Introduction Amoebic liver abscess is the most common extraintestinal manifestation of Entamoeba histolytica infection and remains an important cause of morbidity in endemic regions. Transdiaphragmatic rupture with pleural extension is an uncommon complication in children. Case Presentation A 6-year-old boy presented to the hospital with fever, right upper abdominal pain, and progressive respiratory distress. Contrast-enhanced computed tomography (CECT) demonstrated a large right lobe liver abscess with diaphragmatic perforation and extension into the right pleural cavity, resulting in a massive multiloculated pleural collection and compression of the right lung. The child underwent ultrasound-guided drainage of the liver abscess with a simultaneous video-assisted thoracoscopic surgery (VATS) for evacuation of the pleural collection. Thoracoscopy confirmed direct hepatopleural communication through a diaphragmatic defect, allowing complete drainage of the loculated collection without the need for thoracotomy. The postoperative course was complicated by a transient biliary fistula, which was resolved with conservative management. The child made a complete clinical recovery following prolonged anti-amoebic therapy and appropriate antibiotic treatment. Conclusion Children with amoebic liver abscess who develop respiratory symptoms should undergo early thoracic imaging to rule out an intrathoracic extension of the abscess.
Background Congenital lobar emphysema (CLE) is a rare lower respiratory tract developmental anomaly, characterized by hyperinflation of lung lobular tissue, causing compression atelectasis of adjacent lung segments and mediastinal displacement. While CLE typically involves a single lobe, most commonly the left upper lobe or right middle lobe, bilateral involvement is exceptionally rare. Case presentation A 6-month-old male patient presented to emergency department with progressive respiratory distress, tachypnea, subcostal retractions, recurrent chest infections and an SpO2 of 80% on room air. High-resolution CT of the chest demonstrated asymmetric hyperinflation and hyperlucency of the right middle lobe (RML) and the left upper lobe (LUL), with compressive atelectasis. Under general anesthesia with conventional single-lumen endotracheal intubation, a single-stage bilateral lobectomy was performed via sequential thoracotomies, starting with the right middle lobectomy followed immediately by the left upper lobectomy. Bilateral chest tubes were placed intraoperatively. The child was extubated on postoperative day 2, spent 10 days in the hospital, and showed complete resolution of symptoms with full lung re-expansion at the 6-month follow-up. Conclusion Single-stage bilateral lobectomy for bilateral CLE in infants may be safe and effective in selected cases.
Introduction Duplicated external genitalia associated with imperforate anus is exceptionally rare, and its embryologic mechanism and optimal surgical management remain poorly defined. Accurate preoperative delineation of the duplicated genital tract, urinary tract, and anorectal fistula is essential for successful reconstruction. Case Presentation A full-term female infant presented with two clitoris-like structures, two vaginal openings, and an imperforate anus. Chromosomal analysis showed a normal 46,XX karyotype, and pelvic magnetic resonance imaging demonstrated a single uterus and upper vagina with duplication of the lower vagina and clitoris; the rectum inserted into the posterior aspect of the right lower vagina as a rectovestibular fistula. After diverting colostomy, examination under anesthesia with cystoscopy and vaginal catheterization confirmed a single bladder, two urethral openings communicating with a short common channel, and an intervaginal communication between the duplicated lower vaginas. Definitive reconstruction at 10 months of age consisted of excision of the rudimentary left vagina and duplicated vestibular structures with preservation of the right true vagina and urethra, followed by jump-back anoplasty. Colostomy closure was performed at 1 year of age, and early follow-up showed normal urinary voiding and satisfactory bowel function. Conclusion Duplicated external genitalia with imperforate anus may be limited to the distal genital tract, and identifying the vagina connected to the uterus and the urethra suitable for preservation can guide selective excision of rudimentary duplicated structures without urethroplasty
Introduction The coexistence of esophageal atresia (EA) with congenital diaphragmatic hernia (CDH) is exceptionally rare, with reported cases predominantly involving posterolateral Bochdalek defects. The association of a Morgagni hernia with Gross type C EA and distal tracheoesophageal fistula (TEF) appears particularly uncommon. Case presentation A 7-month-old boy underwent an uncomplicated neonatal repair of Gross type C esophageal atresia with tracheoesophageal fistula (EA/TEF). At 5 months of age, he developed a persistent cough and recurrent wheezing, which did not respond to treatment for presumed asthma. Chest radiography revealed an anterior mediastinal air–fluid level. Computed tomography demonstrated a 4-cm anterior diaphragmatic defect containing the transverse colon, consistent with a Morgagni hernia. An upper gastrointestinal contrast study also revealed severe gastroesophageal reflux. An upper midline laparotomy was performed, through which the herniated colon was reduced, the hernia sac excised, and the diaphragmatic defect closed primarily. A Nissen fundoplication was performed concomitantly. The postoperative course was uneventful, with complete resolution of the respiratory symptoms following the combined surgical repair of the Morgagni hernia and gastroesophageal reflux. At 1-year follow-up, the patient remained asymptomatic, with satisfactory growth and no radiological evidence of recurrence. Conclusion Morgagni hernia should be considered in the differential diagnosis of infants who have respiratory symptoms after the repair of esophageal atresia / tracheoesophageal fistula.
