Journal of Pediatric Surgery Case Reports最新文献

{"title":"Rare urogenital malformation with prune belly syndrome in a newborn female: A case report","authors":"Melanie Elhafid,&nbsp;Kristopher Milbrandt","doi":"10.1016/j.epsc.2025.102973","DOIUrl":"10.1016/j.epsc.2025.102973","url":null,"abstract":"<div><h3>Introduction</h3><div>Prune Belly syndrome (PBS) is a rare congenital anomaly almost exclusive to males. It includes a triad of urinary tract abnormality, lack of abdominal muscles, and cryptorchidism. Urorectal septum malformation sequence (URSMS) is an even more rare congenital anomaly defined by the presence of ambiguous genitalia, absent perineal openings, and lumbosacral abnormalities. The association of PBS and elements of URSMS in a female patient is extremely rare.</div></div><div><h3>Case presentation</h3><div>A female newborn was born at 31 weeks of gestation by vaginal delivery to a multipara 23-year-old woman, who smoked one pack of cigarettes per day during the pregnancy. A prenatal ultrasound done at 25 weeks of gestation showed a cyst in the abdominal cavity, but further evaluation did not occur. Apgar scores were 7 and 7 at 1 and 5 minutes, respectively, and the patient was intubated due to respiratory distress. The initial physical exam revealed global hypotonia, a distended abdomen with deficient abdominal musculature, ambiguous genitalia, and an imperforate anus. With a provisional diagnosis of PBS the patient was transported to our center. An abdominal ultrasound (US) done on the first day of life (DOL) showed a 13.8x 5.7 × 8.8cm cystic abdominal mass, and dilated ureters but no hydronephrosis. We placed a percutaneous suprapubic catheter on DOL1 and took her to the operating room on DOL6 for an exploratory laparotomy plus colostomy. We found the bladder and the uterus fussed together. The colon was seen ending in the fused uterus/vagina, but there was no meconium in the lumen of the bladder. We transected the colon, created a colostomy with the proximal end, and drained the distal end with a Malecot tube. Further postoperative work-up revealed several other anomalies including bilateral intraventricular hemorrhages and polydactyly. Due to refractory ascites and worsening pulmonary hypertension her respiratory requirements continued to increase. Due to the multiple comorbidities, the family decided to withdraw care at the age of 51 days.</div></div><div><h3>Conclusion</h3><div>The prognosis of PBS with URSMS features in females is poor. Early diagnosis is critical to provide appropriate family counselling.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"115 ","pages":"Article 102973"},"PeriodicalIF":0.2,"publicationDate":"2025-02-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143403558","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Spontaneous anastomosis of esophageal atresia without esophageal stricture formation: A case report","authors":"S. Tan Tanny ,&nbsp;S.E. Newman ,&nbsp;M. Safe ,&nbsp;W.J. Teague","doi":"10.1016/j.epsc.2025.102972","DOIUrl":"10.1016/j.epsc.2025.102972","url":null,"abstract":"<div><h3>Introduction</h3><div>Spontaneous esophago-esophageal fistulization is a reported phenomenon in cases of complex esophageal atresia, however, short and long-term complications are common, including stricture formation.</div></div><div><h3>Case presentation</h3><div>A male twin was born at 29 + 6 weeks gestation weighing 1103 g. Passage of a nasogastric tube was attempted but coiled in the upper esophagus, leading to a postnatal diagnosis of esophageal atresia with distal tracheo-esophageal fistula. At thoracotomy on day 1 of life, the tracheo-esophageal fistula was ligated without problems. Esophageal anastomosis to overcome a 1–1.5 vertebral body gap was attempted but abandoned following significant intraoperative anesthetic complications. Instead, the upper and lower esophageal ends were sutured closed and then apposed under tension using interrupted 4/0 Ethibond®. A contrast study on day 18 of life demonstrated spontaneous anastomosis of the esophageal ends, with reflux of contrast between the upper and lower esophagus, and no extraluminal contrast extravasation. Subsequent contrast studies at ages 4 weeks, 5, 10 and 13 months, and 2 years showed no anastomotic stricture. Upper gastrointestinal endoscopy at the age of 2 years showed no esophagitis. Gastro-esophageal reflux symptoms remain controlled with medication and no fundoplication has been performed. High resolution esophageal manometry at the age of 3 years demonstrated weak, but coordinated, distal peristalsis. At the age of 4.5 years, the patient is tolerating an unrestricted diet and has a growth curve that matches the curve of his twin sibling.</div></div><div><h3>Conclusion</h3><div>In cases where primary esophageal anastomosis is not possible, opposing the upper and lower pouches with sutures may result in spontaneous esophageal anastomosis not necessarily associated with an anastomotic stricture.