Journal of Pediatric Surgery Case Reports最新文献_第10页

Vaginal resection of rectovaginal teratoma: A case series 直肠阴道畸胎瘤阴道切除术：病例系列

IF 0.4 Q4 Medicine

Journal of Pediatric Surgery Case Reports

Pub Date : 2024-04-02 DOI: 10.1016/j.epsc.2024.102801

Kylie G. Fowler , Katherine O'Flynn O'Brien , Rebecca Josephson

Introduction

Extragonadal teratomas (EGTs) are histologically similar to gonadal teratomas but arise from structures outside of the ovaries, typically along midline structures such as the coccyx, mediastinum, retroperitoneum, and brain. Surgery is curative, but it can be technically challenging. The following cases represent the only published cases of rectovaginal/perirectal location of EGTs and the only cases of successful vaginal resection.

Cases

We present three cases of female patients ranging in age from 15 to 17 who presented with rectovaginal mature cystic teratomas. Two cases were found incidentally, one patient reported pelvic pain. The masses ranged in size from 3cm to 6cm. Pelvic MRI provided the best imaging technique for surgical planning. The larger masses (5.1 cms and 6 cms) were exerting significant pressure and mass effect on the rectum and vagina. The 3cm mass was located within 1cm of the ureter and therefore, with growth, could impede normal urologic function. Tumor markers were obtained for each patient prior to surgical resection. Initial surgical approach for the first case was combined vaginal-laparoscopic. However, after vaginal resection was the more successful access point to the first teratoma, subsequent cases were resected vaginally with technique described here.

Conclusion

Vaginal surgery offers several benefits including being minimally invasive, providing no visible incisions, utilizing less operating time, and providing rapid recovery. Vaginal resection of the rectovaginal EGTs at our institution proved safe, effective, and minimally invasive. Publication of this approach is important to guide surgical management of these tumors should other clinicians face this rare entity.

导言性腺外畸胎瘤（EGTs）在组织学上与性腺畸胎瘤相似，但来自卵巢以外的结构，通常沿中线结构，如尾骨、纵隔、腹膜后和大脑。手术可以治愈，但在技术上具有挑战性。以下病例是已发表的唯一一例直肠阴道/直肠位置的 EGT 病例，也是唯一一例成功的阴道切除病例。其中两例是偶然发现的，一例患者报告有盆腔疼痛。肿块大小从 3 厘米到 6 厘米不等。盆腔磁共振成像为手术规划提供了最佳成像技术。较大的肿块（5.1 厘米和 6 厘米）对直肠和阴道造成了明显的压力和肿块效应。3 厘米的肿块位于输尿管 1 厘米范围内，因此随着肿块的生长，可能会妨碍正常的泌尿系统功能。手术切除前，为每位患者采集了肿瘤标记物。第一个病例的最初手术方法是阴道镜和腹腔镜联合手术。然而，在阴道切除是第一个畸胎瘤的较成功切入点后，随后的病例均采用本文所述的技术进行阴道切除。结论阴道手术具有多种优势，包括微创、无明显切口、手术时间短、恢复快。在我院进行的直肠阴道 EGT 阴道切除术证明是安全、有效和微创的。如果其他临床医生遇到这种罕见的肿瘤，发表这种方法对指导手术治疗非常重要。

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引用次数: 0

Erratum to “Bilateral phyllodes tumor: Case report” [EPSC 101C (2023) 102753] 对 "双侧蝶骨瘤 "的勘误：病例报告' [EPSC 101C (2023) 102753]>

IF 0.4 Q4 Medicine

Journal of Pediatric Surgery Case Reports

Pub Date : 2024-03-27 DOI: 10.1016/j.epsc.2024.102800

Isabel Mayorga-Pérez , Patricia Mulero-Soto , Kerwin Cruz-De La Rosa , Viviana Negrón-González , Jonathan Hernandez-Rosa , Jose Feneque-Gonzalez , Maria Correa-Rivas , Anwar Abdul-Hadi