Introduction Tubular colonic duplication is a rare congenital anomaly that generally presents as a blind-ending pouch and constipation. Rare fistulous communication with the genitourinary tract can present considerable diagnostic and surgical challenges, particularly when the abnormal anatomy is difficult to delineate using conventional imaging modalities. Case presentation We report the case of a 2-year-old boy with a history of neonatal rectoperineal fistula treated with cutback anoplasty. He also had grade 3 vesicoureteral reflux (VUR) managed with ureteral reimplantation. Despite these interventions, he presented with recurrent urinary tract infections (UTIs), epididymitis and pneumaturia. MRI identified an indeterminate pelvic cyst. However, both MRI and voiding cystourethrography (VCUG) failed to detect a rectourethral fistula. Contrast-Enhanced Colosonography (CECS) was subsequently performed and successfully identified a tubular colonic duplication. The imaging demonstrated that the posterior limb of the duplication connected to the neoanus, while the anterior limb communicated with the prostatic urethra. The patient underwent laparoscopic-assisted mucosectomy of the anterior duplication limb and closure of the rectoprostatic fistula. Postoperative recovery was uneventful. At the one-year postoperative follow-up, the patient demonstrated normal defecation and urination with complete resolution of infections and epididymitis. Conclusions Complex colonic duplication should be considered in children with anorectal malformations and persistent urinary symptoms. CECS seems to be a valuable tool for identifying occult fistulas.
Introduction Although pediatric breast tumors are uncommon and the vast majority are benign, atypical lesions require careful classification to guide management, intervention, and surveillance. We present a case of a rare pediatric breast tumor with overlapping histopathologic features. Case Presentation A 17-year-old female presented with recurrent swelling of the right nipple-areolar complex one year after excision of a presumed benign lesion. Ultrasound demonstrated a lobulated heterogeneous subareolar mass with a distinct but contiguous component closer to the areolar skin. The mass was excised and measured 4.7 x 4.3 x 2.5 cm with cystic, mucinous, and solid components. Histologic evaluation revealed a well-circumscribed lesion composed predominantly of bland stellate to spindle cells in abundant myxoid stroma with associated mucin and mild cytologic atypia without mitoses or necrosis. Immunohistochemistry demonstrated diffuse CD34 positivity, retained Rb1 expression, patchy S100 positivity, and negative desmin and ER staining. Histochemical stains were positive for Alcian blue, Hale’s colloidal iron, and mucicarmine, and negative for PAS. These findings demonstrated overlapping features of nodular mucinosis and MMF. Given the benign nature of both entities, close clinical surveillance and serial examination was recommended. The patient has done well with excellent cosmesis of the resection site, no postoperative complications, and no concerning findings on clinical follow up examinations. Conclusion Breast masses in female teenagers may have more than one histopathological type with overlapping cellular components. As seen in our case, the simultaneous presence of features of nodular mucinosis and myxoid mammary myofibroblastoma is possible.