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"115 ","pages":"Article 102972"},"PeriodicalIF":0.2,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143262502","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Management of total penile amputation following circumcision in a resource-limited setting: A case report","authors":"Suleiman Ayalew ,&nbsp;Michael A. Negussie ,&nbsp;Helina K. Teklehaimanot ,&nbsp;Nahom Getachew Mulatu ,&nbsp;Yishak Abdulsemed ,&nbsp;Mesfin Tesera Wassie","doi":"10.1016/j.epsc.2024.102939","DOIUrl":"10.1016/j.epsc.2024.102939","url":null,"abstract":"<div><h3>Introduction</h3><div>Total penile amputation following circumcision is a rare complication. This case highlights the challenges associated with managing such a catastrophic injury in a resource-limited setting.</div></div><div><h3>Case presentation</h3><div>A 46-day-old male was brought to our hospital with a five-day history of difficulty voiding, irritability, and bleeding from a circumcision done two weeks earlier by a traditional healer under suboptimal conditions. The mother reported a proper initial recovery but stated that the penis became discolored and detached five days prior to presentation. Despite the severe injury, the patient had no fever or systemic symptoms. On admission, he was afebrile and pale. The perineal exam revealed a total penile amputation, and a nasogastric tube placed in the urethra at the referring hospital. Blood tests showed anemia (hemoglobin 8.2 g/dL) but normal renal function. The urinalysis was normal. An abdominal ultrasound (US) ruled out hydronephrosis. The patient received a blood transfusion, intravenous ceftriaxone, and pain medications. He was taken to the operating room for an exam under anesthesia. We did a debridement of the necrotic tissue and reconstructed the urethral opening with skin flaps. We left an 8 French nasogastric tube in the urethra. The postoperative recovery was uneventful. At three months of follow up, he has healed properly and has a patent neourethral meatus.</div></div><div><h3>Conclusion</h3><div>Reconstruction of the urethral opening using locally mobilized skin flaps and urinary drainage via catheter placement can achieve satisfactory outcomes in infants with total penile amputation when advanced surgical resources are unavailable.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"113 ","pages":"Article 102939"},"PeriodicalIF":0.2,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143096287","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Uncommon presentation of perineal rhabdomyosarcoma: A case report","authors":"Danielle Vucenovic ,&nbsp;Alexandra P. Zorzi ,&nbsp;Glenn Bauman ,&nbsp;Amol Mujoomdar ,&nbsp;Daniel J. Indelicato ,&nbsp;Natashia M. Seemann","doi":"10.1016/j.epsc.2024.102940","DOIUrl":"10.1016/j.epsc.2024.102940","url":null,"abstract":"<div><h3>Introduction</h3><div>Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma among children in North America. Early diagnosis and treatment are important to prevent disease progression and improve prognosis.</div></div><div><h3>Case presentation</h3><div>A 6-year-old-boy presented with a 1cm perianal lesion that was tender and erythematous. Presumed diagnosis was perianal abscess: incision &amp; drainage was performed and complicated with significant bleeding requiring arterial embolization. Previous computed tomography (CT) had reported a large abscess cavity. Significant growth of tissue at surgical site and swelling of the right buttock and perineal area prompted further imaging with MRI which demonstrated suspicion for a pelvic soft tissue mass with extension to the perineum. Biopsies confirmed the mass was an embryonal rhabdomyosarcoma (RMS). He was diagnosed with intermediate risk Group III, Stage III translocation-negative rhabdomyosarcoma. He was treated with VAC/VI. Discussion at province-wide tumor boards recommended no further resection given the inability to achieve negative margins. The patient underwent proton beam therapy for local control. There was reduction in size of the pelvic mass, the perineal wound healed, and the defect completely resolved while on active therapy. The patient remains well now eight months into active follow up.</div></div><div><h3>Conclusion</h3><div>Perineal RMS is a rare disease and difficult to diagnosis as it often presents very similarly to perianal abscess. While it is not reasonable to screen all painful perianal masses with MRI, reflection of this case demonstrates some delineating features that can be useful to suggest an alternative diagnosis in order to minimize morbidity and shorten time to diagnosis.