引用次数: 0

Pancreatico-pleural fistula mimicking a recurrent congenital diaphragmatic hernia: A case report 模仿复发性先天性膈疝的胰腺胸膜瘘：病例报告

IF 0.4 Q4 Medicine

Journal of Pediatric Surgery Case Reports

Pub Date : 2024-03-26 DOI: 10.1016/j.epsc.2024.102799

Maximilian Holweg , Justus Lieber , Alfred Königsrainer , Steffen Hartleif , Michael Esser , Jörg Fuchs

Introduction

Pancreaticopleural fistula (PPF) is a very rare complication of pancreatitis. The symptoms are non-specific and the diagnosis is often missed.

Case presentation

A 14-year-old adolescent was admitted to the department of pediatric surgery with several readmissions due to recurrent left pleural effusion and chest pain. The patient also had a history of a left-sided congenital diaphragmatic hernia (CDH), which was surgically corrected via laparotomy at the age of two. The pleural effusion was explained in the context of pneumonia, but elevated serum levels of amylase and lipase prompted imaging. Computer tomography (CT) and standard magnetic resonance imaging (MRI) showed a small gap in the diaphragm and the pancreatic tail nearby leading to the diagnosis of recurrent CDH. Explorative thoracoscopy was performed, but showed the diaphragm intact. Pleurodesis was realized to treat the effusion. Two months later, recurrence of the pleural effusion in the left hemithorax and pancreatitis occurred. Magnetic resonance cholangiopancreatography (MRCP) showed a small subphrenic cyst close to the pancreatic tail and multiple supradiaphragmal cysts for the first time. Persistently, the small gap in the diaphragm close to the pancreatic tail was detected and the now suspected diagnosis was PPF. Subsequently, laparoscopic resection of the pancreatic tail was performed including the closure of the diaphragmatic gap. The course was uneventful, the pleural effusion disappeared, and the levels of serum pancreatic enzymes normalized during follow-up.

Conclusion

PPF is rare and various entities may masquerade the diagnosis. A massive pleural effusion in combination with pancreatitis may indicate this serious condition.

导言胰腺胸膜瘘（PPF）是一种非常罕见的胰腺炎并发症。病例介绍一名 14 岁的青少年因反复出现左侧胸腔积液和胸痛而多次再次入院，被收住在小儿外科。患者还有左侧先天性膈疝（CDH）病史，两岁时通过开腹手术进行了矫正。胸腔积液被解释为肺炎，但血清中淀粉酶和脂肪酶水平的升高促使患者进行造影检查。计算机断层扫描（CT）和标准磁共振成像（MRI）显示，膈肌和胰腺尾部附近有一个小缺口，诊断为复发性 CDH。进行了探查性胸腔镜检查，但显示膈肌完好无损。胸腔穿刺术治疗了积液。两个月后，左半身胸腔积液和胰腺炎复发。磁共振胰胆管造影（MRCP）显示，胰腺尾部附近有一个膈下小囊肿，并首次发现多个膈上囊肿。随后又发现靠近胰腺尾部的膈肌上有一个小间隙，目前怀疑诊断为 PPF。随后，患者接受了腹腔镜胰尾切除术，包括关闭膈肌间隙。病程顺利，胸腔积液消失，随访期间血清胰酶水平恢复正常。大量胸腔积液合并胰腺炎可能预示着这一严重疾病。

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引用次数: 0

Hepatic epidermoid cyst in a pediatric patient: A case report 一名儿童患者的肝表皮样囊肿：病例报告

IF 0.4 Q4 Medicine

Journal of Pediatric Surgery Case Reports

Pub Date : 2024-03-14 DOI: 10.1016/j.epsc.2024.102796

Cesar E. Larancuent , Mandolin Ziadie , Noor Kassira

Introduction

Hepatic epidermoid cysts are uncommon benign liver lesions that infrequently manifest in pediatric populations. Out of the limited reported cases, only 11 are pediatric. Despite their rarity, their potential for various clinical presentations necessitates careful examination and understanding.

