Pub Date : 2025-01-01DOI: 10.1016/j.epsc.2024.102938
Jennifer Milsten , Selin A. Weitkamp , Alexander G. Aghte , Joern-Hendrik Weitkamp
Introduction
Congenital teratomas affecting the head and neck region are rare, accounting for only 2–5% of all germ cell tumors. Congenital cervical teratomas occur in 1 per 20,000 to 40,000 live births. Due to their location, giant neck teratomas obstruct the airway and esophagus, leading to polyhydramnios and requiring an ex-utero intrapartum therapy (EXIT) procedure to allow survival.
Case presentation
A large fetal neck mass resulting in fetal neck extension and polyhydramnios was noted at 24 weeks' gestation by ultrasound and further characterized by fetal MRI. The infant was delivered by classical c-section for non-reassuring fetal heart rate status at 33 weeks’ gestational age. The mass was overlying both mandibles, extending down the neck, leading to extensive neck extension and obscuring anatomic landmarks. Resuscitation required puncturing vocal cord adhesions with a rigid bronchoscope and suctioning of airway fluid prior to intubation. The extensively vascularized mass was successfully resected at one week of age, when it weighed 1088 g and was 21cm × 12cm x 8 cm in dimension. Pathology was consistent with a grade 3, stage II mixed immature and mature teratoma including tissue derivatives of three germ layers.
Conclusion
Despite the unusually large size of this giant congenital neck teratoma, management by a multidisciplinary team with prenatal planning of resuscitation and postnatal treatment allowed for a positive outcome for the infant.
{"title":"Giant congenital cervical Teratoma: A case report","authors":"Jennifer Milsten , Selin A. Weitkamp , Alexander G. Aghte , Joern-Hendrik Weitkamp","doi":"10.1016/j.epsc.2024.102938","DOIUrl":"10.1016/j.epsc.2024.102938","url":null,"abstract":"<div><h3>Introduction</h3><div>Congenital teratomas affecting the head and neck region are rare, accounting for only 2–5% of all germ cell tumors. Congenital cervical teratomas occur in 1 per 20,000 to 40,000 live births. Due to their location, giant neck teratomas obstruct the airway and esophagus, leading to polyhydramnios and requiring an ex-utero intrapartum therapy (EXIT) procedure to allow survival.</div></div><div><h3>Case presentation</h3><div>A large fetal neck mass resulting in fetal neck extension and polyhydramnios was noted at 24 weeks' gestation by ultrasound and further characterized by fetal MRI. The infant was delivered by classical c-section for non-reassuring fetal heart rate status at 33 weeks’ gestational age. The mass was overlying both mandibles, extending down the neck, leading to extensive neck extension and obscuring anatomic landmarks. Resuscitation required puncturing vocal cord adhesions with a rigid bronchoscope and suctioning of airway fluid prior to intubation. The extensively vascularized mass was successfully resected at one week of age, when it weighed 1088 g and was 21cm × 12cm x 8 cm in dimension. Pathology was consistent with a grade 3, stage II mixed immature and mature teratoma including tissue derivatives of three germ layers.</div></div><div><h3>Conclusion</h3><div>Despite the unusually large size of this giant congenital neck teratoma, management by a multidisciplinary team with prenatal planning of resuscitation and postnatal treatment allowed for a positive outcome for the infant.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"112 ","pages":"Article 102938"},"PeriodicalIF":0.2,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143147597","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}
Diagnosis of diaphragmatic disease using dynamic chest radiography (DCR), a novel imaging testing modality has been previously reported in adults; however, there have been no prior reports of its use in pediatric patients. Herein, we report the novel case of a 14-year-old girl diagnosed with diaphragmatic eventration (DE) using DCR with extremely low-dose pulsed X-rays.
Case series
A 14-year-old girl with pectus excavatum was referred to our hospital for surgery. Pre-operative chest radiography revealed that the right diaphragm was higher than the left however, this difference was judged to be within the normal range. Computed tomography (CT) revealed a Haller index of 3.00. Although the patient had no symptoms, she complained of an unsatisfactory appearance, which was considered an indication for surgery. The Nuss procedure was performed under artificial pneumothorax, and DE was not suspected. Six months after surgery, we performed DCR instead of chest radiographs to evaluate the position of the bar and the patient's respiratory status. Utilizing the captured images, we further measured and analyzed the diaphragm movement. DCR revealed a clear impairment of diaphragmic movement on the affected side, and the patient was subsequently diagnosed with DE. However, as she was asymptomatic, we decided to follow up conservatively.
Conclusion
This is the first report of DCR in a pediatric patient. DCR appears to be a useful tool for the diagnosis of diaphragmatic eventration in children.