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"113 ","pages":"Article 102940"},"PeriodicalIF":0.2,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143096288","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hand-assisted laparoscopic surgery for Wilms tumor in a child: A case report","authors":"Giovanni Torino ,&nbsp;Ottavio Adorisio ,&nbsp;Agnese Roberti ,&nbsp;Tiziana Russo ,&nbsp;Francesco Turrà ,&nbsp;Giovanni Di Iorio","doi":"10.1016/j.epsc.2024.102937","DOIUrl":"10.1016/j.epsc.2024.102937","url":null,"abstract":"<div><h3>Introduction</h3><div>Hand-assisted laparoscopic surgery (HALS) is a technique that uses a device that allows the surgeon to insert a hand into the abdomen while maintaining the pneumoperitoneum. While there are a few reports of HALS in pediatric patients with benign diseases, there are no documented cases in pediatric oncology.</div></div><div><h3>Case presentation</h3><div>A 13-year-old girl presented with the onset of left-sided abdominal pain. An abdominal ultrasound (US) revealed a mass in the left kidney. A magnetic resonance (MRI) of the abdomen and a total-body computerized tomography (CT) scan confirmed the presence of a 10-cm left renal mass without evidence of metastatic lesions. Following the UMBRELLA SIOP-RTSG 2016 protocol, the patient underwent preoperative chemotherapy, after which a follow-up MRI showed a reduction in the mass size to 7 cm. We subsequently proceeded with a hand-assisted laparoscopic left nephrectomy. A GelPort® (Applied Medical, Rancho Santa Margarita, CA, USA) was inserted through a 6-cm Pfannenstiel incision. Two additional 5-mm and 10-mm trocars were added in the left upper quadrant and the umbilicus. During the operation, a neoplastic infiltration was seen on the diaphragm, which had not been seen on MRI. A portion of the diaphragm was resected, and the defect was repaired using an interrupted resorbable suture. Eight lymph nodes were sampled from the hilar, supra-hiliar, infra-hiliar, paraaortic, and inter-aortocaval areas. Pathological examination confirmed a blastemal-predominant Wilms tumor, negative lymph nodes, and a positive diaphragmatic infiltrate. The postoperative course was uneventful, and the patient was discharged on the third postoperative day. She received the postoperative chemotherapy regimen indicated by the protocol. At a follow up of 41 months she has no recurrence.</div></div><div><h3>Conclusion</h3><div>Hand-assisted laparoscopic surgery appears to be a safe and effective approach for the resection of solid renal tumors requiring total nephrectomy, potentially reducing the risk of tumor spillage and bleeding.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"113 ","pages":"Article 102937"},"PeriodicalIF":0.2,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143096289","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pitfalls in the rural care of gastroschisis: A case report","authors":"Maria Koenen ,&nbsp;Suzanne Reuter ,&nbsp;Amanda O'Neil ,&nbsp;Jon Ryckman","doi":"10.1016/j.epsc.2024.102934","DOIUrl":"10.1016/j.epsc.2024.102934","url":null,"abstract":"<div><h3>Introduction</h3><div>Gastroschisis is a common congenital abdominal wall defect that requires early, specialized care to optimize long-term outcomes. In rural areas, it is crucial that rural providers and transport teams are comfortable optimizing the gastroschisis patient in the immediate post-natal period prior to arriving at a hospital with pediatric surgical care.</div></div><div><h3>Case presentation</h3><div>A baby born at 32 weeks gestation in a remote Indian Health Services hospital was found to have gastroschisis at delivery. Transfer to the closest pediatric hospital, over 300 miles away, was initiated. While waiting for the NICU flight team to arrive, the rural hospital team cared for the infant. The bowel wrap caused the bedding to become cold and wet, and the baby was hypothermic when the flight team arrived. The local team started an IV, but was unable to achieve additional access and gave antibiotics instead of bolus or maintenance IV fluids. The infant arrived at the children's hospital over 6 hours after birth hypothermic, acidotic, and with severe bowel matting. At the receiving hospital, the gastroschisis was managed with a preformed silo and daily reductions until the baby was appropriate for surgical closure. Complete reduction of the bowel and surgical closure of the abdominal wall was achieved at day of life 15. A sutured repair of the fascia was performed. The baby recovered well from surgery and was started on feedings five days later. A standard feeding protocol is used for infants with gastroschisis, and this infant achieved full feedings 20days after surgery. No complications of gastroschisis occurred after repair.</div></div><div><h3>Conclusion</h3><div>The care of babies born with gastroschisis in remote or rural centers without a definitive neonatal care team can be challenging due to lack of expertise, lack of appropriate equipment, and communication difficulties with definitive care centers during the acute incident. Collaboration with rural centers can identify areas of weakness or need which may lead to the development of a simple guideline to care for these infants.