Journal of Pediatric Surgery Case Reports最新文献

Introduction

A congenital hepatoblastoma (CH) is a rare liver tumor that can lead to severe outcomes due to unexpected rapid tumor growth. We report a case of CH with respiratory and circulatory failure caused by tumor growth, in which the patient underwent an emergency tumor resection and survived.

Case presentation

A male fetus was diagnosed by fetal ultrasound with a giant abdominal mass at 34 weeks and 5 days of gestation. On the same day he developed fetal distress was delivered by emergency Caesarean section. His birthweight was 3060 g, and his Apgar scores were 4 at 1 minute and 5 at 5 minutes. Immediately after birth he developed respiratory and circulatory failure due to compression of the thoracic organs by the giant abdominal mass. He was intubated shortly after birth and cared for in the intensive care unit. Alpha-fetoprotein (AFP) was 484,000 ng/mL (Normal range: 15,700 to 146,500 ng/mL). A contrast-enhanced computerized tomography (CT) revealed a tumor with a maximum diameter of 150 mm in the right lobe of the liver, suspicious for a congenital hepatoblastoma (CH). His clinical status deteriorated abruptly so he underwent an emergency right liver lobectomy. During the procedure, he developed cardiopulmonary arrest and the tumor was grossly resected while on chest compressions. He was resuscitated and the surgical bed was left with packed gauze. He gradually improved and the packing was removed on postoperative day 10. Pathology confirmed CH. He gradually recovered and was discharged home at 4 months of age. At 8 months of age a recurrence was diagnosed based on rising levels of AFP. He underwent a resection of the recurrence followed by adjuvant chemotherapy. He is free of further recurrence at 2 years of age.

Conclusion

Emergency resection of large CH is a high-risk operation but can potentially lead to survival, even if the margins are not tumor-free. Close follow up is mandatory.

Introduction

Congenital lobar emphysema (CLE) is a condition characterized by overinflation of one or more pulmonary lobes due to air-trapping, which can cause severe compression of adjacent structures. Bilateral CLE is rare but can cause severe respiratory distress.

Case presentation

A 9-day-old female born at 38 weeks who had respiratory distress since birth was transferred to our institution for further care. The prenatal history was unremarkable. Bloodwork was within normal limits.

She was put on non-invasive continuous positive airway pressure. On physical exam she had decreased air entry over the lower two-thirds of the lung fields bilaterally. A chest x-ray revealed air trapping in the left upper hemithorax and the right lower hemithorax. Contrast-enhanced computed tomography confirmed bilateral CLE of the left upper lobe and the right middle lobe causing severe compression of the adjacent lobes. She was intubated and transferred to the intensive care unit. Due to concerns regarding adherence to follow up and further access to medical care, we decided to attempt a single-stage bilateral thoracotomy/lobectomy. On day of life 15 she was taken to the operating room. We first did a left thoracotomy and a left upper lobectomy. The operation was uneventful, and she remained hemodynamically stable throughout the case. We closed the left thoracotomy and decided to proceed with a right thoracotomy and right middle lobectomy. She tolerated that procedure well. She was extubated in the operating room and was transferred to the intensive care unit. She recovered well and was discharged from the hospital on no respiratory support on postoperative day 13. Six months after the operation she remains asymptomatic and is thriving well.

Conclusion

In a resource-limited setting with poor parental adherence for follow-up, single-stage bilateral thoracotomy is a viable option for the management of bilateral congenital lobar emphysema.

Introduction

Pilonidal disease is a common condition in adolescents and young adults that is managed with medications, local wound care and surgery. Immunosuppression is not a standard therapy utilized in this disease but is routinely prescribed in hidradenitis, a folliculitis in the axillae and groin rather than in the gluteal cleft.

Case presentation

We present the case of a patient with severe pilonidal disease, with multiple collections in and above the gluteal cleft. Prior to presentation to our clinic, they had failed a standard treatment regimen for three years which included local wound care, multiple courses of antibiotics and incision and drainage procedures. They started on a treatment plan focused on hygiene and hair removal after being evaluated in our clinic. During this time, a concomitant diagnosis of leukemia was made and they were treated with induction chemotherapy and subsequent bone marrow transplant. Their pilonidal disease fully resolved after immunosuppression.

Conclusion

This patient's severe pilonidal disease resolved with immunosuppression, supporting an inflammatory basis of the condition.

Introduction

Adrenocortical carcinoma (ACC) is rare, aggressive, and metastasizes mainly to the lungs, liver, and regional lymph nodes. This is the first reported case of a pediatric patient with adrenohepatic fusion (AHF) and functional ACC that solely invaded the liver.

