Journal of Pediatric Surgery Case Reports最新文献

Introduction

Nodular lymphoid hyperplasia is usually encountered by gastroenterologists during a workup for chronic abdominal pain accompanied by food intolerance, hematochezia, or diarrhea. When an intestinal endoscopy is performed, the usual finding is multiple small submucosal nodules that are identified as lymphoid tissue by biopsy.

Case presentation

A previously healthy 8-year-old boy presented with a several-day history of intermittent abdominal pain and vomiting. He had no history of diarrhea or bloody stools. He was afebrile, and all of the other vital signs were normal. His abdomen was distended and tender, particularly in the right upper quadrant. Blood work was normal, except for an elevated C-reactive protein. His imaging work-up consisted of an abdominal ultrasound that ruled out acute appendicitis and a computerized tomography (CT) scan that showed images suggestive of ileo-colic intussusception, with a soft tissue mass in the right upper quadrant. Due to his age, reduction of the intussusception by insufflation was not attempted. An exploratory laparotomy was done through an incision in the right lower quadrant. We found no intussusception; rather, the wall of the cecum and the base of the appendix were markedly thickened, causing a partial obstruction of the cecal lumen and the ileo-cecal valve. We proceeded with an ileo-cecectomy and ileocolic anastomosis. He was discharged home on the 4th postoperative day. The pathologic and molecular analysis confirmed the diagnosis of follicular lymphoid hyperplasia.

Conclusion

Nodular lymphoid hyperplasia of the ileo-cecal region can mimic an ileo-colic intussusception. It should be included in the differential diagnosis, particularly in patients outside the age range of idiopathic intussusception.

Background

Fetus in fetu (FIF) is an exceedingly rare congenital anomaly characterized by the presence of a partially developed fetus within the body of its twin. It is a rare entity with fewer than 200 cases documented worldwide. Typically presenting as an abdominal mass in early childhood, this condition remains a diagnostic challenge due to its infrequency and varied clinical manifestations.

Case presentation

We present the case of a 1-year-old female child referred to the pediatric surgery department due to an abdominal mass detected by her parents. Physical examination revealed a firm, ill-defined mass in the right upper abdomen. Ultrasonography delineated a heterogeneous, calcified mass in the right upper quadrant. Laboratory investigations (complete blood count, viral markers), including tumor markers (AFP, beta-hCG), were within normal limits.

A midline laparotomy was performed, and intraoperative findings revealed a well-encapsulated mass adherent to the retroperitoneum, adjacent to the liver. Careful dissection was carried out to preserve vital structures, and the mass was successfully removed without rupture. Histopathological examination confirmed the diagnosis of fetus in fetu, displaying a spectrum of tissues reminiscent of embryonic development, including skin, adnexal structures, brain tissue, mature cartilage, adipose tissue, bone marrow elements, and a partially developed vertebral column. After the surgical resection, the patient experienced an uneventful postoperative recovery. At one year of follow up the patient has shown no signs of recurrence.

Conclusion

FIF is characterized by diagnostic challenges, often requiring postoperative histological confirmation. Histopathological examination confirmed the presence of FIF. Surgical excision, is crucial for favorable outcomes, especially in preventing potential complications. Comprehensive histopathological evaluation remains imperative for accurate diagnosis.

Introduction

Forkhead box protein P1 (FOXP1) is a transcriptional factor that plays a role in gene regulation in a wide array of human tissues. Mutations of this gene result in developmental delay, language deficits, brain, cardiac and urogenital anomalies. Some evidence also connects these mutations with congenital diaphragmatic hernia (CDH) and Hirschsprung's disease (HD). This report describes a case of a newborn with a FOXP1 mutation who was diagnosed with both CDH and HD, suggesting the gene's possible association with both phenotypes.

Case report

The patient was born at 38w2d to a healthy mother. Multiple congenital anomalies were discovered prenatally, including Dandy-Walker malformation, bilateral cleft lip and palate, polydactyly and syndactyly. No chromosomic aneuploidies were identified; however, whole exome sequencing analysis detected a missense mutation of FOXP1. The patient passed meconium on day-of-life zero and progressed appropriately. Presentation of CDH manifested at 7 weeks with respiratory failure following delayed intrathoracic herniation. Chest x-ray confirmed the diagnosis, and a small type A defect was identified at the time of surgery. At 12 weeks, worsening abdominal distention prompted workup of Hirschsprung's disease. Suction rectal biopsy confirmed the diagnosis. Both CDH and HD were addressed surgically upon diagnosis. Post-operative courses were uncomplicated.