{"title":"Evaluation of pediatric diaphragmatic eventration using dynamic chest radiography: A case report","authors":"Takatoshi Abe , Kozo Nomura , Seisho Sakai , Rie Tanaka , Shuhei Minami , Hidenori Iwasaki , Yasuhiro Ikawa","doi":"10.1016/j.epsc.2024.102932","DOIUrl":"10.1016/j.epsc.2024.102932","url":null,"abstract":"<div><h3>Introduction</h3><div>Diagnosis of diaphragmatic disease using dynamic chest radiography (DCR), a novel imaging testing modality has been previously reported in adults; however, there have been no prior reports of its use in pediatric patients. Herein, we report the novel case of a 14-year-old girl diagnosed with diaphragmatic eventration (DE) using DCR with extremely low-dose pulsed X-rays.</div></div><div><h3>Case series</h3><div>A 14-year-old girl with pectus excavatum was referred to our hospital for surgery. Pre-operative chest radiography revealed that the right diaphragm was higher than the left however, this difference was judged to be within the normal range. Computed tomography (CT) revealed a Haller index of 3.00. Although the patient had no symptoms, she complained of an unsatisfactory appearance, which was considered an indication for surgery. The Nuss procedure was performed under artificial pneumothorax, and DE was not suspected. Six months after surgery, we performed DCR instead of chest radiographs to evaluate the position of the bar and the patient's respiratory status. Utilizing the captured images, we further measured and analyzed the diaphragm movement. DCR revealed a clear impairment of diaphragmic movement on the affected side, and the patient was subsequently diagnosed with DE. However, as she was asymptomatic, we decided to follow up conservatively.</div></div><div><h3>Conclusion</h3><div>This is the first report of DCR in a pediatric patient. DCR appears to be a useful tool for the diagnosis of diaphragmatic eventration in children.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"112 ","pages":"Article 102932"},"PeriodicalIF":0.2,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143147610","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 : 2025-01-01DOI: 10.1016/j.epsc.2024.102930
Lori-Anne Lajeunesse , Mona Beaunoyer , Monia Marzouki , Caroline P. Lemoine
Introduction
Despite a high mortality rate during the first year of life, an increasing number of patients with Trisomy 18 (T18) survive into childhood. Offering invasive medical and surgical procedures to this population continues to raise ethical questions. We present the case of a child with T18 and hepatoblastoma, highlighting therapeutic and ethical challenges.
Case presentation
A 2.5-year-old female with T18 known for a bicuspid aortic valve and developmental delay presented with an abdominal mass. Alpha-fetoprotein level was elevated (46,540 ng/mL) and imaging showed a single exophytic segment 5–6 liver mass and no metastatic disease, compatible with a PRETEXT II hepatoblastoma. Multidisciplinary discussions took place focusing on treatment options, the patient's quality of life, and her global prognosis. Given the patient's overall good condition, few comorbidities, and the family's wishes to proceed with curative measures, a bisegmentectomy 5,6 was performed. The patient was discharged 12 days postoperatively having suffered no surgical complication. Pathology revealed a mixed epithelial and mesenchymal hepatoblastoma. She received two cycles of adjuvant chemotherapy (cisplatin) without suffering from any complication. Now 5.5 years post-treatment, she remains disease-free. The family is grateful their daughter's chromosomal anomaly did not negatively influence medical and surgical teams in offering her optimal treatment options.
Conclusion
Unless suffering from severe underlying medical comorbidities, patients with T18 and hepatoblastoma can receive gold standard care. Multidisciplinary collaboration involving surgeons, medical providers, as well as families are essential to determine optimal individualized treatment strategies.
{"title":"Balanced care in managing hepatoblastoma in a patient with trisomy 18: A case report","authors":"Lori-Anne Lajeunesse , Mona Beaunoyer , Monia Marzouki , Caroline P. Lemoine","doi":"10.1016/j.epsc.2024.102930","DOIUrl":"10.1016/j.epsc.2024.102930","url":null,"abstract":"<div><h3>Introduction</h3><div>Despite a high mortality rate during the first year of life, an increasing number of patients with Trisomy 18 (T18) survive into childhood. Offering invasive medical and surgical procedures to this population continues to raise ethical questions. We present the case of a child with T18 and hepatoblastoma, highlighting therapeutic and ethical challenges.</div></div><div><h3>Case presentation</h3><div>A 2.5-year-old female with T18 known for a bicuspid aortic valve and developmental delay presented with an abdominal mass. Alpha-fetoprotein level was elevated (46,540 ng/mL) and imaging showed a single exophytic segment 5–6 liver mass and no metastatic disease, compatible with a PRETEXT II hepatoblastoma. Multidisciplinary discussions took place focusing on treatment options, the patient's quality of life, and her global prognosis. Given the patient's overall good condition, few comorbidities, and the family's wishes to proceed with curative measures, a bisegmentectomy 5,6 was performed. The patient was discharged 12 days postoperatively having suffered no surgical complication. Pathology revealed a mixed epithelial and mesenchymal hepatoblastoma. She received two cycles of adjuvant chemotherapy (cisplatin) without suffering from any complication. Now 5.5 years post-treatment, she remains disease-free. The family is grateful their daughter's chromosomal anomaly did not negatively influence medical and surgical teams in offering her optimal treatment options.</div></div><div><h3>Conclusion</h3><div>Unless suffering from severe underlying medical comorbidities, patients with T18 and hepatoblastoma can receive gold standard care. Multidisciplinary collaboration involving surgeons, medical providers, as well as families are essential to determine optimal individualized treatment strategies.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"112 ","pages":"Article 102930"},"PeriodicalIF":0.2,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143147596","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 : 2025-01-01DOI: 10.1016/j.epsc.2024.102935
Suleiman Ayalew , Michael A. Negussie , Hiwot Tesfaselassie Afework , Dagnachew Akalu Kassie , Abebe Molla Mogess , Mesfin Tesera Wassie
Introduction
Pentalogy of Cantrell is a rare congenital condition involving defects in the abdominal wall, sternum, diaphragm, pericardium, and heart. This report adds to the scarce literature by presenting a unique case from a resource-limited setting.