Case presentation

A 10-year-old female with a history of constipation presented with persistent abdominal pain. Initial suspicions, influenced by her travel history to Dubai and interactions with camels and dolphins, led to considerations of infectious etiologies like echinococcosis. Imaging revealed a complex cystic mass in the liver's right lobe, measuring 4.5 x 5.2 × 6.0 cm. Despite a range of diagnostic efforts, including imaging with ultrasound and MRI as well as infectious disease and gastroenterology consults, the cyst's nature remained ambiguous. After interventional radiology (IR) drainage, symptoms continued and the cyst remained visible on imaging, but cytology of the fluid revealed it to be a benign epidermoid cyst. Surgical intervention, specifically a laparoscopic deroofing, was performed, resulting in symptom relief and prevention of potential future complications. Six months post-surgery, no further dominant cyst was present.

Conclusion

This case accentuates the diagnostic complexities surrounding hepatic epidermoid cysts in pediatric patients. A multidisciplinary approach, along with surgical intervention, proved instrumental in diagnosing and managing this rare condition. It reinforces the importance of careful examination and collaboration in managing atypical pediatric liver conditions.

导言肝表皮样囊肿是一种不常见的肝脏良性病变，很少发生在儿童身上。在有限的报道病例中，仅有 11 例为儿科病例。尽管肝表皮样囊肿非常罕见，但其潜在的各种临床表现却需要仔细检查和了解。由于她曾到迪拜旅行，并与骆驼和海豚有过接触，最初怀疑是感染性病因，如棘球蚴病。影像学检查发现肝脏右叶有一个复杂的囊性肿块，大小为 4.5 x 5.2 x 6.0 厘米。尽管进行了一系列诊断，包括超声波和核磁共振成像以及传染病和消化内科会诊，但囊肿的性质仍然不明确。经介入放射学（IR）引流后，症状仍在持续，囊肿在影像学上仍清晰可见，但液体的细胞学检查显示这是一个良性表皮样囊肿。经过手术治疗，特别是腹腔镜下切除术，症状得到缓解，并避免了今后可能出现的并发症。结论：该病例凸显了儿科肝表皮样囊肿诊断的复杂性。事实证明，多学科方法和手术干预有助于诊断和处理这一罕见病症。该病例强调了仔细检查和合作治疗非典型儿科肝病的重要性。

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引用次数: 0

Psoas abscess presenting as a bowel obstruction in a child: A case report 以肠梗阻为表现的儿童腰大肌脓肿：病例报告

IF 0.4 Q4 Medicine

Journal of Pediatric Surgery Case Reports

Pub Date : 2024-03-14 DOI: 10.1016/j.epsc.2024.102797

Thuy-Ngan Nguyen-Thi , Khoa Nguyen-Dang , Ngoc Duong-Minh , Hanh-Duyen Bui-Thi , Minh-Hong Pham-Thi

Introduction

Psoas abscess (PA) is an uncommon condition in children where pus accumulates in the psoas muscle. The disease symptoms are subtle and nonspecific, often causing delayed diagnosis.

Case presentation

Our case describes an unusual manifestation of PA caused by methicillin-resistant Staphylococcus aureus (MRSA) when an 11-year-old male patient was admitted with suspected bloodstream infection from the gastrointestinal tract and bowel obstruction following closed trauma from a bicycle fall. An emergency laparotomy revealed turbid abdominal fluid with pseudomembranes and no apparent cause of peritonitis was found upon observing the entire abdominal cavity. On the fourth day after admission, blood, sputum, and peritoneal fluid cultures were all positive for MRSA. Despite broad-spectrum antibiotic treatment targeting MRSA, the patient's clinical condition did not improve. PA was confirmed via abdominal computed tomography on the ninth-day post-hospitalization with a persistent MRSA bloodstream infection. Successful treatment of PA was achieved through open surgery. The patient's condition improved and was discharged on the 40th day after admission.

Conclusion

PA can present as bowel obstruction. Therefore, PA should be ruled out in patients with bowel obstruction of unknown etiology, especially if the patient has a history of trauma to the hip region.

