Calcification within the urachus: A case report of rare presentation of urachal cyst with calculus formation.

Urachal anomalies are rare congenital lesions of the genitourinary tract and are important causes of pediatric and adolescent hospital presentations. It can mimic many other causes of intraabdominal pathology and fever, and pose diagnostic challenges, often aided by imaging, is crucial to prevent complications such as infection and calculus formation. Surgical intervention, preferably laparoscopic, is the primary treatment, with complete excision necessary to avoid potential malignant transformation.

Intraoperative electrocorticography-guided resection of the epileptogenic zone in an unusual porencephalic cyst: case report and literature review.

Introduction: Porencephalic cysts resulting from perinatal artery infarctions typically manifest as large cysts accompanied by pre-existing neurological deficits. A small porencephalic cyst without any neurological deficit is a rare cause of medically refractory epilepsy. Case presentation: A 23-year-old female presented with a history of medically refractory epilepsy secondary to a small right parieto-temporal porencephalic cyst. Despite optimal anti-seizure medications, seizures persisted. Surgical intervention was planned, and intraoperative electrocorticography (ioECoG) was used to delineate the epileptogenic zone (EZ), which was found to be two gyri posterior to the cyst. Discussion: Very focal ischaemia resulting in a small porencephalic cyst from perinatal artery infarction exhibits a distinct organization of the EZ involving wider area posteriorly indicating involvement of arterial territory distal to the cyst. This contrasts with the typical perilesional EZ observed in other lesional epilepsy causes. Conclusion: Our findings emphasize the need to consider aetiology during interpretation of ioECoG to better define the electrophysiological border between the normal and epileptogenic brain, aiding in achieving a better surgical outcome.

A Case Report of Ruptured Intracranial Dermoid Cyst.

Intracranial dermoid cysts, rare congenital lesions originating from ectodermal elements during neural tube closure, are explored in the context of a 45-year-old female presenting with a sudden-onset severe headache, nausea, and vomiting. A thorough neurological examination revealed no focal deficits, prompting a computed tomography scan that identified multiple extra-axial intracranial fat density lesions indicative of dermoid cysts. Laboratory and cerebrospinal fluid analysis confirmed inflammatory changes, characterized by an increased white blood cell count. Successful surgical intervention followed, resulting in the complete removal of the cyst and the patient's subsequent full recovery with the resolution of symptoms. This case highlights the intricate nature of intracranial dermoid cysts and underscores the critical importance of prompt recognition in effectively mitigating potential complications.

Intralobar Pulmonary Sequestration: A Case Report.

Pulmonary sequestration is a congenital lung malformation characterized by a mass of nonfunctioning lung tissue that receives its arterial supply from an aberrant systemic artery. If symptomatic, most newborns present with respiratory distress. Recurrent infection is the most common presentation after the neonatal period. It is often diagnosed prenatally and is treated with elective surgical resection between ages six and twelve months. We present a case of an infant diagnosed with congenital pulmonary airway malformation prenatally revealed to be pulmonary sequestration at the age of six months, emphasizing the need for appropriate postnatal imaging.

Unusual Presentation of a Congenital Ranula Cyst in a Newborn.

Congenital ranula cysts are rare, benign cysts that arise from the obstruction or rupture of the sublingual gland ducts in the oral cavity of newborns. Here, we present a case of a congenital ranula cyst in a newborn, highlighting the clinical presentation, diagnosis, and management of this rare condition. The neonate presented with a smooth, painless, and non-tender mass in the floor of the mouth, which was identified as a sublingual cyst via ultrasonography. The neonate underwent successful surgical excision of the cyst, with no complications or recurrence observed during the follow-up period. Congenital ranula cysts are a rare but treatable condition that can present in the oral cavity of newborns, and early diagnosis and surgical excision are crucial to prevent complications and ensure optimal outcomes. Healthcare providers should consider congenital ranula cysts as a differential diagnosis for any newborn presenting with a mass in the oral cavity.

Presenting a Case of an Extralobar and Extrapleural Pulmonary Sequestration in a Four-Month-Old Infant.

Pulmonary sequestration is a congenital disease formed by embryogenic separation of the lung parenchyma, halting development and function. It has an aberrant blood supply and can provide a nidus for infection and respiratory compromise. It can be diagnosed prenatally with surgical resection after delivery reserved as the best mode of treatment. In literature, six to twelve months is the most optimal time for elective surgical repair giving time for some maturation to withstand single lung ventilation and operation before the risk of infection heightens after 12 months. We present a case of an infant that had an elective repair at four months of age with no postoperative sequelae highlighting that surgeons can perform elective repair sooner than six months of age and that surgical decision-making should be on a case-by-case basis.

Pulmonary sclerosing pneumocytoma-a case series.

Pulmonary sclerosing pneumocytoma (PSP) is a rare and interesting pulmonary tumour that presents a diagnostic challenge to the surgeon due to its bland symptomatology, indolent course, non-specific radiological features and ambiguous histopathological findings, which could lead to errors in management and prognostication. Herein, we present a series of 4 patients with PSP who were managed in our institution over a period of 8 years and briefly discuss relevant literature.