Case presentation
An 8-h-old male neonate was admitted to the NICU with a thoracoabdominal defect observed at birth. Delivered in transit at term to a healthy mother with no prenatal anomalies identified, the neonate exhibited a pulsatile mass from the xiphisternum to the umbilicus, covered by a thin membrane with visible cardiac activity. Imaging and clinical evaluation confirmed herniation of the heart and liver through the defect, consistent with ectopia cordis and omphalocele, accompanied by a sternal defect and diaphragmatic communication. Echocardiography revealed severe intracardiac anomalies, including a single ventricle, single atrium, truncus arteriosus, and mild pericardial effusion. Additionally, a meningomyelocele was identified alongside bilateral clubfoot deformities. Initial management included stabilization with fluids, antibiotics, oxygen, and protective dressings for the exposed structures. The neonate experienced cardiac arrest on the third day of admission, and surgical intervention was not performed.
Conclusion
Prenatal screening and prompt multidisciplinary intervention are essential to improve outcomes in cases of Pentalogy of Cantrell.
{"title":"Pentalogy of Cantrell in a resource-limited setting: A case report","authors":"Suleiman Ayalew , Michael A. Negussie , Hiwot Tesfaselassie Afework , Dagnachew Akalu Kassie , Abebe Molla Mogess , Mesfin Tesera Wassie","doi":"10.1016/j.epsc.2024.102935","DOIUrl":"10.1016/j.epsc.2024.102935","url":null,"abstract":"<div><h3>Introduction</h3><div>Pentalogy of Cantrell is a rare congenital condition involving defects in the abdominal wall, sternum, diaphragm, pericardium, and heart. This report adds to the scarce literature by presenting a unique case from a resource-limited setting.</div></div><div><h3>Case presentation</h3><div>An 8-h-old male neonate was admitted to the NICU with a thoracoabdominal defect observed at birth. Delivered in transit at term to a healthy mother with no prenatal anomalies identified, the neonate exhibited a pulsatile mass from the xiphisternum to the umbilicus, covered by a thin membrane with visible cardiac activity. Imaging and clinical evaluation confirmed herniation of the heart and liver through the defect, consistent with ectopia cordis and omphalocele, accompanied by a sternal defect and diaphragmatic communication. Echocardiography revealed severe intracardiac anomalies, including a single ventricle, single atrium, truncus arteriosus, and mild pericardial effusion. Additionally, a meningomyelocele was identified alongside bilateral clubfoot deformities. Initial management included stabilization with fluids, antibiotics, oxygen, and protective dressings for the exposed structures. The neonate experienced cardiac arrest on the third day of admission, and surgical intervention was not performed.</div></div><div><h3>Conclusion</h3><div>Prenatal screening and prompt multidisciplinary intervention are essential to improve outcomes in cases of Pentalogy of Cantrell.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"112 ","pages":"Article 102935"},"PeriodicalIF":0.2,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143147609","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}
While ascariasis is typically asymptomatic or presents with mild gastrointestinal symptoms, it can lead to severe complications, such as intestinal obstruction and volvulus, particularly in children with a high worm burden.