导言腰肌脓肿（PA）是一种不常见的儿童疾病，脓液积聚在腰肌内。我们的病例描述了由耐甲氧西林金黄色葡萄球菌（MRSA）引起的腰大肌脓肿的不寻常表现。当时，一名 11 岁的男性患者因自行车摔倒造成的闭合性外伤而入院，疑似胃肠道血流感染和肠梗阻。急诊剖腹探查术发现腹腔积液混浊，并伴有假膜，观察整个腹腔未发现明显的腹膜炎病因。入院后第四天，血液、痰液和腹腔液培养均呈 MRSA 阳性。尽管针对 MRSA 进行了广谱抗生素治疗，但患者的临床症状并未改善。入院后第 9 天，腹部计算机断层扫描证实 PA 存在持续的 MRSA 血流感染。通过开腹手术成功治疗了 PA。结论 PA 可表现为肠梗阻。因此，对于病因不明的肠梗阻患者，尤其是髋部有外伤史的患者，应排除 PA 的可能。

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引用次数: 0

Torsed and amputated ovary in the canal of Nuck in a 3-year-old girl: A case report 一名 3 岁女孩的卵巢在纳克管内扭转并被切除：病例报告

IF 0.4 Q4 Medicine

Journal of Pediatric Surgery Case Reports

Pub Date : 2024-03-14 DOI: 10.1016/j.epsc.2024.102798

Yohei Sanmoto

Introduction

Amputated ovaries caused by prenatal torsion often remain asymptomatic, but their natural history is not fully understood.

Case presentation

A 3-year-old girl presented with swelling in the right inguinal region. She had been diagnosed with a cyst on her left ovary during the fetal period. Postnatal ultrasound examination revealed necrosis and solid-cystic transformation of the left ovary. The necrotic ovary decreased in size over time without intervention. At the time of consultation, a nonreducible, firm, and elastic mass was found in the right inguinal region. Initially, a hydrocele of the canal of Nuck was suspected, but an ultrasound examination showed that the mass had heterogeneous solid components. Given the distinctive clinical progression, an amputated ovary was suspected, and surgical exploration was carried out. During the operation, an initial laparoscopic examination revealed the absence of the left ovary, as well as bilateral inguinal hernias. Since the mass could not be manually reduced back into the abdominal cavity, a small incision was made on the right inguinal region to directly visualize it and remove it. The inguinal hernias were repaired laparoscopically. The mass was formed predominantly of necrotic tissue partially calcified, and there was no viable ovarian tissue. Clinical and pathological findings strongly suggested that the torsed necrotic left ovary had migrated into the canal of Nuck and presented as an inguinal mass.

Conclusion

A torsed necrotic ovary may invade the canal of Nuck and potentially present as an inguinal mass.

导言：产前扭转导致的卵巢肿大通常没有症状，但其自然病史尚不完全清楚。病例介绍一名 3 岁女孩因右侧腹股沟区肿胀而就诊。她在胎儿期被诊断为左侧卵巢囊肿。产后超声波检查发现左侧卵巢坏死并发生实性囊变。随着时间的推移，坏死的卵巢逐渐缩小，但未进行干预。就诊时，发现右侧腹股沟区有一个不可复发、坚实而有弹性的肿块。起初怀疑是纳克管鞘膜积液，但超声检查显示肿块有异质固体成分。鉴于其独特的临床表现，医生怀疑是卵巢被切除，于是进行了手术探查。手术过程中，腹腔镜初步检查发现左侧卵巢缺失，并伴有双侧腹股沟疝。由于无法手动将肿块缩回腹腔，因此在右侧腹股沟区做了一个小切口，以便直接观察和切除肿块。腹股沟疝通过腹腔镜进行了修补。肿块主要由部分钙化的坏死组织形成，没有存活的卵巢组织。临床和病理检查结果强烈提示，扭转坏死的左侧卵巢已移入Nuck管，并表现为腹股沟肿块。

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引用次数: 0

Pilonidal disease in siblings - A case series 兄弟姐妹中的蝶鞍疾病--病例系列

IF 0.4 Q4 Medicine

Journal of Pediatric Surgery Case Reports

Pub Date : 2024-03-14 DOI: 10.1016/j.epsc.2024.102795

Norah E. Liang , Claire Abrajano , Bill Chiu

Introduction

Pilonidal disease can occur in siblings, and family history of pilonidal disease can predispose to earlier onset of disease. However, there is scant literature regarding the occurrence or presentation of pilonidal disease in siblings. We present the largest case series to date of six sibling pairs with pilonidal disease, including one pair of identical twins.