Introduction Long-gap esophageal atresia (LGEA) remains challenging, particularly after esophagostomy following failed repair. Case presentation An 11-month-old girl with type A esophageal atresia (EA) was referred after initial management at another institution. Based on a medical-record extract and clinical history, gastrostomy was performed on day 3. At 53 days, thoracoscopy identified a six-vertebral-body gap (T2-T8), and internal traction was initiated. Eight days later, the traction sutures cut through both segments, causing exudate and inflammation; a cervical esophagostomy was created and the distal segment was closed. Because the proximal segment had been exteriorized from the posterior mediastinum, fluoroscopy could not provide a meaningful two-segment gap measurement or three-dimensional (3D) distal pouch assessment. Two CT examinations using gastrostomy-administered enteral contrast, intravenous contrast, pediatric dose-reduction settings, and 3D reconstruction defined the distal pouch’s cranial extent, caliber, course, and relationships to adjacent mediastinal structures. Three thoracoscopic distal-segment traction procedures were performed at 1.5 to 3-month intervals; the first two were combined with extra-thoracic Kimura elongation of the proximal segment. A tension-free anastomosis was created by right thoracotomy at 2.2 years. Four balloon dilatations treated an anastomotic stricture. Gastrostomy feeding ceased at 2.5 years, and the tube was removed six months later. At the last follow-up at 4.5 years of age, she tolerated a full oral diet without dysphagia. Conclusion Thoracoscopic serial internal traction of the distal esophagus in addition to extra-thoracic elongation of the proximal pouch may allow an eventual esophageal anastomosis in selected patients with type A esophageal atresia who underwent a previous esophagostomy.
INTRODUCTION Antenatally diagnosed mediastinal teratomas are rare neonatal tumours that may cause significant cardiopulmonary compromise because of their location and mass effect. Prenatal detection facilitates planned postnatal evaluation and timely surgical management. CASE PRESENTATION A male fetus was diagnosed at 32 weeks' gestation with a cystic retrosternal anterior mediastinal lesion on routine obstetric ultrasonography. There was no polyhydramnios, fetal hydrops, or associated congenital anomaly, and the fetus remained haemodynamically stable without evidence of airway or cardiovascular compromise. The pregnancy was managed expectantly without fetal intervention. A 3.2-kg appropriate-for-gestational-age male infant was delivered vaginally at 37 weeks' gestation and remained asymptomatic without respiratory compromise.Postnatal chest radiography and contrast-enhanced computed tomography demonstrated a 6.5 × 4.1 × 4 cm heterogeneous anterior mediastinal mass with predominantly cystic components and focal soft-tissue attenuation, compressing the heart and adjacent right lung without radiological evidence of invasion.Serum alpha-fetoprotein and beta-human chorionic gonadotropin levels were appropriate for age. Complete excision was performed on day 8 of life through a right posterolateral thoracotomy. Intraoperatively, the encapsulated tumour occupied the anterior mediastinum and was adherent to the thymus without invasion of adjacent mediastinal structures. It was dissected free and removed en bloc with an intact capsule, without rupture or tumour spillage. Histopathological examination demonstrated a Grade III immature teratoma with clear surgical margins. In view of complete excision, the absence of malignant germ cell components or residual disease, and age-appropriate tumour marker levels, the multidisciplinary tumour board recommended surveillance without adjuvant chemotherapy. At 8 months of follow-up, the infant was thriving, with no clinical, biochemical, or radiological evidence of recurrence. CONCLUSION Early prenatal detection and complete surgical excision may enable favourable outcomes with surveillance alone, even in neonatal Grade III mediastinal immature teratomas.