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"113 ","pages":"Article 102934"},"PeriodicalIF":0.2,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143096291","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pilonidal omphalitis in a teenager: A case report","authors":"Humaid Alzaabi,&nbsp;Mohammad Ilyas Bader","doi":"10.1016/j.epsc.2024.102948","DOIUrl":"10.1016/j.epsc.2024.102948","url":null,"abstract":"<div><h3>Introduction</h3><div>Pilonidal omphalitis (PO) is a rare manifestation of retained hair in the umbilicus that can lead to inflammation and present with umbilical pain and discharge.</div></div><div><h3>Case presentation</h3><div>A previously healthy 15-year-old male presented with a one-year history of foul-smelling, serosanguinous umbilical discharge and associated pain. He reported no other symptoms or underlying issues. A course of topical antiseptic (povidone-iodine) was ineffective, and ultrasound imaging revealed no underlying pathology. Umbilical exploration identified an umbilical pilonidal sinus containing hair. The hair was removed, and the granulation tissue was cauterized using bipolar diathermy. The postoperative course was uneventful, and during routine follow-up visits, the patient reported significant symptom resolution with no further complaints.</div></div><div><h3>Conclusion</h3><div>Pilonidal omphalitis should be included in the differential diagnosis for young adolescents with significant hair growth who present with umbilical discharge. Prompt and thorough treatment is essential to prevent a prolonged disease course.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"113 ","pages":"Article 102948"},"PeriodicalIF":0.2,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143096292","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Unusual presentation of multiple ingested magnets in a 12-year-old boy: A case report","authors":"Ashley Ramirez ,&nbsp;Yi-Horng Lee ,&nbsp;Vasantha Kondamudi ,&nbsp;Francisca Velcek","doi":"10.1016/j.epsc.2024.102931","DOIUrl":"10.1016/j.epsc.2024.102931","url":null,"abstract":"<div><h3>Introduction</h3><div>Magnetic foreign body ingestions were previously quite rare, but as newer and stronger magnets become more commonly incorporated into daily lives, ingestions are becoming more frequent. This case involves an unlikely patient with a deceiving presentation which obscured the severity of his jejunal perforation.</div></div><div><h3>Case</h3><div>This is a 12-year-old boy with no past medical history who presented with non-peritonitic, mild abdominal pain. Vital signs and laboratory testing were all within normal limits. Patient did not disclose a history of foreign body ingestion when he first presented. His initial physical exam was significant for mild left sided abdominal tenderness without any peritoneal signs. He was evaluated with a CT scan of the abdomen and pelvis, and to everyone's surprise, there was a string of round metallic objects present, resembling magnetic beads. Only at that time did the patient admit to ingesting magnetic beads that were purchased for his younger sister as a toy. He never disclosed the timing of the ingestion or the circumstance in which he ingested them. The CT scan did not demonstrate any free air or inflammation around the bowel loops that contained these beads. Since his abdominal exam was relatively benign and the CT scan did not reveal any concerning findings, we elect to obtain an abdominal X-ray at that time as a baseline with the plan to follow serial abdominal X-rays to track the progression of these beads throughout the GI tract. A repeat abdominal X-ray 6 hours later did not show any progression of the ingested beads, and his abdominal pain became more intense during the time of our observation. This prompted operative intervention via exploratory laparotomy. A periumbilical midline incision was made and the bowel loops were inspected. Intraoperatively, we observed that the magnetic beads have perforated through the jejunal wall with serosal inflammation of the adjacent bowel loop. We did not see any fistula between any bowel loops. Repair was accomplished via resecting the small bowel segment containing the perforation and primary anastomosis. The inflamed serosal layer at the adjacent loop was plicated with a series of Lembert sutures. The patient recovered well without complications. He was discharged to home on the fifth postoperative day, and he remained well when he returned for follow up three months later.</div></div><div><h3>Conclusion</h3><div>A low threshold for surgical intervention should be maintained for children presenting with multiple magnet ingestion that is visualized on imaging. This is true especially in the setting of worsening pain and evidence of abdominal tenderness even when there is no free air seen on imaging studies.