Case presentation

Six sibling pairs were treated for pilonidal disease in our clinic. Of the six sibling pairs, five were same sex siblings and one was opposite sex siblings. The age at presentation ranged from 13.4 to 23.0 years old, with a median age of 15.7 years [interquartile range (IQR): 14.3–20.2]. The median age difference between siblings was 618 days (IQR: 407–1071), with a median length of follow up of 388 days (IQR: 185.5–449.0). The median symptom duration at the time of presentation was 249.5 days (IQR: 131.2–697.5). Four of the 12 patients presented with draining granuloma or secondary sinus, including both of the siblings in the identical twin pair. Eight of the 12 patients were treated with operative management initially, while four were treated non-operatively. None of the patients who underwent initial operative management experienced recurrence. Patients managed non-operatively were treated with regular and laser epilation. All patients who underwent operative management received a Gips procedure followed by regular and laser epilation.

Conclusion

Pilonidal disease can occur in siblings, suggesting that it may be associated with genetic predisposition. Additionally, siblings can present with differing severities of pilonidal disease.

导言：朝天鼻病可能会发生在兄弟姐妹身上，有朝天鼻病家族史的人可能会更早发病。然而，有关兄弟姐妹中发生或表现皮炎的文献却很少。我们展示了迄今为止最大的病例系列，其中有六对兄弟姐妹患有皮炎，包括一对同卵双胞胎。在这六对兄妹中，五对是同性兄妹，一对是异性兄妹。发病年龄从 13.4 岁到 23.0 岁不等，中位年龄为 15.7 岁[四分位距（IQR）：14.3-20.2]。兄弟姐妹之间的年龄差异中位数为 618 天（IQR：407-1071），随访时间中位数为 388 天（IQR：185.5-449.0）。发病时症状持续时间的中位数为 249.5 天（IQR：131.2-697.5）。12 名患者中有 4 人伴有引流性肉芽肿或继发性窦，其中包括一对同卵双胞胎中的两个兄弟姐妹。12 名患者中，8 人最初接受了手术治疗，4 人接受了非手术治疗。最初接受手术治疗的患者无一复发。非手术治疗的患者接受了常规和激光脱毛治疗。所有接受手术治疗的患者都接受了 Gips 手术，然后进行常规脱毛和激光脱毛。此外，同胞兄弟姊妹可能会出现不同严重程度的朝天鼻病。

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引用次数: 0

Splenic infarction following recurrent torsion of a wandering spleen: A case report 游走脾反复扭转后的脾梗死：病例报告

IF 0.4 Q4 Medicine

Journal of Pediatric Surgery Case Reports

Pub Date : 2024-03-11 DOI: 10.1016/j.epsc.2024.102793

Mohammad Tareq Rahimi , Abdul Jamil Rasooli , Soghra Khaliqi , Nawaz Sharif Kashaf , Palwasha Wardak Mohtasebzada , Roohullah Hares

Background

Wandering spleen (WS) is a rare condition characterized by hypermobility of the spleen due to maldevelopment or absence of the ligaments that hold it in its normal location. Splenic infarction following torsion of a wandering spleen is a rare cause of acute abdomen in children and can be difficult to diagnose, especially at secondary healthcare facilities.