Introduction Optimal management of pediatric choledocholithiasis is not widely established. Endoscopic retrograde cholangiopancreatography (ERCP) is widely used in adults, but it is often technically challenging due to size limitations in young children especially in the setting of stone impaction in the distal common bile duct (CBD) leading to lower success rates which often necessitate surgical intervention. Case Presentation We present a 6-year-old girl with hereditary spherocytosis who presented with abdominal pain, jaundice, dark urine, conjugated hyperbilirubinemia, elevated transaminase levels, and pancreatitis. Ultrasound demonstrated hepatomegaly, a sludge-filled enlarged gallbladder, and common bile duct (CBD) dilation, while magnetic resonance cholangiopancreatography identified a distally impacted CBD stone. Initial endoscopic retrograde cholangiopancreatography using an Olympus TJF-190V duodenoscope was unsuccessful because the impacted stone prevented cannulation of the ampulla. A laparoscopic–endoscopic rendezvous procedure was subsequently performed using a 5 mm umbilical port, 5 mm right anterior axillary and right midclavicular ports, and a 12 mm epigastric port. Intraoperative cholangiography demonstrated an abrupt distal CBD cutoff. A 5 Fr biliary catheter and 0.035 inch guidewire were advanced transcystically into the duodenum under fluoroscopic guidance. The guidewire was captured endoscopically with a snare, permitting advancement of the sphincterotome, CBD cannulation, sphincterotomy, stone extraction, and biliary stent placement. Completion cholangiography confirmed unobstructed biliary drainage, and laparoscopic cholecystectomy was performed. The patient had no postoperative complications, demonstrated improvement in liver function tests and bilirubin levels, and was discharged four days after the procedure. Approximately one month later, she underwent repeat ERCP for removal of a residual stone and the biliary stent. Her symptoms subsequently improved, and she continues to follow with gastroenterology and hematology/oncology for management of hereditary spherocytosis. Conclusion The laparoscopic assisted rendezvous technique appears to be a safe and effective approach for the management of choledocholithiasis in children.
Introduction Spastic left colon syndrome is a recently described complication of chronic antegrade continence enema (ACE) therapy characterized by flush-related symptoms and contrast enema findings of a dilated proximal colon with a spastic left colon. Management strategies have not been previously described. Case Presentations Four adolescents with spastic left colon syndrome were treated between 2018 and 2023. A 16-year-old female with Hirschsprung disease developed urgency, watery stools, bloating, and incomplete evacuation after 10 years of ACE therapy. She successfully transitioned off enemas to senna and remained symptom-free at 2-year follow-up. A 16-year-old male with anorectal malformation developed worsening fecal incontinence after 3 years of ACE use. Reduced flush frequency and loperamide allowed eventual discontinuation of enemas and medications, with sustained symptom resolution at 2 years. A 16-year-old male with anorectal malformation developed urgency, frequent small-volume stools, and activity-related symptoms after 5 years of ACE therapy. Senna alone was unsuccessful; gradual reduction of flush volume combined with intermittent loperamide resulted in predictable bowel function without urgency or accidents at 6-month follow-up. An 18-year-old female with spina bifida developed prolonged evacuation times and fecal incontinence after 8 years of ACE therapy. Hyoscyamine before flushes and intermittent bisacodyl improved continence and evacuation efficiency. All patients required close follow-up because minor regimen adjustments produced substantial symptom changes. Conclusion Decreasing flush volume and frequency with the addition of medication to slow colonic motility appears to be most helpful in strategy for the management of children with spastic left colon syndrome.
Introduction Pheochromocytomas and paragangliomas (PPGL) are neuroendocrine tumors that arise from the adrenal medulla and extra adrenal paraganglia. Catecholamine excess is characterized by episodic or persistent hypertension, tachycardia, and rarely, catecholamine-induced cardiomyopathy. Case Presentation A 6-year-old girl presented with anxiety, palpitations, diaphoresis, and worsening dyspnea without orthopnea. Diagnostic work-up was notable for family history of VHL, elevated catecholamines, and imaging that demonstrated a retroperitoneal mass near the left adrenal gland consistent with a paraganglioma. Echocardiogram showed dilated left that included initiating an alpha-adrenergic-blockade for blood pressure (BP) control, careful volume expansion, and with an ACE-inhibitor for supplemental BP control. An inotropic agent was given prior to for beta-blockade. A tyrosine hydroxylase inhibitor treated paroxysmal increases in pressure. The patient had severe anxiety requiring intensive psychological management . The medical team selected a window of physiologic stability for surgery accepting a low EF. The paraganglioma was removed with intraoperative stabilization of heart rate and BP. Post-operatively, she stayed in the ICU for 4 days, with a slow wean of cardiotropic medications. EF had recovered to near-normal levels ten months later. She will undergo lifetime surveillance for PPGL recurrence and other manifestations of VHL. Conclusion Preoperative optimization of patients with catecholamine-induced cardiomyopathy may require alpha and beta blockade, adjuncts for BP and tyrosine hydroxylase. While BP and HR may normalize, full cardiac function recovery may not occur in the presence of a secreting tumor. Preparation for catecholamine surges and cardiac failure during tumor removal is mandatory. Proactive management of anxiety is essential.