Case presentation
A 9-year-old girl presented to the pediatric emergency department with four days of central abdominal pain, repeated bilious vomiting containing worms, increasing abdominal distension, constipation, and a low-grade fever. She was taken to the operating room for an exploratory laparotomy for suspected bowel obstruction. Intraoperatively, 300 mL of pus was found in the peritoneal cavity, along with a 360-degree gangrenous volvulus of small bowel involving most of the small bowel, from 80 cm distal to the ligament of Treitz to 20 cm proximal to the ileocecal valve. Adhesions and fibrin deposits were noted throughout the peritoneum. The purulent fluid was suctioned out, and the ischemic bowel was completely resected. A large burden of Ascaris worms was found in the lumen of the bowel and was completely removed. We did a jejunostomy and an ileal mucous fistula. Postoperatively, the patient received intravenous ceftriaxone, metronidazole, potassium supplements, and maintenance fluids. Due to the high output of the jejunostomy, we decided to take down the jejunostomy shortly after the initial operation. The ostomy closure was complicated by an anastomotic leak. We explored her and found a pin hole on the antimesenteric side of the anastomosis. We repaired the leak primarily, but the patient developed a recurrence of the leak shortly thereafter, at which time we decided tore-create the jejunostomy and the mucous fistula. Before we could provide further care, her parents decided to leave the hospital against medical advice. We eventually contacted the parents and learned that she died at another institution 10 days after leaving our hospital. The cause of her death was not disclosed.
Conclusion
Even though intestinal infestations are generally mildly symptomatic, Ascariasis and other parasitic infestations should be included in the differential diagnosis of children who live in endemic areas and develop acute abdominal conditions.
{"title":"Gangrenous small bowel volvulus due to ascariasis in a 9-year-old female: A case report","authors":"Bethlehem Aliye Asfaw , Kinfemicheal Tilahun Yigzaw , Om Prakash Bhatta , Yonathan Aliye Asfaw , Bewketu Abate Fenta , Mesfin Tesera Wassie","doi":"10.1016/j.epsc.2024.102936","DOIUrl":"10.1016/j.epsc.2024.102936","url":null,"abstract":"<div><h3>Introduction</h3><div>While ascariasis is typically asymptomatic or presents with mild gastrointestinal symptoms, it can lead to severe complications, such as intestinal obstruction and volvulus, particularly in children with a high worm burden.</div></div><div><h3>Case presentation</h3><div>A 9-year-old girl presented to the pediatric emergency department with four days of central abdominal pain, repeated bilious vomiting containing worms, increasing abdominal distension, constipation, and a low-grade fever. She was taken to the operating room for an exploratory laparotomy for suspected bowel obstruction. Intraoperatively, 300 mL of pus was found in the peritoneal cavity, along with a 360-degree gangrenous volvulus of small bowel involving most of the small bowel, from 80 cm distal to the ligament of Treitz to 20 cm proximal to the ileocecal valve. Adhesions and fibrin deposits were noted throughout the peritoneum. The purulent fluid was suctioned out, and the ischemic bowel was completely resected. A large burden of Ascaris worms was found in the lumen of the bowel and was completely removed. We did a jejunostomy and an ileal mucous fistula. Postoperatively, the patient received intravenous ceftriaxone, metronidazole, potassium supplements, and maintenance fluids. Due to the high output of the jejunostomy, we decided to take down the jejunostomy shortly after the initial operation. The ostomy closure was complicated by an anastomotic leak. We explored her and found a pin hole on the antimesenteric side of the anastomosis. We repaired the leak primarily, but the patient developed a recurrence of the leak shortly thereafter, at which time we decided tore-create the jejunostomy and the mucous fistula. Before we could provide further care, her parents decided to leave the hospital against medical advice. We eventually contacted the parents and learned that she died at another institution 10 days after leaving our hospital. The cause of her death was not disclosed.</div></div><div><h3>Conclusion</h3><div>Even though intestinal infestations are generally mildly symptomatic, Ascariasis and other parasitic infestations should be included in the differential diagnosis of children who live in endemic areas and develop acute abdominal conditions.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"112 ","pages":"Article 102936"},"PeriodicalIF":0.2,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143147598","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 : 2024-12-31DOI: 10.1016/j.epsc.2024.102955
M. Savadier, N. Maistry, D.S. Harrison
Introduction
Tumoral Calcinosis is a rare, benign metabolic disorder, characterized by the deposition of calcium phosphate crystals in extra-articular soft tissues. Less than 400 cases have been described globally, with only around 10 % being described in the pediatric population. Large bilateral masses are exceedingly rare.
Case report
We present a case of an 8-year-old female with bilateral 15 × 10cm posterior chest wall masses, who presented following a one-year history of enlargement. The patient had no medical or family history. A diagnosis of primary hyperphosphataemic tumoral calcinosis was suspected based on biochemistry and imaging. The patient had a raised phosphate and normal parathyroid hormone level, while ultrasound and Computed Tomography scans showed multilocular, calcified lesions with solid and cystic components. Core needle biopsy confirmed a diagnosis of tumoral calcinosis. Staged surgery was undertaken to resect the masses, which had extensive regional invasion. Clear margins were attained, with a rim of normal tissue on histology. The patient developed recurrent bilateral 15 × 20cm masses one year later, having defaulted post-operative treatment with Acetazolamide and Aluminium Hydroxide. Further surgery and medical management with the above agents was required. Six months post-surgery for the recurrence, no further recurrence was noted, but following poor adherence to her medical therapy another year later, there has been recurrence on the right, measuring 5 × 5cm. Treatment with Acetazolamide and Aluminium Hydroxide continue, with close observation for further growth.