Case presentation

A 10-year-old girl presented to our clinic with a two-week history of abdominal pain, fever and vomiting. On physical examination she was pale, cachectic and dysmorphic. Painful splenomegaly was noted on palpation. Inflammatory markers were elevated (CRP: 26.7 mg/L; WBC: 20.4 x 109/L). A contrast-enhanced CT of the abdomen revealed splenomegaly with complete splenic infarction due to torsion of the splenic pedicle, consistent with a wandering spleen. The patient underwent an emergent laparotomy through a left subcostal incision. A markedly enlarged spleen was found, with its pedicle completely torsed. The spleen had no attachments to the abdominal wall or diaphragm and appeared non vital. We proceeded with a complete splenectomy. The patient recovered well and was discharged in stable condition on the fifth postoperative day. At 6 months of follow up, she has had no recurrent episodes of abdominal pain.

Conclusion

While torsion of a WS is a rare condition, it must be included in the differential diagnosis of children with abdominal pain of unknown etiology.

背景游走性脾脏（WS）是一种罕见的疾病，其特点是由于固定脾脏在正常位置的韧带发育不良或缺失而导致脾脏活动过度。游走性脾扭转引起的脾梗死是儿童急腹症的罕见病因，而且很难诊断，尤其是在二级医疗机构。体格检查时，她面色苍白、消瘦、畸形。触诊时发现脾脏肿大。炎症指标升高（CRP：26.7 mg/L；WBC：20.4 x 109/L）。腹部对比增强 CT 显示脾脏肿大，脾蒂扭转导致脾脏完全梗死，与游走性脾脏一致。患者经左肋下切口接受了急诊开腹手术。发现脾脏明显肿大，脾蒂完全扭转。脾脏与腹壁或膈肌无粘连，看起来没有生命迹象。我们对其进行了脾脏全切除术。患者恢复良好，术后第五天病情稳定出院。结论虽然 WS 扭转是一种罕见病，但必须将其纳入病因不明的腹痛患儿的鉴别诊断中。

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引用次数: 0

Laparoscopic-assisted omentectomy for omental torsion: A case report 腹腔镜辅助网膜切除术治疗网膜扭转：病例报告

IF 0.4 Q4 Medicine

Journal of Pediatric Surgery Case Reports

Pub Date : 2024-03-03 DOI: 10.1016/j.epsc.2024.102792

Takeshi Matsumoto, Yukiko Tani, Naruhiko Murase

Introduction

Omental torsion is an uncommon cause of acute abdominal pain in children and conservative treatment has been successful in some cases. We report a case in which the patient's symptoms did not improve despite conservative treatment and required a surgical intervention.

Case presentation

A 5-year-old girl was referred to our hospital because of right-sided abdominal pain and fever for 2 days. The vital signs on admission were within normal limits except for low-grade fever (37.6 °C). Laboratory examinations revealed an inflammatory reaction. White blood cell count was 10,500 cells/μl with 67.1 % neutrophils, and serum C-reactive protein concentration was 6.01 mg/dl. Ultrasonography revealed a hypoechoic mass in the right lower abdomen, and contrast-enhanced computed tomography showed a fatty mass with a whirl sign located between the anterior abdominal wall and the ascending colon. Conservative therapy with fasting, analgesics and antibiotics was initially chosen. However, because the symptoms were not relieved after 24 h, we decided to proceed with a surgical management. A single-incision laparoscopic surgery was done the following day. The operation included an adhesiolysis and omentectomy. The patient was discharged home after 5 days after re-gaining proper oral intake, without postoperative complications.

Conclusion

Omental torsion is a rare acute abdominal condition in children that may respond to conservative treatment. If conservative treatment is ineffective for more than 24–48 hours, surgical intervention is recommended.