Introduction Congenital thymic cysts are rare benign mediastinal lesions that are infrequently encountered in neonates. Prenatal detection is uncommon, and hemorrhagic degeneration may further obscure their radiologic appearance, making preoperative distinction from malignant mediastinal tumors challenging. Case Presentation A term neonate with a prenatally detected mediastinal mass developed respiratory distress shortly after birth, requiring neonatal intensive care admission. Chest radiography, ultrasonography, and contrast-enhanced computed tomography demonstrated a large heterogeneous anterior mediastinal mass with cystic and solid components, raising suspicion for a mediastinal teratoma. Owing to persistent respiratory compromise and concern for malignancy, the patient underwent right anterior thoracotomy on the fifth day of life. Intraoperatively, the lesion was found to contain extensive clotted blood and was completely excised. Histopathological examination demonstrated thymic tissue with scattered Hassall corpuscles, extensive intracystic hemorrhage, cystic degeneration, and inflammatory changes, confirming the diagnosis of a hemorrhagic thymic cyst without evidence of malignancy. The postoperative course was uneventful, with marked improvement in respiratory status. Conclusion Hemorrhagic thymic cyst should be considered in the differential diagnosis of anterior mediastinal masses presenting with neonatal respiratory distress.
Background Gastric duplication cysts (GDCs) are rare congenital malformations, accounting for 2–8% of alimentary tract duplications. Intrathoracic location is exceptionally uncommon, and ectopic pancreatic tissue within the cyst lining has rarely been described in this location. Case Presentation We report a 1-year-6-month-old boy who presented with respiratory distress two weeks after an initial presentation elsewhere for fever, tachypnea, and vomiting, where imaging showed a posterior mediastinal mass and an attempted excision by thoracotomy was aborted intraoperatively due to surgical difficulty. On transfer, chest CT revealed a large left pneumohydrothorax with complete lung collapse and mediastinal shift. Chest tube drainage yielded turbid "café-au-lait" fluid, and broad-spectrum antibiotics were started. Left posterolateral thoracotomy 48 hours after chest tube insertion revealed a 6x4.5cm retropleural cystic mass connected to the abdominal cavity via a tract, excised using a Harmonic ultrasonic scalpel. Histopathology confirmed a gastric duplication cyst with antral gastric mucosa and ectopic pancreatic tissue comprising 10% of the lining, with mucosal ulceration. The patient recovered uneventfully and was discharged on postoperative day 5, remaining asymptomatic with full lung re-expansion at 1-month follow-up. Conclusion Gastric duplication should be included in the differential diagnosis of children who develop respiratory symptoms and are found to have a mediastinal mass on imaging studies.
Introduction Renal Ewing Sarcoma (EWS) is a rare, malignant tumor with a poor prognosis. Its nonspecific presentation often delays diagnosis, and standardized treatment guidelines remain limited. Case Series Case 1 involved a 12-year-old girl with abdominal pain, gross hematuria, and polyuria. Symptoms persisted for 4 months despite antibiotic treatment for presumed urinary tract infection, after which a palpable right abdominal mass was detected. Imaging revealed a heterogeneous right renal mass with extensive necrosis and calcifications. Right radical nephrectomy and lymph node dissection were performed, and pathology confirmed renal EWS. She received adjuvant vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide over 7 months. At six months off treatment, the patient remained disease-free but developed renal dysfunction and hypotension.Case 2 concerned a 13-year-old girl with a history of treated abdominal group A Burkitt lymphoma. Surveillance ultrasound detected a new right renal lesion while asymptomatic. CT-guided biopsy, histopathology, and identification of an EWSR:FLI1 fusion gene confirmed renal EWS. Following 3 months of neoadjuvant chemotherapy with vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide, open radical nephrectomy was performed for local control. She experienced complications during consolidation chemotherapy, including necrotizing colitis and Clostridium septicum sepsis, and died six months after diagnosis. Conclusion Management of pediatric renal EWS should incorporate multimodal therapy centered on chemotherapy and surgical resection while carefully considering the risk of treatment-related toxicity, particularly in patients with prior chemotherapy exposure or a solitary functioning kidney.