Conclusion
A combination of targeted medical therapy with Acetazolamide and Aluminium Hydroxide, and early surgical resection, may limit the risk of recurrence in hyperphosphatemic tumoral calcinosis in the pediatric population.
{"title":"Tumoral calcinosis in an 8-year-old girl: A case report","authors":"M. Savadier, N. Maistry, D.S. Harrison","doi":"10.1016/j.epsc.2024.102955","DOIUrl":"10.1016/j.epsc.2024.102955","url":null,"abstract":"<div><h3>Introduction</h3><div>Tumoral Calcinosis is a rare, benign metabolic disorder, characterized by the deposition of calcium phosphate crystals in extra-articular soft tissues. Less than 400 cases have been described globally, with only around 10 % being described in the pediatric population. Large bilateral masses are exceedingly rare.</div></div><div><h3>Case report</h3><div>We present a case of an 8-year-old female with bilateral 15 × 10cm posterior chest wall masses, who presented following a one-year history of enlargement. The patient had no medical or family history. A diagnosis of primary hyperphosphataemic tumoral calcinosis was suspected based on biochemistry and imaging. The patient had a raised phosphate and normal parathyroid hormone level, while ultrasound and Computed Tomography scans showed multilocular, calcified lesions with solid and cystic components. Core needle biopsy confirmed a diagnosis of tumoral calcinosis. Staged surgery was undertaken to resect the masses, which had extensive regional invasion. Clear margins were attained, with a rim of normal tissue on histology. The patient developed recurrent bilateral 15 × 20cm masses one year later, having defaulted post-operative treatment with Acetazolamide and Aluminium Hydroxide. Further surgery and medical management with the above agents was required. Six months post-surgery for the recurrence, no further recurrence was noted, but following poor adherence to her medical therapy another year later, there has been recurrence on the right, measuring 5 × 5cm. Treatment with Acetazolamide and Aluminium Hydroxide continue, with close observation for further growth.</div></div><div><h3>Conclusion</h3><div>A combination of targeted medical therapy with Acetazolamide and Aluminium Hydroxide, and early surgical resection, may limit the risk of recurrence in hyperphosphatemic tumoral calcinosis in the pediatric population.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"114 ","pages":"Article 102955"},"PeriodicalIF":0.2,"publicationDate":"2024-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143148293","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 : 2024-12-27DOI: 10.1016/j.epsc.2024.102949
Yidnekachew Getachew , Jejaw Endale , Mihret S. Tesfaye , Hiwote M. Tsega , Azeb Mulugeta
Introduction
Concurrent esophageal atresia with tracheoesophageal fistula and Hirschsprung disease is a rare combination of congenital anomalies, with only four cases reported to date in full-term neonates.
Case presentation
A 4-day-old full-term neonate from rural Ethiopia was brought to our pediatric surgical unit with symptoms of respiratory distress and excessive frothy secretions. Chest exam revealed intercostal and subcostal retractions along with crepitation in the right posterior chest. The abdomen was distended, and a positive blast sign was noted during the rectal exam. Echocardiography showed a small patent ductus arteriosus. We attempted to pass an orogastric tube, but it was not possible. Chest and abdominal X-rays showed air in the stomach and the orogastric tube in the upper esophagus, suspicious for an esophageal atresia with a tracheoesophageal fistula. The patient had persistent thrombocytopenia, requiring multiple platelet transfusions, and was treated for aspiration pneumonia before he was deemed stable to undergo an operation. He received daily maintenance fluids with electrolytes for nutritional support and underwent daily rectal irrigations. On day of life 17 he was taken to the operating room. We found and ligated a distal tracheoesophageal fistula and did an end-to-end esophageal anastomosis via right thoracotomy. Because of the ongoing need for daily rectal irrigations, we also did a full thickness transanal rectal biopsy and confirmed the diagnosis of Hirschsprung disease. Postoperatively, the patient had no complications and has since started breastfeeding. He continues to receive rectal stimulation and irrigation and is on the wait list for his pull-through operation.
Conclusion
A high index of suspicion for Hirschsprung disease is crucial in patients with esophageal atresia and tracheoesophageal fistula, delayed meconium passage and lack of spontaneous bowel function.