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"113 ","pages":"Article 102931"},"PeriodicalIF":0.2,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143096290","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cervical esophageal duplication cyst in a pediatric patient: A case report","authors":"Souha Laarif,&nbsp;Rabiaa Ben Abdallah,&nbsp;Cyrine Saadi,&nbsp;Fatma Trabelsi,&nbsp;Aida Daïb,&nbsp;Youssef Hellal","doi":"10.1016/j.epsc.2025.102963","DOIUrl":"10.1016/j.epsc.2025.102963","url":null,"abstract":"<div><h3>Introduction</h3><div>Esophageal duplications are rare congenital anomalies with an incidence of 1 case per 100,000 live births. They most commonly present in a cystic form, and involvement of the cervical esophagus is particularly rare.</div></div><div><h3>Case presentation</h3><div>A 2-year-old girl was referred to us due to a progressively enlarging right cervical mass and dysphagia. She had no past medical or surgical history. On physical exam we found a 4-cm nodule in the right cervical region. The nodule was soft, mobile, non-tender and not inflamed. A cervicothoracic magnetic resonance imaging (MRI) showed a 4× 4 × 5 cm unilocular paratracheal cystic mass with airway displacement and moderate tracheal stenosis. The patient was taken to the operating room for a cervical exploration through a right cervicotomy. We found a cystic mass surrounded by a thin smooth muscle layer firmly attached to the esophageal wall but not communicated with the esophageal lumen. The mass was detached off all the surrounding structures and resected completely. The histological examination confirmed the diagnosis of a cervical esophageal duplication cyst. Postoperative, a contrast study showed regular peristalsis, no extravasation and no stenosis. At three years of follow-up, she has no signs of recurrence.</div></div><div><h3>Conclusion</h3><div>Although rare, cervical esophageal duplications should be considered in the differential diagnosis of children who present with a palpable cervical mass. Complete surgical resection is the definitive treatment.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"114 ","pages":"Article 102963"},"PeriodicalIF":0.2,"publicationDate":"2025-01-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143148297","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Marfan syndrome coagulopathy in a patient undergoing bilateral video assisted thoracic surgery (VATS): A case report","authors":"Joanna Sajdlowska ,&nbsp;Agron Zuta ,&nbsp;John Paul Bustamante ,&nbsp;Nishith Bhattacharyya","doi":"10.1016/j.epsc.2025.102968","DOIUrl":"10.1016/j.epsc.2025.102968","url":null,"abstract":"<div><h3>Introduction</h3><div>Marfan syndrome is an inherited connective tissue disease that contributes to a wide range of health challenges, many of which can cause potential harm to several organs due to underlying coagulopathy.</div></div><div><h3>Case presentation</h3><div>A 20-year-old male with Marfan syndrome underwent an elective bilateral video-assisted thoracic surgery (VATS) for the management of recurrent pneumothorax after three prior episodes that required hospitalization. The procedure included mechanical and chemical pleurodesis, stapling of the apex of the right lung due to the presence of subpleural blebs, and placement of bilateral chest tubes (one per side). The patient was electively admitted to the pediatric intensive care unit (PICU) after the operation. The postoperative chest x-ray done for hours after the operation showed a large amount of fluid in the left pleural cavity, suspicious for a left hemothorax. A unit of fresh frozen plasma (FFP) was promptly administered, and the patient was taken back to the operating room for a VATS evacuation of the hemothorax and to upsize the chest tube. All clots were removed from the left chest, which was copiously irrigated with 8 L of fluid. No arterial bleeding was identified. A thromboelastography (TEG) test was performed and the resuscitation continued with two additional units of packed red blood cells intra-operatively plus postoperative 1:1:1 administration of FFP, platelets and cryoprecipitate. The patient remained hemodynamically stable overnight in the PICU. Four days later, he was medically cleared for a safe discharge.</div></div><div><h3>Conclusion</h3><div>Patients with Marfan syndrome have an intrinsic coagulopathy and need closer surveillance, as well as prompt intervention if needed, during and after surgical procedures.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"114 ","pages":"Article 102968"},"PeriodicalIF":0.2,"publicationDate":"2025-01-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143148246","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}