Conclusion

This report demonstrates an unusual association of CDH and HD in a patient with a FOXP1 mutation. Both diseases were on the mild spectrum and demonstrated delayed presentations. The role of this FOXP1 mutation as a genetic cause of both diseases warrants further investigation.

Introduction

Mediastinal teratomas are rare in children and adolescents; however, they often present diagnostic and therapeutic challenges, given their potential for malignancy. Rapid intervention, particularly surgical excision, is crucial for large, life-threatening masses following clinical stabilization.

Case presentation

A 9-year-old female presented with progressive left costal pain and dyspnea on exertion, culminating in acute respiratory failure prior to admission. Examination revealed a hypopigmented square patch in the left lower quadrant of the abdomen. Initial chest x-ray (CXR) showed complete opacification of the left lung with mediastinal shift to the right. Subsequent chest CT scan confirmed a 14.2cm × 11.2cm x 14.5cm complex, multiloculated, multiseptated mass suggestive of neoplasia. Local mass effect resulted in the obliteration of the left mainstem bronchi, left upper and lower lobe bronchi, lung atelectasis, and a large left pleural effusion. Surgical excision was planned following consultation with Pediatric Anesthesiology and Intensivist. The patient underwent left posterolateral thoracotomy through the fifth intercostal space, during which a large complex cystic mass (17.5 cm × 16.8 cm x 7.7 cm) arising from the thymus was excised after careful dissection from surrounding lung parenchyma and pericardium. Postoperatively, she recovered well in the Pediatric Intensive Care Unit (PICU) and was discharged on postoperative day #8 after pathology confirmed a benign teratoma.

Conclusion

Mediastinal teratomas in pediatric patients presenting with life-threatening signs and symptoms necessitate early recognition, involvement of a multidisciplinary team, and prompt surgical excision to ensure optimal outcomes.

Introduction

Septic thrombophlebitis (STP) of deep veins is a rare condition that harbors significant morbidity in the pediatric population. Most cases of STP in pediatric patients have been associated with head and neck infections such as in Lemierre syndrome.

Case presentation

A 3-year-old male presented with a 10-day history of fever, abdominal pain, and diarrhea and was diagnosed with perforated appendicitis. He underwent laparoscopic hand-assisted appendectomy and was discharged on post-operative day 5. He re-presented to the emergency department two weeks later with several day history of abdominal distension and vomiting and imaging demonstrated a high-grade small bowel obstruction. He underwent exploratory laparotomy and ileocecectomy. His postoperative course was complicated by acute respiratory failure secondary to abdominal compartment syndrome requiring decompressive laparotomy. His clinical status improved and he was able to undergo abdominal closure 4 days later. He then developed persistent fevers and was found to have deep venous thrombosis of bilateral femoral veins extending to the infrarenal inferior vena cava consistent with septic thrombophlebitis. He was successfully treated for his deep venous thrombosis with a course of antibiotics and therapeutic anticoagulation and has made a full recovery.

Conclusion

STP of deep veins is generally managed with antibiotics and therapeutic anticoagulation and rarely requires invasive interventions. This case highlights that in young children, the inferior vena cava is small enough caliber to develop thrombosis and that STP of the pelvic veins and inferior vena cava can occur secondary to intraabdominal infection and its sequelae.

Introduction

Conjoined twins are rare congenital malformations that occur in identical twins who share both a placenta and an amniotic sac. If one twin dies, emergency separation is necessary to save the other twin.

Case presentation

A pair of conjoined twin girls, one stillborn and one alive, was referred to our facility 18 hours after delivery from a primary health facility. The twins were diagnosed at birth in a primigravida who had obstructed labor and were delivered by emergency cesarean section. They were joined at the lower chest and upper abdomen and had separate umbilical cords. Imaging studies to determine the extent of organ involvement in the twins were not conducted due to the unavailability of the necessary equipment at our facility. By the second day of life, the surviving twin showed sepsis and was given intravenous fluids and antibiotics. On the sixth day of life, they were taken to the operating room for separation. The twins shared part of the abdominal wall, part of the chest wall and had fused livers, all of which were separated. Following the operation, the surviving twin was treated for sepsis and received nutritional rehabilitation. After a hospital stay of six weeks she was discharged home.

Conclusion

An urgent operation to separate conjoined twins when one of them is stillborn is mandatory, even in a setting with limited diagnostic and surgical capabilities.

Introduction

Pediatric gastric volvulus (GV) with diaphragmatic eventration is a rare but lethal condition.