{"title":"Concurrent esophageal atresia with tracheoesophageal fistula and hirschsprung disease in a term neonate: A case report","authors":"Yidnekachew Getachew , Jejaw Endale , Mihret S. Tesfaye , Hiwote M. Tsega , Azeb Mulugeta","doi":"10.1016/j.epsc.2024.102949","DOIUrl":"10.1016/j.epsc.2024.102949","url":null,"abstract":"<div><h3>Introduction</h3><div>Concurrent esophageal atresia with tracheoesophageal fistula and Hirschsprung disease is a rare combination of congenital anomalies, with only four cases reported to date in full-term neonates.</div></div><div><h3>Case presentation</h3><div>A 4-day-old full-term neonate from rural Ethiopia was brought to our pediatric surgical unit with symptoms of respiratory distress and excessive frothy secretions. Chest exam revealed intercostal and subcostal retractions along with crepitation in the right posterior chest. The abdomen was distended, and a positive blast sign was noted during the rectal exam. Echocardiography showed a small patent ductus arteriosus. We attempted to pass an orogastric tube, but it was not possible. Chest and abdominal X-rays showed air in the stomach and the orogastric tube in the upper esophagus, suspicious for an esophageal atresia with a tracheoesophageal fistula. The patient had persistent thrombocytopenia, requiring multiple platelet transfusions, and was treated for aspiration pneumonia before he was deemed stable to undergo an operation. He received daily maintenance fluids with electrolytes for nutritional support and underwent daily rectal irrigations. On day of life 17 he was taken to the operating room. We found and ligated a distal tracheoesophageal fistula and did an end-to-end esophageal anastomosis via right thoracotomy. Because of the ongoing need for daily rectal irrigations, we also did a full thickness transanal rectal biopsy and confirmed the diagnosis of Hirschsprung disease. Postoperatively, the patient had no complications and has since started breastfeeding. He continues to receive rectal stimulation and irrigation and is on the wait list for his pull-through operation.</div></div><div><h3>Conclusion</h3><div>A high index of suspicion for Hirschsprung disease is crucial in patients with esophageal atresia and tracheoesophageal fistula, delayed meconium passage and lack of spontaneous bowel function.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"114 ","pages":"Article 102949"},"PeriodicalIF":0.2,"publicationDate":"2024-12-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143147679","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 : 2024-12-26DOI: 10.1016/j.epsc.2024.102951
Fereshteh Moshfegh , Zahra Hosseinzade , Mohammad Javanbakht , Shahla Ansari , Ali Manafi Anari , Khatere Tavajohi
Introduction
Pleuropulmonary blastoma (PPB) in children can present with symptoms such as chest or abdominal pain, cough, fever, and respiratory distress, often leading to misdiagnosis as infections, pneumothorax, or pneumonia.
Case presentation
A 4-year-old male presented with generalized abdominal pain, hematuria, and respiratory symptoms. chest pain, cough, fever, and chills. Despite outpatient treatment with antibiotics, his condition failed to improve, and he was admitted to the hospital. Chest X-ray showed that the right hemithorax was whited out. Chest ultrasound a mass-like area in the right lower lobe. A chest computed tomography (CT) showed a large pleural effusion and a completely collapsed right lung. Laboratory tests showed elevated inflammatory markers and leukocytosis. Contrast-enhanced computed tomography (CECT) showed a large, well-defined heterogeneous mass measuring 98 × 86 mm in the right hemithorax. He was taken to the operating room for a resection. Through a right thoracotomy we found and resected a tumor measuring 3 × 14 × 16 cm arising from the lower lobe, and we resected a small portion of a rib that appeared involved by the tumor. Postoperative studies showed metastatic disease in several ribs. Pleural fluid cytology showed malignant cells, and the histopathology of the specimen confirmed the diagnosis of a type III PPB. Genetic testing was positive for a DICER1 mutation. Chemotherapy with ifosfamide, vincristine, actinomycin-D and doxorubicin was initiated five days after diagnosis. He currently continues his treatment.
Conclusion
Chest tumors should be suspected in patients that present respiratory symptoms that do not respond to standard measures and have abnormal findings on plain chest films. PPB should be suspected if a lung mass is found. Patients with confirmed PPB must undergo genetic testing to rule out DICER1 mutations, which are associated with a variety of other malignancies.