Introduction Hepatic hemangiomas are the most common benign vascular liver tumours in children. While typically solid, these lesions may rarely undergo cystic degeneration, significantly altering their radiological presentation. The resulting cystic morphology can closely mimic simple hepatic cysts or other cystic liver lesions on imaging, posing a major diagnostic pitfall — particularly in resource-limited settings where contrast-enhanced studies may be unavailable. Case presentation A 4-year-old girl presented with a 6-month history of progressive abdominal distension. A palpable, painless mass extended from the right flank to the left hypochondrium. Abdominal ultrasonography revealed a large hepatic cyst with communicating locules. Non-contrast abdominopelvic computed tomography showed a 17.2 × 12.1 × 9.8 cm hypodense cystic formation with internal septations in the right hepatic lobe, initially misdiagnosed as a simple hepatic cyst. Tumour markers including alpha-fetoprotein were normal. At surgery, aspiration yielded 1250 mL of clear serous fluid. A well-defined cleavage plane allowed complete excision of the mass from segments V and VIII. The cyst wall was thick with a highly vascularised internal lining. Histopathological examination revealed dilated vascular channels lined by flattened endothelial cells, confirming cavernous hemangioma. Recovery was uneventful, and the patient remained asymptomatic at 3 months' follow-up. Conclusion Extensive cystic degeneration can make a cavernous hepatic hemangioma yield clear serous — not haemorrhagic — fluid and mimic a simple hepatic cyst; a non-haemorrhagic aspirate does not exclude a hemangioma. When contrast-enhanced imaging is unavailable, a thick vascularised wall and internal septations should redirect the differential toward a cystic vascular tumour.
Introduction Cervico-thoracic ganglioneuromas are rare, benign neurogenic tumors that are rare in children under five years of age. Management of acute airway compromise caused by a massive superior mediastinal mass extending into the neck with critical vascular encasement is challenging. Case Presentation A four-year-old girl presented with fever, persistent cough, and progressive respiratory distress. Magnetic Resonance Imaging revealed a massive lobulated mass of 5.7*7*9.2 cms occupying the superior, posterior, and middle mediastinum, extending into the lower neck and completely encasing the right subclavian artery. A multidisciplinary pediatric and cardiothoracic team performed a near-total surgical debulking through a cervico-thoracic approach (with a midline sternotomy extending into the neck towards right), intentionally leaving a small residual cuff of tumor to avoid catastrophic injury to the encased subclavian vessels. Postoperative histopathology confirmed a mature ganglioneuroma with clusters of mature ganglion cells and a dense stroma of Schwann cells. The patient was successfully extubated on postoperative day 1, discharged on day 13, and remains completely asymptomatic at her three-month follow-up. Conclusion Surgical debulking may be a safe and effective treatment strategy for benign pediatric ganglioneuromas when complete excision poses a high risk of major neurovascular injury.
Introduction Sacrococcygeal teratoma is the most common congenital neoplasm and predominantly affects female infants. Warm autoimmune hemolytic anemia (WAIHA) is rare in childhood, and its association with teratoma, particularly sacrococcygeal teratoma, is exceptionally uncommon. Case Presentation We report a 1-month-old female infant admitted for severe WAIHA revealed by acute jaundice, dark urine, hepatosplenomegaly, profound regenerative anemia, and a direct antiglobulin test positive for immunoglobulin G. Clinical examination identified a sacrococcygeal mass, and pelvic magnetic resonance imaging demonstrated a solid-cystic lesion with significant intrapelvic extension, suggestive of an Altman type II sacrococcygeal teratoma. Initial management included packed red blood cell transfusions, intravenous methylprednisolone followed by oral prednisone, and folic acid supplementation. Complete surgical excision with coccygectomy was performed, and histopathological examination confirmed a mature teratoma without malignant features. Hemoglobin normalized by postoperative day 15, the direct Coombs test became negative, and the child remained clinically well at 2 years of follow-up, with preserved psychomotor development, walking, posture, and fecal continence. Conclusion Severe WAIHA in an infant with a sacrococcygeal mass should prompt evaluation for a tumor-associated process, as complete tumor excision may be followed by hematologic remission.