Case presentation

The patient was a 2 year and 9 month old female, with a 3-month history of weight gain, signs of virilization, Cushing's syndrome, hypertension, and a palpable mass in the right hypochondrium and epigastrium. Blood tests revealed the following: hemoglobin, 16.3 g/dL; cortisol, 25.3 μg/dL; and total serum testosterone, 1489.01 ng/mL. Ultrasonography and computed tomography initially revealed a hepatic tumor measuring 100 × 111 × 106 mm, with a volume of 615 mL. The patient was diagnosed with functional ACC. Curative resection (R0 resection) was performed, involving partial resection of the right hepatic lobe. The resected tumor was 12 × 10 × 7 cm and 650 g, with evidence of invasion into the liver capsule, which was consistent with ACC stage III (T₄N₀M₀). Management included mitotane, cisplatin, etoposide, and doxorubicin, and favorable progress was noted at the 2-year and 3-month follow-up.

Conclusion

In cases with AHF, a sufficient portion of the right hepatic lobe must be resected to ensure complete removal of the tumor.

Introduction

Wandering spleen is a rare anatomic finding, either congenital or acquired, that results in hypermobility of the spleen. This paper describes a wandering spleen in a patient with a complex surgical history including vanishing gastroschisis.

Case presentation

A 2-year-old female with past medical history of gestational age of 34 weeks, intestinal failure secondary to vanishing gastroschisis, serial transverse enteroplasty (STEP) procedure, and a supplemental nutrition requirement presented with a one-day history of non-bloody, non-bilious emesis, decreased urine output, and decreased oral intake. The vital signs, physical examination and laboratory investigations were normal. Small bowel follow through fluoroscopy study showed no contrast exiting the stomach consistent with gastric outlet obstruction. Patient was taken for exploratory laparotomy and found to have a gastric volvulus secondary to a wandering spleen. At operation, the gastric volvulus was reduced and a splenopexy performed incorporating the use of a mesh bag which was tacked to the abdominal wall in the left upper quadrant. The patient was discharged on POD 4 on their home TPN regimen and enteral feeds were reinstated on POD 14.

Conclusion

Wandering spleen as an etiology of gastric volvulus may be seen in patients with a history of gastroschisis. Intraoperative decision making should maintain the goal of splenic salvage and preservation of intestinal length.

Introduction

We report a rare occurrence in which a term infant with intestinal anomalous congenital bands (ACB), presented with bloody stools and necrotizing enterocolitis (NEC), instead of the typical intestinal obstruction picture. Our case differs from the reported cases of ACB in its presentation as bloody stools and necrotizing enterocolitis, rather than the classic symptoms of intestinal obstruction (IO).

Case presentation

A term neonate who was admitted to the Neonatal Intensive Care Unit (NICU) because of hypoglycemia, developed bloody stools and a clinical picture of NEC. She was treated by antibiotics and kept NPO for 7 days during which her symptoms resolved. Upon refeeding and after reaching full feeding volumes, her bloody stools recurred which warranted expanding the differential diagnosis and further investigations including lower gastrointestinal (GI) contrast study. The lower GI contrast study showed multiple filling defects indicating GI obstruction. Laparotomy was performed to explore and release the cause of obstruction. Laparotomy revealed Anomalous Congenital Bands (ACB) in the colon and cecum causing almost complete obstruction of the intestinal lumen. The ACB were excised, and the stricture segments of bowel were resected, and primary anastomosis was completed. Feeding was resumed with no recurrence of symptoms.

Conclusion

Anomalous congenital bands may present with bloody stools and clinical picture of NEC rather that the classic presentation of intestinal obstruction.

Introduction

Anastomosing hemangiomas represent a rare variant of benign vascular hemangioma which rarely arise from the liver parenchyma. The peculiarity of these tumors is that they can mimic malignant tumors, such as hepatocellular carcinoma, hepatoblastoma, and angiosarcoma. Notably, all documented cases where this type of liver tumor was described in the world's literature exclude cases involving infant patients.

Case presentation

Our clinical case demonstrates the presence of an anastomosing hemangioma in the right lobe of the liver in a 5-month-old child, necessitating radical surgical intervention. According to ultrasound and CT scan findings, a tumor measuring 7.5 by 6 by 4.5 cm was detected in the segments V, VII and VIII of the liver. A differential diagnosis between hepatoblastoma and angiosarcoma was conducted. Blood tests, including alpha-fetoprotein levels, were within normal limits. Percutaneous biopsy revealed no cellular atypia, while immunohistochemical examination identified anastomosing hemangioma positive for CD-31 and CD-34 staining. Given the size of the tumor, an anatomical right hepatectomy was performed. The patient was discharged on the 7th postoperative day without surgical complications. After 6 months, no recurrence of the tumor was detected.

