DOIUrl":"10.1016/j.epsc.2024.102882","url":null,"abstract":"<div><h3>Introduction</h3><p>Mediastinal teratomas present unique surgical challenges during resection. Here, we describe a novel use of Indocyanine Green (ICG) for real time identification of tumor borders and resection planes of a malignant mediastinal teratoma and right lung nodule.</p></div><div><h3>Case presentation</h3><p>A 13-year-old male with Klinefelter's Syndrome presented for right upper quadrant pain, difficulties walking and eating, and with elevated alpha-fetoprotein (AFP) and beta human chorionic gonadotropin (β-hCG). A CT scan demonstrated a lobulated right-sided mediastinal mass, as well as an isolated right middle lobe lung nodule. The patient underwent elective resection. He was injected with 3 mg/kg ICG one day prior to his planned operation. After median sternotomy, ICG imaging aided in the identification of the mediastinal mass, tumor borders and resection planes, particularly where the tumor was continuous with the right lobe of the thymus and along the deeper structures of the mediastinum. The right lung nodule was similarly visualized and resected. Pathologic examination of the mass revealed components of immature teratoma, choriocarcinoma, yolk sac tumor, and seminoma. The lung nodule demonstrated only chronic inflammation and compressive atelectasis from the tumor's mass effect. The patient recovered well and was discharged on post operative day 6. He follows with the oncology team for outpatient chemotherapy.</p></div><div><h3>Conclusion</h3><p>Though rare, teratomas in the mediastinum can occasionally be malignant and have the potential to involve adjacent structures, thereby creating a technical challenge at surgery. This case demonstrates the usefulness of ICG for intraoperative visualization of tumors to facilitate safe resection and improved outcomes.</p></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"110 ","pages":"Article 102882"},"PeriodicalIF":0.2,"publicationDate":"2024-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2213576624001106/pdfft?md5=cbbd85495a8203881f4f514e61c7727a&pid=1-s2.0-S2213576624001106-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142232475","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 : 2024-09-10DOI: 10.1016/j.epsc.2024.102874
Juan R. Medina-Morell , Kenneth Avilés-Fernández , Zabeth Krystal Hernandez-Sanchez , Victor Ortiz-Justiniano
Introduction
Angiolipomatosis is a rare benign condition involving subcutaneous fat and blood vessels, particularly uncommon in pediatric patients.
Case presentation
An 11-year-old male presented with multiple firm, non-tender masses in the right gluteal and perianal regions, the largest measuring 8 cm for approximately 8 months duration. The patient underwent staged surgical excisions under general anesthesia due to extensive disease burden. In the first stage, perianal and medial gluteal masses were excised. Lipomatous masses were carefully dissected from the external anal sphincter to the posterior superior iliac spine (PSIS) with preservation of the external anal sphincter. The procedure was well tolerated, but the patient developed a postoperative wound infection, requiring intravenous antibiotics, debridement, and the creation of a loop colostomy to control wound contamination. Six weeks later, a second-stage excision removed the remaining gluteal masses, including a palpable mass measuring 8 x 4 × 2.5 cm. Hemostasis was achieved, and a Jackson-Pratt drain was placed to manage post-op wound fluid accumulation. The patient recovered well after both surgeries and at the 3-month follow-up, there were no complications. Pathological analysis confirmed angiolipomatosis in the gluteal region and diffuse lipomatosis in the perianal region. No recurrence was observed over a 3-year follow-up period.
Conclusion
Angiolipomatosis should be considered as a differential diagnosis for pediatric soft tissue masses in uncommon locations like the gluteal and perianal regions. Atypical presentations can be successfully managed with careful surgical planning helping to avoid unnecessary radical procedures. A staged approach is recommended when encountering diffuse disease, as in this case, and consideration should be given to diverting ostomy if wound contamination is a concern.