导言网膜扭转是导致儿童急性腹痛的一个不常见原因，保守治疗在某些病例中取得了成功。我们报告了一个病例，患者在接受保守治疗后症状仍未改善，需要进行手术治疗。病例介绍一名 5 岁女童因右侧腹痛和发热 2 天转诊至我院。入院时，除低烧（37.6 °C）外，其他生命体征均在正常范围内。实验室检查显示有炎症反应。白细胞计数为 10,500 cells/μl，中性粒细胞占 67.1%，血清 C 反应蛋白浓度为 6.01 mg/dl。超声波检查显示右下腹部有一个低回声肿块，对比增强计算机断层扫描显示位于前腹壁和升结肠之间的脂肪肿块呈漩涡状。最初选择了禁食、止痛和抗生素的保守治疗。然而，由于 24 小时后症状仍未缓解，我们决定进行手术治疗。第二天，我们进行了单切口腹腔镜手术。手术包括粘连溶解和卵巢切除术。结论网膜扭转是一种罕见的儿童急腹症，保守治疗可能无效。如果保守治疗超过 24-48 小时无效，建议进行手术治疗。

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引用次数: 0

Split notochord syndrome with neuroenteric fistula: A case report 伴有神经肠瘘的脊索分裂综合征：病例报告

IF 0.4 Q4 Medicine

Journal of Pediatric Surgery Case Reports

Pub Date : 2024-03-02 DOI: 10.1016/j.epsc.2024.102791

Michael A. Negussie , Leul M. Manyazewal , Fitsum A. Gemechu , Samuel Mesfin , Abebe Mekonnen , Tihitena Negussie

Introduction

Split notochord syndrome (SNS) is a rare congenital anomaly characterized by malformations in the spine, central nervous system and gastrointestinal tract. This paper presents a case of SNS accompanied by a neuroenteric fistula (NEF).

Case presentation

A 7-day-old male neonate was admitted to our NICU for the evaluation and management of a lower back swelling with meconium extrusion, following an emergency cesarean section at 38 + 3 weeks gestational age due to meconium-stained amniotic fluid. The mother had a normal antenatal care history without reported fetal abnormalities. The patient had a noticeable lower back mass with active meconium oozing, accompanied by flaccid paralysis of the lower extremities. Blood work revealed elevated inflammatory markers and normal renal function. Abdominal CT scan and MRI identified a complex spinal malformation indicative of SNS, characterized by a thoracolumbar cystic mass, spinal bifida, non-fusion of lumbar vertebral bodies, a neuroenteric fistula, and Chiari II malformation, in addition to a right inguinal hernia and hydronephrosis. The patient underwent a laparotomy during which intestinal malrotation and a colonic duplication were also found. We did a NEF separation, a double barrel sigmoid colostomy, and a Ladd's procedure. By the 23rd day of life, the patient developed fever, tachypnea, tachycardia, and had purulent discharge from the surgical site. Despite the antibiotic treatment, the patient's condition deteriorated. He died on day-of-life 28 from overwhelming sepsis.

Conclusion

Our case highlights the complexity of patients with SNS and the need for enhanced surveillance and prompt responses in patients with this rare congenital malformation.

导言分裂脊索综合征（SNS）是一种罕见的先天性畸形，以脊柱、中枢神经系统和胃肠道畸形为特征。病例介绍一名 7 天大的男性新生儿因胎粪染羊水而在孕 38+3 周时进行了紧急剖宫产手术，之后因腰部肿胀并伴有胎粪挤出而被送入我院新生儿重症监护室进行评估和治疗。产妇的产前检查记录正常，未报告胎儿异常。患者腰部有明显肿块，伴有活动性羊水渗出，下肢弛缓性瘫痪。血液检查显示炎症指标升高，肾功能正常。腹部CT扫描和核磁共振成像检查发现，除了右侧腹股沟疝和肾积水外，患者还伴有胸腰椎囊性肿块、脊柱裂、腰椎体不融合、神经肠管瘘、Chiari II畸形等复杂的脊柱畸形，显示为SNS。患者接受了开腹手术，手术中还发现了肠旋转不良和结肠重复。我们为他做了 NEF 分离术、双管乙状结肠造口术和 Ladd's 手术。出生后第 23 天，患者出现发热、呼吸急促、心动过速，手术部位有脓性分泌物。尽管接受了抗生素治疗，患者的病情还是恶化了。结论：我们的病例凸显了 SNS 患者的复杂性，以及对这种罕见先天性畸形患者加强监测和及时应对的必要性。

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