{"title":"Pleuropulmonary blastoma in A 4-year-Old male: A case report","authors":"Fereshteh Moshfegh , Zahra Hosseinzade , Mohammad Javanbakht , Shahla Ansari , Ali Manafi Anari , Khatere Tavajohi","doi":"10.1016/j.epsc.2024.102951","DOIUrl":"10.1016/j.epsc.2024.102951","url":null,"abstract":"<div><h3>Introduction</h3><div>Pleuropulmonary blastoma (PPB) in children can present with symptoms such as chest or abdominal pain, cough, fever, and respiratory distress, often leading to misdiagnosis as infections, pneumothorax, or pneumonia.</div></div><div><h3>Case presentation</h3><div>A 4-year-old male presented with generalized abdominal pain, hematuria, and respiratory symptoms. chest pain, cough, fever, and chills. Despite outpatient treatment with antibiotics, his condition failed to improve, and he was admitted to the hospital. Chest X-ray showed that the right hemithorax was whited out. Chest ultrasound a mass-like area in the right lower lobe. A chest computed tomography (CT) showed a large pleural effusion and a completely collapsed right lung. Laboratory tests showed elevated inflammatory markers and leukocytosis. Contrast-enhanced computed tomography (CECT) showed a large, well-defined heterogeneous mass measuring 98 × 86 mm in the right hemithorax. He was taken to the operating room for a resection. Through a right thoracotomy we found and resected a tumor measuring 3 × 14 × 16 cm arising from the lower lobe, and we resected a small portion of a rib that appeared involved by the tumor. Postoperative studies showed metastatic disease in several ribs. Pleural fluid cytology showed malignant cells, and the histopathology of the specimen confirmed the diagnosis of a type III PPB. Genetic testing was positive for a DICER1 mutation. Chemotherapy with ifosfamide, vincristine, actinomycin-D and doxorubicin was initiated five days after diagnosis. He currently continues his treatment.</div></div><div><h3>Conclusion</h3><div>Chest tumors should be suspected in patients that present respiratory symptoms that do not respond to standard measures and have abnormal findings on plain chest films. PPB should be suspected if a lung mass is found. Patients with confirmed PPB must undergo genetic testing to rule out DICER1 mutations, which are associated with a variety of other malignancies.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"114 ","pages":"Article 102951"},"PeriodicalIF":0.2,"publicationDate":"2024-12-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143148296","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 : 2024-12-24DOI: 10.1016/j.epsc.2024.102947
Ahmed Eldeib , Carlos A. Reck , Koby Herman , Emily Yang , Stefani Manis , Francisca Velcek
Introduction
Sacrococcygeal teratomas (SCTs) are rare germ cell tumors, predominantly diagnosed in neonates. These tumors are categorized as mature, immature, or malignant, with mature teratomas posing a lower risk for malignant transformation. While symptoms commonly arise from the mass effect, late presentation in adolescents or adults is rare.
Case presentation
A 16-year-old female with no significant past medical or surgical history presented with perianal pain, constipation, and fever. On physical exam, the rectum was collapsed by a mass. The laboratory work-up showed leukocytosis and an elevated C-reactive protein. Lactate dehydrogenase (LDH), alpha-fetoprotein (AFP), and beta-human chorionic gonadotropin (β-HCG) levels were within normal limits. A CT scan of the abdomen and pelvis revealed a 12 x 16 × 16 cm complex mass in the presacral region. Further evaluation with a pelvic MRI confirmed the presence of a multiloculated mass with mixed solid and cystic components, rim enhancement, and possible coccygeal involvement suggestive of a sacrococcygeal teratoma. Given the size and location of the mass, we decided for a combined abdomino-perineo-sacral surgical approach. The patient received Ceftriaxone and Metronidazole before the operation. We started with a midline laparotomy and detached the tumor from the intra-abdominal structures, and we continued with a posterior sagittal approach. The tumor appearance was consistent with a mature teratoma and had purulent fluid within the cystic component. We removed the mass completely and did a coccygectomy. The postoperative course was complicated by a retroperitoneal collection and a posterior wound dehiscence that required several washouts, debridement, vacuum-assisted closure therapy, and a long course of antibiotics. She was discharged home on postoperative day 32. She is being followed with yearly MRIs.
Conclusion
Superinfection of mature teratomas can lead to postoperative infectious complications. Pre-operative and postoperative antibiotics are recommended when resecting mature teratomas.