Case presentation

A 1-year-old girl complained of progressive vomiting, abdominal pain, and distension. On examination, plain abdominal radiograph demonstrated an air bubble sign in the left upper abdomen with elevation of the hemidiaphragm, while water-soluble contrast follow-through imaging showed partial gastric rotation. During exploratory laparotomy, gastric volvulus (GV) with left diaphragmatic eventration was identified. This abnormality was reduced by performing diaphragmatic plication and gastropexy. Postoperatively, the patient recovered without complications and was discharged on the tenth day of treatment.

Conclusion

Pediatric gastric volvulus (GV) with diaphragmatic eventration is a surgical emergency. This case underscores the importance of considering GV in pediatric patients presenting with vomiting and abdominal symptoms, specifically when radiographic evidence suggests gastric distension and diaphragmatic abnormalities.

Introduction

Pneumoperitoneum in neonates is an ominous sign of gastrointestinal (GI) perforation which often requires emergent surgical intervention. Although Meckel's diverticulum (MD) is the most common congenital anomaly of the GI tract, spontaneous MD perforation leading to pneumoperitoneum in the immediate postnatal period is an extremely rare occurrence. This report describes the case of a perforated MD presenting as pneumoperitoneum on day of life (DOL) one.

Case presentation

A 1-day-old 36 week gestational age male presented with acute abdominal distention and guarding upon examination. Two-view abdominal radiographs revealed pneumoperitoneum without pneumatosis intestinalis, portal venous gas, or bowel dilation. Exploratory laparotomy was performed and bilious ascites was encountered – secondary to a perforated MD in the distal ileum. The perforated MD and adjacent small bowel were resected and a double-barrel ileostomy was created. He recovered successfully and without complication in the acute postoperative period. Histopathologic findings were consistent with a perforated MD with ectopic pancreatic tissue.

Conclusion

This rare case highlights the importance of early surgical intervention for neonates with pneumoperitoneum secondary to a perforated MD. Heightened clinical suspicion for a perforated MD should be considered among term/late preterm neonates presenting with pneumoperitoneum and low suspicion for necrotizing enterocolitis.

Introduction

The combination of a giant omphalocele (GO) and congenital diaphragmatic hernia (CDH) is extremely rare and results in high mortality and morbidity rates in neonates. The most difficult period for survival is the immediate postnatal period, with approximately one-third of newborns dying within the first 48 h after birth.

Case presentation

We present the case of a newborn girl with a GO and congenital left-sided Bochdalek hernia who survived beyond the neonatal period and underwent radical surgery. The female neonate, weighing 2946 g, was delivered by scheduled Cesarean section at 37 weeks gestation. She had a left-sided Bochdalek CDH containing a small portion of the small bowel and a GO containing a large portion of the liver. There were no other associated malformations, and the pulmonary hypoplasia and pulmonary hypertension in the neonatal period were not fatal. We performed an operation to repair the diaphragmatic hernia, initially approaching it from her abdominal wall defect. We performed multiple operations to close the abdominal wall. Initially, during the neonatal period, we closed it with a skin layer; however, poor weight gain occurred afterward. Considering gastroesophageal reflux as a complication, we performed mesh-assisted abdominal wall closure and fundoplication at 10 months of age, successfully resolving the underlying disease and complications.

Conclusion

Repair of the CDH from the abdominal wall defect provided good visual field and safer primary direct closure. Without using artificial materials in the CDH until closure of the GO avoided infection and resulted in a favorable outcome in this case.

Introduction

Porcelain gallbladder (PGB) is a gallbladder with calcium encrusted on its inner wall. PGB is rare in the pediatric population with only four cases reported in the literature. We present a patient who is the youngest reported case of porcelain gallbladder.

Case presentation

A 4-year-old full-term male with a history of chronic constipation presented with abdominal pain prompting a plain abdominal X-ray which revealed an incidental calcified gallbladder. This was confirmed with an abdominal ultrasound (US) and computed tomography (CT) that identified calcifications in the gallbladder wall. An elective laparoscopic cholecystectomy was performed, given the reported association of PGB with gallbladder cancer. Final pathology confirmed calcification of the gallbladder, along with evidence of cholelithiasis, and cholecystitis, but no dysplasia or malignancy. The patient was discharged on post-operative day 2 without any complications.

Conclusion

A PGB is a rare finding in the pediatric population, with prior studies indicating an association with malignancy and cholecystitis in the adult population. However, there is not enough data to support the association of PGB with future pathologies in the pediatric population and surgical intervention may not be warranted.