The Lugones Procedure for Surgical Repair of Scimitar Syndrome: Preserved Growth in a Young Infant.

Surgical repair of Scimitar syndrome is challenging, especially in small patients. Our images demonstrate that the pericardial tunnel technique is feasible even in low-weight patient and that it provides a good growth potential.

Robotic lobectomy in children with severe bronchiectasis: A worthwhile new technology.

BACKGROUND/PURPOSE: Lobectomy is required in children affected by non-responsive, symptomatic, localized bronchiectasis, but inflammation makes thoracoscopy challenging. We present the first published series of robotic-assisted pulmonary lobectomy in children with bronchiectasis. METHODS: Retrospective analysis of all consecutive patients who underwent pulmonary lobectomy for severe localized bronchiectasis (2014-2019) via thoracoscopic versus robotic lobectomy. Four 5 mm ports were used for thoracoscopy; a four-arm approach was used for robotic surgery (Da Vinci Surgical Xi System, Intuitive Surgical, California). RESULTS: Eighteen children were operated (robotic resection, n = 7; thoracoscopy, n = 11) with infected congenital pulmonary malformation, primary ciliary dyskinesia, and post-viral infection. There were no conversions to open surgery with robotic surgery, but five with thoracoscopy. Total operative time was significantly longer with robotic versus thoracoscopic surgery (mean 247 ± 50 versus 152 ± 57 min, p = 0.008). There were no significant differences in perioperative complications, length of thoracic drainage, or total length of stay (mean 7 ± 2 versus 8 ± 3 days, respectively). No blood transfusions were required. Two thoracoscopic patients had a type-3 postoperative complication. CONCLUSIONS: Pediatric robotic lung lobectomy is feasible and safe, with excellent visualization and bi-manual hand-wrist dissection - useful properties in difficult cases of infectious pathologies. However, instrumentation dimensions limit use in smaller thoraxes.

Evaluation of a Lung Nodule in the Setting of Metastatic Gastrointestinal Stromal Tumor: Surgical Considerations in Carney's Triad.

In 1977, Carney et al. first described an association of gastric epithelioid leiomyosarcoma or gastrointestinal stromal tumor (GIST), pulmonary chondroma, and extra-adrenal paraganglioma. This previously unrecognized disorder came to be known as Carney's triad. We describe a case of a 27-year-old female with metastatic GIST, diagnosed with Carney's triad following pulmonary wedge resection, and highlight the surgical implication of this rare disease association.

Arteriovenous Malformations: Congenital or Acquired Lesions?

OBJECTIVE: Arteriovenous malformations (AVMs) were believed to be congenital. However, an increasing number of de novo AVM cases have questioned this doctrine. METHODS: A consensus meeting of international experts attempted to establish a consensus on the nature of these relatively rare but challenging vascular lesions. In addition, an extensive search of the subject was performed using the PubMed medical database. RESULTS: All participants agreed that genetic factors may play a role in the pathogenesis of AVMs. All but 1 participant believed that an underlying genetic predisposition may be detected later on in a patient's life, whereas genetic variations may contribute to sporadic AVM formation. The presence of genetic variations alone may not be enough for an AVM formation. A second hit is probably required. This consensus opinion is also supported by our literature search. CONCLUSIONS: We discuss the literature on the genetics of AVMs and compare it with the consensus meeting outcomes. The congenital or noncongenital character of intracranial AVMs has an impact on the understanding their biological behavior, as well as their efficient short-term and long-term management.

Congenital Pulmonary Airway Malformation - 19-Year Experience from a Tertiary Care Center in India.

Background: Congenital pulmonary airway malformation (CPAM) is a collection of non-hereditary, developmental anomalies. Our aim was to analyze the histological profiles and prevalence of CPAMs diagnosed in our center. Methods: A retrospective study of all CPAMs diagnosed from January 1999 to May 2018 from a general hospital pathology service was performed. Results: There were 79 cystic lesions encountered in fetuses, neonates, and children, 15 of which were CPAMs {5/2372 (0.21%) autopsies and 10/216026 (0.0046%) surgical resections}. The male:female ratio was 1:1.14. Gestational age of antenatal cases ranged from 22 to 32 weeks, postnatal ages ranged from 7 days to 15 years (mean 2.9 years). The cases were right-sided (8/15;53.3%), left-sided (4/15;26.7%) and bilateral (3/15,20%). Seven (46.7%), 4 (26.7%),3 (20%) and 1 (6.7%) were types 1, 2, 3 and 4, respectively. None of the surgical cases had postoperative mortality or morbidity. Conclusions: Prompt recognition and surgical resectability resulted in normal growth and symptom free survival in our postnatally diagnosed patients. Mortality in antenatally diagnosed fetuses remains high (5/11;45%).

An Unusual Case of Noncatamenial Recurrent Pneumothorax.