{"title":"Late presentation of a Type IV sacrococcygeal teratoma in a 16-year-old female: A case report","authors":"Ahmed Eldeib , Carlos A. Reck , Koby Herman , Emily Yang , Stefani Manis , Francisca Velcek","doi":"10.1016/j.epsc.2024.102947","DOIUrl":"10.1016/j.epsc.2024.102947","url":null,"abstract":"<div><h3>Introduction</h3><div>Sacrococcygeal teratomas (SCTs) are rare germ cell tumors, predominantly diagnosed in neonates. These tumors are categorized as mature, immature, or malignant, with mature teratomas posing a lower risk for malignant transformation. While symptoms commonly arise from the mass effect, late presentation in adolescents or adults is rare.</div></div><div><h3>Case presentation</h3><div>A 16-year-old female with no significant past medical or surgical history presented with perianal pain, constipation, and fever. On physical exam, the rectum was collapsed by a mass. The laboratory work-up showed leukocytosis and an elevated C-reactive protein. Lactate dehydrogenase (LDH), alpha-fetoprotein (AFP), and beta-human chorionic gonadotropin (β-HCG) levels were within normal limits. A CT scan of the abdomen and pelvis revealed a 12 x 16 × 16 cm complex mass in the presacral region. Further evaluation with a pelvic MRI confirmed the presence of a multiloculated mass with mixed solid and cystic components, rim enhancement, and possible coccygeal involvement suggestive of a sacrococcygeal teratoma. Given the size and location of the mass, we decided for a combined abdomino-perineo-sacral surgical approach. The patient received Ceftriaxone and Metronidazole before the operation. We started with a midline laparotomy and detached the tumor from the intra-abdominal structures, and we continued with a posterior sagittal approach. The tumor appearance was consistent with a mature teratoma and had purulent fluid within the cystic component. We removed the mass completely and did a coccygectomy. The postoperative course was complicated by a retroperitoneal collection and a posterior wound dehiscence that required several washouts, debridement, vacuum-assisted closure therapy, and a long course of antibiotics. She was discharged home on postoperative day 32. She is being followed with yearly MRIs.</div></div><div><h3>Conclusion</h3><div>Superinfection of mature teratomas can lead to postoperative infectious complications. Pre-operative and postoperative antibiotics are recommended when resecting mature teratomas.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"114 ","pages":"Article 102947"},"PeriodicalIF":0.2,"publicationDate":"2024-12-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143148245","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 : 2024-12-20DOI: 10.1016/j.epsc.2024.102946
Nathalie Carey , Luis H. Braga , Bruno Leslie , J Mark Walton , Michael H. Livingston
Introduction
Cloaca with a common channel greater than 3 cm typically requires urogenital separation rather than total urogenital mobilization. The purpose of this report was to describe our experience managing a female infant with an usual form of cloaca where all structures inserted onto the bladder neck.
Case presentation
A female infant with a single perineal opening underwent loop sigmoid colostomy shortly after birth. She voided spontaneously and had no hydrocolpos. She underwent exam under anesthesia, cystoscopy, and cloacogram under a single anesthetic at 5 months of age. This revealed five structures that inserted directly into the bladder: a central rectal fistula, two hemivaginas and hemiuteri bilaterally, and two ureters without hydronephrosis. The outflow tract was a long common channel measuring 5.2 cm. This patient was reviewed in a multidisciplinary setting and underwent repair at 10 months of age. We performed a midline laparotomy and opened the anterior wall of the bladder to visualize the structures that inserted posteriorly. Foley catheter and ureteric stents were placed. The rectal fistula and hemivaginas were mobilized off the bladder internally. Vaginal replacement was performed using a transverse portion of rectum. Additional colonic length was achieved by converting the loop colostomy to an end-loop. The abdomen was closed and posterior sagittal anorectoplasty was performed in a prone position.
Conclusion
This infant underwent repair of a “bladder neck” cloaca using a transvesicular approach. She is now 35 months of age and thriving. She developed neurogenic bladder requiring clean intermittent catheterization and is working on fecal continence.
{"title":"Repair of “bladder neck” cloaca using a trans-vesicle approach: A case report","authors":"Nathalie Carey , Luis H. Braga , Bruno Leslie , J Mark Walton , Michael H. Livingston","doi":"10.1016/j.epsc.2024.102946","DOIUrl":"10.1016/j.epsc.2024.102946","url":null,"abstract":"<div><h3>Introduction</h3><div>Cloaca with a common channel greater than 3 cm typically requires urogenital separation rather than total urogenital mobilization. The purpose of this report was to describe our experience managing a female infant with an usual form of cloaca where all structures inserted onto the bladder neck.</div></div><div><h3>Case presentation</h3><div>A female infant with a single perineal opening underwent loop sigmoid colostomy shortly after birth. She voided spontaneously and had no hydrocolpos. She underwent exam under anesthesia, cystoscopy, and cloacogram under a single anesthetic at 5 months of age. This revealed five structures that inserted directly into the bladder: a central rectal fistula, two hemivaginas and hemiuteri bilaterally, and two ureters without hydronephrosis. The outflow tract was a long common channel measuring 5.2 cm. This patient was reviewed in a multidisciplinary setting and underwent repair at 10 months of age. We performed a midline laparotomy and opened the anterior wall of the bladder to visualize the structures that inserted posteriorly. Foley catheter and ureteric stents were placed. The rectal fistula and hemivaginas were mobilized off the bladder internally. Vaginal replacement was performed using a transverse portion of rectum. Additional colonic length was achieved by converting the loop colostomy to an end-loop. The abdomen was closed and posterior sagittal anorectoplasty was performed in a prone position.</div></div><div><h3>Conclusion</h3><div>This infant underwent repair of a “bladder neck” cloaca using a transvesicular approach. She is now 35 months of age and thriving. She developed neurogenic bladder requiring clean intermittent catheterization and is working on fecal continence.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"114 ","pages":"Article 102946"},"PeriodicalIF":0.2,"publicationDate":"2024-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143147674","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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