Introduction Paediatric abdominal lymphatic malformations (LM) are rare and challenging lesions, particularly when deeply located or involving multiple compartments. Conventional management (surgery or percutaneous sclerotherapy) may be limited by incomplete resolution or procedural morbidity. Bleomycin electro-sclerotherapy (BEST) is a promising minimally invasive technique, although its role in intra-abdominal disease remains poorly defined. Case series A 25-month-old child affected by a microcystic LM of the tongue and a mesenteric macrocystic LM (90 × 20 × 70 mm) in the jejunum. BEST was selected to reduce the total bleomycin dose and optimize therapeutic outcomes. A laparoscopic approach was elected for the treatment of the mesenteric lesion, whilst the LM of the tongue was treated with a linear electrode. The procedure was performed under general anaesthesia. The lingual LM showed a minor focal recurrence at 8 months, successfully treated with corticosteroids. MRI at 9 months did not demonstrate any significant residual lingual or abdominal disease.A 26-month-old child was affected by a mesenteric macrocystic LM (90 × 57 × 37 mm) in the ileum. Given the large extent of the lesion we opted to perform BEST under laparoscopy, aiming to preserve the involved organs while administering a reduced dose of bleomycin. Ultrasound at both 1 and 3 months demonstrated complete resolution of the lesion, which was subsequently confirmed by MRI at 6 months and abdominal pain disappearance. Conclusion The combination of BEST and laparo-assisted management appears to be a feasible and safe minimally invasive strategy for selected pediatric abdominal lymphatic malformations.
Introduction The upper gastrointestinal (UGI) series is the reference-standard imaging study for suspected malrotation in neonates, yet a falsely reassuring study is a recognized pitfall that can delay life-saving surgery. Case Presentation Case 1 was a term male (37+3 weeks) who presented on day of life (DOL) 1 with bilious emesis and failure to pass meconium. An initial UGI was equivocal, with a duodenal C-loop that did not definitively cross the midline but no identified obstruction; he was admitted to the neonatal intensive care unit and managed with orogastric decompression while bilious output continued. A repeat UGI on DOL 7, obtained because symptoms persisted, showed dilated duodenal loops with reduced distal progression, prompting laparotomy on DOL 8 that revealed malrotation with a 720° midgut volvulus and viable bowel. Case 2 was a late-preterm female (35+5 weeks) admitted for respiratory distress and suspected sepsis, with a distended, gas-filled stomach on plain radiograph requiring Replogle suction. An initial UGI suggested normal rotation but did not clearly identify the duodenal sweep; a second UGI on DOL 2, obtained after rectal irrigation to clarify the equivocal duodenal anatomy, showed narrowing at the second–third duodenal junction with only a trickle of distal contrast. Laparoscopic exploration on DOL 3, converted to open, confirmed malrotation with a 270° volvulus and viable bowel. Both neonates underwent Ladd’s procedure with appendectomy and recovered without long-term sequelae. Conclusion A negative or equivocal UGI does not exclude malrotation with volvulus. Patients with suspected midgut volvulus should be explored regardless of the imaging findings.
Introduction Surgery for rectovestibular fistula with a normal anus (H-fistula) is challenging because of its high recurrence rate. We report successful treatment of recurrent H-fistula using perineal body reconstruction. Case presentation A girl presented with perineal skin erosion and was diagnosed with an H-type rectovestibular fistula at 3 months of age. At 1 year of age, she underwent fistula excision using the vestibuloanal pull-through technique; however, the fistula recurred 2 months later. Because fecal leakage occurred only with watery stools, revision surgery was deferred until further growth. During follow-up, she was diagnosed with type 1 hyper-IgE syndrome after evaluation for recurrent pneumonia, and reoperation was postponed until 10 years of age. With the patient in the lithotomy position, a transverse perineal incision was made, and the perineal body was exposed by deep dissection. The fistula was divided, and the vestibular and rectal defects were closed separately. The lateral extensions of the perineal body (LEPBs), a connective tissue mass extending laterally on both sides of the perineal body, were then mobilized bilaterally, approximated in the midline with 3-0 absorbable sutures to recreate the central perineal body, and reinforced by multilayered closure from the deep to the superficial plane. Oral intake was resumed on postoperative day 1, and the patient was discharged on postoperative day 4. No wound complications or recurrence were observed during 12 months of follow-up, and bowel function remained unchanged. Conclusion Perineal body reconstruction seems to be a feasible and effective treatment option for recurrent H-type rectovestibular fistulas.