导言血管脂肪瘤病是一种罕见的累及皮下脂肪和血管的良性疾病，在儿童患者中尤为罕见。病例介绍一名 11 岁的男性患者因右侧臀部和肛周出现多个坚实、无触痛的肿块而就诊，最大的肿块达 8 厘米，病程约 8 个月。由于疾病范围广泛，患者在全身麻醉下接受了分期手术切除。在第一阶段，切除了肛周和臀部内侧肿块。在保留肛门外括约肌的情况下，从肛门外括约肌到髂后上棘（PSIS）仔细切除了脂肪瘤。患者对手术的耐受性良好，但术后出现伤口感染，需要静脉注射抗生素、清创，并建立环状结肠造口术以控制伤口污染。六周后，第二阶段切除术切除了剩余的臀部肿块，包括一个可触及的肿块，大小为 8 x 4 x 2.5 厘米。术中止血成功，并放置了杰克逊-普拉特引流管来处理术后伤口积液。两次手术后患者恢复良好，3个月随访时未出现并发症。病理分析证实臀部有血管脂肪瘤，肛周有弥漫性脂肪瘤。结论对于臀部和肛周等不常见部位的小儿软组织肿块，应将血管脂肪瘤病作为鉴别诊断。通过仔细的手术规划，可以成功处理非典型表现，避免不必要的根治性手术。在遇到像本病例这样的弥漫性疾病时，建议采用分期手术的方法，如果担心伤口污染，应考虑将造口引流。
{"title":"Perianal and gluteal lipomatosis in a child: A case report","authors":"Juan R. Medina-Morell , Kenneth Avilés-Fernández , Zabeth Krystal Hernandez-Sanchez , Victor Ortiz-Justiniano","doi":"10.1016/j.epsc.2024.102874","DOIUrl":"10.1016/j.epsc.2024.102874","url":null,"abstract":"<div><h3>Introduction</h3><p>Angiolipomatosis is a rare benign condition involving subcutaneous fat and blood vessels, particularly uncommon in pediatric patients.</p></div><div><h3>Case presentation</h3><p>An 11-year-old male presented with multiple firm, non-tender masses in the right gluteal and perianal regions, the largest measuring 8 cm for approximately 8 months duration. The patient underwent staged surgical excisions under general anesthesia due to extensive disease burden. In the first stage, perianal and medial gluteal masses were excised. Lipomatous masses were carefully dissected from the external anal sphincter to the posterior superior iliac spine (PSIS) with preservation of the external anal sphincter. The procedure was well tolerated, but the patient developed a postoperative wound infection, requiring intravenous antibiotics, debridement, and the creation of a loop colostomy to control wound contamination. Six weeks later, a second-stage excision removed the remaining gluteal masses, including a palpable mass measuring 8 x 4 × 2.5 cm. Hemostasis was achieved, and a Jackson-Pratt drain was placed to manage post-op wound fluid accumulation. The patient recovered well after both surgeries and at the 3-month follow-up, there were no complications. Pathological analysis confirmed angiolipomatosis in the gluteal region and diffuse lipomatosis in the perianal region. No recurrence was observed over a 3-year follow-up period.</p></div><div><h3>Conclusion</h3><p>Angiolipomatosis should be considered as a differential diagnosis for pediatric soft tissue masses in uncommon locations like the gluteal and perianal regions. Atypical presentations can be successfully managed with careful surgical planning helping to avoid unnecessary radical procedures. A staged approach is recommended when encountering diffuse disease, as in this case, and consideration should be given to diverting ostomy if wound contamination is a concern.</p></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"110 ","pages":"Article 102874"},"PeriodicalIF":0.2,"publicationDate":"2024-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2213576624001027/pdfft?md5=610a72bc4e7dba2d9f0ec228ec4209ba&pid=1-s2.0-S2213576624001027-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142232474","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 : 2024-09-10DOI: 10.1016/j.epsc.2024.102876
Miguel Serpa Irizarry , Gabriela Rosado-Gonzalez , Victor Ortiz-Justiniano
Introduction
Wolf-Hirschhorn Syndrome (WHS) is a rare genetic disorder caused by a partial deletion of the short arm of chromosome 4 (4p-). First described in 1961, this syndrome has a 2:1 female predominance and an estimated frequency of 1/50,000–1/20,000. The phenotypic spectrum of WHS is wide and encompasses multiple organ systems including intrauterine growth restriction, distinctive craniofacial anomalies, global developmental delay and intellectual disability.