In a 28-year-old female, seven recurrent pneumothoraces occurred during a period of 2 years despite multiple thoracic interventions, all on the left thoracic side. Despite profound differential diagnostic analysis, the underlying cause remains unclear. An accumulation of conditions in this patient affecting only the left half of the body is remarkable: sinistral glaucoma as an infant, a sinistral pigmentation disorder, and a sinistral Bochdalek hernia.

Pulmonary Nodular Lymphoid Hyperplasia in a 33-Year-Old Woman.

Pulmonary nodular lymphoid hyperplasia is a reactive lymphoproliferative disease. It is very rare, which means that many aspects of the disease are unknown or have not been proven. Pulmonary nodular lymphoid hyperplasia can be symptomatic or asymptomatic, progressive or not, and solitary or multiple, and a surgical approach is the current treatment of choice. We present a case of pulmonary nodular lymphoid hyperplasia that was visualized as multiple ground glass opacities on a computed tomography (CT) scan, and observed for 1 year because the patient was pregnant. Over this period, the number and extent of the opacities progressed, but no symptoms were reported. A surgical biopsy was done and some remaining lesions regressed on follow-up CT scans, while others progressed, without any appearance of symptoms.

Pulmonary Nodular Lymphoid Hyperplasia in a 33-Year-Old Woman

Pulmonary nodular lymphoid hyperplasia is a reactive lymphoproliferative disease. It is very rare, which means that many aspects of the disease are unknown or have not been proven. Pulmonary nodular lymphoid hyperplasia can be symptomatic or asymptomatic, progressive or not, and solitary or multiple, and a surgical approach is the current treatment of choice. We present a case of pulmonary nodular lymphoid hyperplasia that was visualized as multiple ground glass opacities on a computed tomography (CT) scan, and observed for 1 year because the patient was pregnant. Over this period, the number and extent of the opacities progressed, but no symptoms were reported. A surgical biopsy was done and some remaining lesions regressed on follow-up CT scans, while others progressed, without any appearance of symptoms.

Flexible Laryngoscopy in Management of Congenital Stridor.

The incidence of congenital stridor is on rise due to improved neonate and infant survival rate. The gold standard investigation for evaluation of stridor is rigid laryngotracheobronchoscopy, but this is invasive and requires general anesthesia. Flexible fibreoptic laryngoscopy, a relatively simple and less invasive procedure can be done under topical anaesthesia for evaluation of stridor. In this study, we have presented our experience of flexible laryngoscopy in children with congenital stridor, their results and management. Laryngomalacia was the commonest cause of stridor accounting for 80% of cases. 15% (6) patients required tracheostomy for relieving airway obstruction and 22.5% (9) patients required a definitive surgical procedure for correcting the cause. Flexible laryngoscopy is safe and gives a comprehensive analysis of airway including its dynamic functions. We also recommend flexible laryngoscopy as a frontline investigation for evaluation of stridor.

A bronchogenic cyst of the larynx: A case report.

A 12-year-old female presented with a 2-month history of dyspnea and a progressively worsening hoarseness lasting >10 years. The subsequently identified mass was completely resected from the patient's supraglottic area using a laser and suspension laryngoscope. The mass was diagnosed as a bronchogenic cyst using histopathology. While the prognosis for these cysts is good, long-term follow-up remains necessary for such patients because there are several reports in the literature indicating the potential for malignancy to develop. The patient in the present study experienced no complications or recurrence of the bronchogenic cyst 2 years following surgery. To the best of our knowledge, only 2 cases of bronchogenic cysts arising from the larynx have been reported in China, and the present study introduces the first case of a bronchogenic cyst of the larynx in a Chinese patient reported in English.

It's Rare So Be Aware: Pleuropulmonary Blastoma Mimicking Congenital Pulmonary Airway Malformation.

Pleuropulmonary blastoma (PPB) is a rare aggressive malignant tumor of infancy and early childhood. The tumor arises in the lung and pleura and is regarded as a pulmonary dysontogenetic or embryonic neoplasm. Four types are defined in literature. Type I PPB is a rare, cystic lung neoplasm in infants characterized by subtle malignant changes and a good prognosis. Recurrences after type I PPB are usually advanced with a poor prognosis. We report this case to increase awareness about this entity so that the pediatricians, pediatric surgeons, radiologist, and pathologist recognize it early.

Unilateral hypertransparency on chest radiograph: the congenital Poland Syndrome.

Unilateral hypertransparent hemithorax requires a particular diagnostic approach as it can be the result of diverse pulmonary diseases, including pneumothorax, large pulmonary embolus, unilateral large bullae, mucous plag, airway obstruction and contralateral pleural effusion. Congenital syndromes with chest wall abnormalities, are rare, but often underdiagnosed causes. Poland Syndrome consists of such a rare, congenital anomaly and is characterized by the absence of the pectoralis major muscle and upper limb ipsilateral abnormalities. We present a case of a patient with acute exacerbation of chronic obstructive pulmonary disease (COPD) and a unilateral hypertransparency on chest radiology, attributed to the underlying Poland Syndrome.