Splenic cysts are relatively uncommon entities in surgical practice and treatment options vary. We present a case of a young adult woman who presented with a left-sided abdominal mass. A large splenic cyst was diagnosed by abdominal ultrasound and computed tomography. Laparoscopic partial excision with marsupialisation was performed with uneventful recovery and minimal blood loss. Histopathology revealed an epidermoid cyst of the spleen. This report describes the case, and includes a short review of the literature. Laparoscopic partial excision with marsupialisation is a safe and appropriate method of treatment for large splenic cysts.
Giant pheochromocytomas are rare silent entities that do not present with the classical symptoms commonly seen in catecholamine-secreting tumors. In many cases they are accidentally discovered. The algorithm to diagnose a pheochromocytoma consists of biochemical evaluation and imaging of a retroperitoneal mass. The female patient in this case report presented with a palpable abdominal mass and was cured with surgical resection. She suffered no recurrence or complications on follow-up. The left retroperitoneal mass measured 27 × 18 × 12 cm and weighed 3,315 grams. Biochemical, radiological, and pathological examinations confirmed the diagnosis of a pheochromocytoma. In this paper, we report on our experience treating this patient and provide a summary of all giant pheochromocytomas greater than 10 cm reported to date in English language medical journals. Our patient's giant cystic pheochromocytoma was the fourth heaviest and fifth largest maximal diameter identified using our literature search criteria. Additionally, this tumor had the largest maximal diameter of all histologically confirmed benign/low metastatic risk pheochromocytomas. Giant cystic pheochromocytomas are rare entities requiring clinical suspicion coupled with strategic diagnostic evaluation to confirm the diagnosis.
The study suggests that if stringent measures are taken to shorten the operating time, LC can be safely and effectively performed in SCD patients with minimal perioperative complications.
The Klippel-Trénaunay syndrome is an unusual syndrome of vascular and dermatologic manifestation in which patients demonstrate hemihypertrophy of the soft tissue and bones of one limb, cutaneous haemangiomas and varicosities in anatomically abnormal positions. Described in 1900 by two French physicians, the etiology remained unclear until recently, when evidence emerged that there was a genetic basis for this sporadic disorder. Genes that encoded pathological angiogenic factors and caused vascular dysmorphogenesis, explaining the molecular bases of this syndrome, were identified. Several angiogenic genes were identified but one gene, the
AGGF1
(formerly
VG5Q
) gene, was seen in mutations involving patients diagnosed with Klippel-Trénaunay syndrome. Furthermore, this syndrome was also noted to have overlapping clinical features linked with the “overgrowth syndromes,” in which genetic mutations along somatic lines were identified. These involved The PI3K enzyme which forms part of the phosphoinositide 3–kinase pathway which is encoded by the PIK3CA-gene. This enzyme mediates embryonic cellular growth in-utero and diseases involved in this pathway are classified as members of the PIK3CA-related overgrowth syndrome. This paper reviews the status of what is now known about the molecular genetics of this unusual, but clinically challenging disorder and its differentiation from similar diseases, linked with the PIK3CA-gene and the related overgrowth syndromes.
We report a case of a 6-year-old girl suffering from morbid obesity, Blount;s disease, and significant social and functional impairment who underwent a laparoscopic sleeve gastrectomy. One year later, she has shown remarkable improvement in all aspects of her health emphasizing the success of the procedure. A follow-up laparoscopic Roux-en-Y gastric bypass or biliopancreatic diversion (BPD) are options if she regains weight as she gets older. This case is noteworthy for several reasons. The age of the patient is younger than any currently on record who has had this treatment. Additionally, the utilization of a sleeve gastrectomy as a first-step procedure, to be followed by Roux-en-Y gastric bypass or BPD, remains a novel treatment for morbid obesity in a pediatric population.
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