Case presentation
We present the case of a hispanic male who was born at 39 WGA via C-Section to a 28 y/o G3P1A1 mother without past medical history. The patient experienced intrauterine growth restriction which triggered further prenatal evaluation revealing a 36.14 mb terminal deletion of 4p16.3- > p14 and he was diagnosed with Wolf-Hirschhorn Syndrome. At birth, physical exam was remarkable for a wide nose, cleft lip and cleft palate, hypertelorism, micrognathia, and bilateral corneal clouding. Musculoskeletal exam showed bilateral clubbed feet and genitourinary exam revealed, bifid scrotum with undescended testes. Anorectal examination was pertinent for an imperforate anus without associated fistulas or abscess. After chest x-ray, abdominopelvic ultrasound and echocardiogram ruled out any major contraindication for surgery, he was managed with end colostomy and mucus fistula for fecal diversion. Due to history of poor feedings during the postnatal period, gastrostomy tube placement was also performed. The patient was discharged home at day 26 without immediate postoperative complications.
Conclusion
We herein present possibly the first case of a male with WHS born with an imperforate anus that was managed surgically with end colostomy and mucus fistula. This case underscores the complexity and severity of WHS, by adding to its myriad of clinical manifestations and emphasizing the importance of reporting novel presentations and their management to improve care strategies for patients with WHS.
{"title":"“Wolf-Hirschhorn syndrome presenting with imperforate anus: A case report”","authors":"Miguel Serpa Irizarry , Gabriela Rosado-Gonzalez , Victor Ortiz-Justiniano","doi":"10.1016/j.epsc.2024.102876","DOIUrl":"10.1016/j.epsc.2024.102876","url":null,"abstract":"<div><h3>Introduction</h3><p>Wolf-Hirschhorn Syndrome (WHS) is a rare genetic disorder caused by a partial deletion of the short arm of chromosome 4 (4p-). First described in 1961, this syndrome has a 2:1 female predominance and an estimated frequency of 1/50,000–1/20,000. The phenotypic spectrum of WHS is wide and encompasses multiple organ systems including intrauterine growth restriction, distinctive craniofacial anomalies, global developmental delay and intellectual disability.</p></div><div><h3>Case presentation</h3><p>We present the case of a hispanic male who was born at 39 WGA via C-Section to a 28 y/o G3P1A1 mother without past medical history. The patient experienced intrauterine growth restriction which triggered further prenatal evaluation revealing a 36.14 mb terminal deletion of 4p16.3- > p14 and he was diagnosed with Wolf-Hirschhorn Syndrome. At birth, physical exam was remarkable for a wide nose, cleft lip and cleft palate, hypertelorism, micrognathia, and bilateral corneal clouding. Musculoskeletal exam showed bilateral clubbed feet and genitourinary exam revealed, bifid scrotum with undescended testes. Anorectal examination was pertinent for an imperforate anus without associated fistulas or abscess. After chest x-ray, abdominopelvic ultrasound and echocardiogram ruled out any major contraindication for surgery, he was managed with end colostomy and mucus fistula for fecal diversion. Due to history of poor feedings during the postnatal period, gastrostomy tube placement was also performed. The patient was discharged home at day 26 without immediate postoperative complications.</p></div><div><h3>Conclusion</h3><p>We herein present possibly the first case of a male with WHS born with an imperforate anus that was managed surgically with end colostomy and mucus fistula. This case underscores the complexity and severity of WHS, by adding to its myriad of clinical manifestations and emphasizing the importance of reporting novel presentations and their management to improve care strategies for patients with WHS.</p></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"110 ","pages":"Article 102876"},"PeriodicalIF":0.2,"publicationDate":"2024-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2213576624001040/pdfft?md5=2329af0652643f93fe44449c7df9a5e3&pid=1-s2.0-S2213576624001040-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142167298","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}
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