Conclusion

This particular case highlights the importance of differential diagnosis of this type of tumor in children since anastomosing hemangiomas can mimic malignant liver tumors.

Introduction

Gastrostomy tube hypertrophic granulation tissue development, prolapse, and leakage are common problems that are often treated with tube re-siting. Wedge gastrostomy tube site resection is a revision technique used to enable continued use of the original tube site while removing affected tissue. The aim of this study was to describe this technique and outcomes in a series of patients requiring this intervention. The wedge gastrostomy tube site resection involves wedge excision of hypergranulation tissue or prolapsed gastric tissue and primary closure of the remaining defect. We reviewed outcomes for two children who underwent wedge gastrostomy tube site resection for medically refractory hypergranulation tissue.

Case presentation

Two patients presented with hypertrophic granulation tissue at their gastrostomy tube sites. In Case 1, the patient underwent two revisions spaced two years apart and has been symptom-free for 9 months. In Case 2, the patient required a single revision and has been symptom-free for 13 months. Neither patient had complications related to their gastrostomy tube site revision in the immediate postoperative period.

Conclusion

Wedge gastrostomy tube site resection is a useful strategy to remove reactive tissue that contributes to leakage around a gastrostomy tube and avoids the morbidities of tube re-siting.

Level of evidence

IV.

Introduction

Venolymphatic malformations are benign, but rare congenital lesions that can be found throughout the body. They tend to grow in size along with the patient, and thus are especially rare in the pre-adolescent pediatric population. In this article, we present a case of a vulvar lymphatic/vascular malformation in a 3-year-old female.

Case Presentation

A 3-year-old, otherwise healthy, female presented with a labial/clitoral mass that had been growing over the past 2 years. A previous biopsy demonstrated benign vascular proliferation with stromal components, concerning for a vascular malformation versus hamartoma. Pelvic MRI was consistent with a lymphatic malformation. She was evaluated by both a pediatric endocrinologist and a genetic counselor who ruled out underlying hormonal abnormalities, such as central puberty or congenital adrenal hyperplasia, or genetic causes, such as neurofibromatosis 1. The 3.6 × 2.1 × 1.7cm mass was surgically excised with a multidisciplinary team with pediatric surgery, gynecologic oncology, and plastic and reconstructive surgery with final pathology demonstrating a lymphatic/vascular malformation. A rotational advancement flap was used to reconstruct the area. At 9 months, the sensation was preserved with good cosmesis.

Conclusion

Vulvar venolymphatic malformations in the pediatric population are exceedingly rare. This is a highly sensitive area and should thus be approached both methodically and cautiously with a multidisciplinary approach.

Introduction

Lymphatic malformations (LMs) or lymphangiomas are rare congenital anomalies affecting the lymphatic system. They are predominantly found in the head and neck, with less common occurrences in abdominal cavity or scrotum. Bleomycin is among the sclerotic agents used, capable of inducing a non-specific inflammatory process leading to thrombosis and fibrosis of the cyst. However, there is limited literature regarding the efficacy of Bleomycin as a standalone treatment for large and mixed types of cystic LMs.

Case presentation

A 17-year-old male presented with swelling in the right inguinal and scrotal regions, without tenderness. Ultrasound and contrast-enhanced computed tomography scan (CT scan) revealed a large, well-defined multiloculated cystic lesion measuring 7x8x8cm in the pelvic region, extending into the left inguinoscrotal region (15x6x8cm), consistent with LMs. Treatment comprised aspiration of cystic fluid followed by Bleomycin injection, administered at a maximum dosage of 0.5 IU/kg body weight under general anesthesia. A sequence of five sclerotherapy sessions of Bleomycin injections was carried out, with each session spaced one month apart. Following 15 months of observation, no intra-abdominal cystic masses were identified, and scrotal ultrasound displayed near-complete resolution. Only a fibrotic tissue measuring 1.5 × 1cm in size was identified in the scrotum, and it remained stable across three consecutive scans, indicating that no further intervention was required.

Conclusion

Based on this case study, sclerotherapy using intralesional Bleomycin injection for large, complex LMs in children appears to be safe and effective. This scarless approach seems to provide good cosmetic outcomes and poses a minimal risk of neurovascular injury.