A 58-Year-Old Woman with Acute Torsion of the Small Bowel Due to Diffuse Intestinal Lipomatosis.

BACKGROUND Diffuse intestinal lipomatosis is a rare condition that infiltrates mature fatty tissue into the intestinal submucosa and subserosa of the small or large intestine and can present with intestinal obstruction or torsion. This report is of the case of a 58-year-old woman who had acute torsion of the small bowel due to diffuse small intestinal lipomatosis. CASE REPORT A 58-year-old woman, who was otherwise in good health, arrived at our Emergency Department experiencing sudden, intense pain in the lower abdomen. She also reported abdominal swelling, feelings of nausea, vomiting, and reduced ability to defecate for at least 2 days. The next morning, contrast-enhanced abdominal computed tomography (CT) scan was performed, showing diffuse thickening of the small intestinal wall with hypodensity, fatty density, lumen narrowing, and wall thinning. The small intestine demonstrated a whirlpool-like distribution in the lower right abdomen and localized thickening of the small intestinal wall, suggesting acute intestinal torsion. An hour later, an emergency operation was performed to remove part of the small intestine. Three days later, pathological results showed a thin intestinal wall, expansion of the mucosal layer and submucosa, and hyperplasia of adipose tissue. CONCLUSIONS This report presents a rare case of torsion and small bowel obstruction caused by diffuse intestinal lipomatosis and focuses on the abdominal enhanced CT scan, which showed diffuse thickening of the small intestine, with multiple areas of fat density and torsion of the small intestine in the right lower abdomen. Histopathology is also presented, with the result showing intestinal lipomatosis.

Clinical characteristics and surgical management of facial infiltrating lipomatosis: a single center experience.

BACKGROUND: Facial infiltrating lipomatosis (FIL) is a rare condition characterized by congenital facial enlargement. Beyond its impact on physical appearance, FIL can also impair essential facial functions such as swallowing, chewing, vision, and breathing, imposing a substantial physiological and psychological burden. Currently, fewer than 80 cases of FIL have been reported, and the characteristics and management strategies for FIL remain unclear. METHODS: We reviewed the clinical, surgical, and radiological records of 39 FIL patients who were treated at our center. Of these, genetic testing was performed for 21 patients. RESULTS: Aberrant overgrowth involves subcutaneous fat, bones, muscles, glands, tongue, lips, and teeth. Epidermal nevi could be observed in the dermatomes innervated by the three branches of the trigeminal nerve, with the highest frequency seen in the dermatome of the mandibular branch. Four patients exhibited concurrent hemimegalencephaly (HMEG), with one case presenting HMEG on the opposite side of the FIL. Nineteen patients were confirmed to harbor the PIK3CA mutation. Thirty-three patients underwent surgical procedures, with a post resection recurrence rate of approximately 25%. CONCLUSIONS: A variety of maxillofacial structures may be involved in FIL. PIK3CA mutations are important pathogenic factors. Emerging targeted therapies could present an additional treatment avenue in the future. However, surgery currently remains the predominant treatment choice for FIL. The timing and modality of surgery should be individually customized, taking into account each patient's unique circumstances. Notably, there is a significant possibility of postoperative recurrence during childhood and adolescence, necessitating early strategic planning of disease management.

Ultrasonographic findings in nevus lipomatosus cutaneous superficialis: What differentiates this tumor from other soft tissue tumors?

Nevus lipomatosus cutaneous superficialis is a rare, benign hamartoma characterized by mature adipocyte proliferation in the dermis. It is frequently difficult to distinguish clinically from soft tissue tumors, including lipoma, neurofibroma, venous malformation, and angiolipoma. Notably, the classical form, which shows multiple and sometimes enlarged nodules, is difficult to differentiate from liposarcoma based on clinical examination, computed tomography, and magnetic resonance imaging findings. Therefore, to ascertain the utility of ultrasonography in diagnosing nevus lipomatosus cutaneous superficialis, sonographic examinations were performed on eight patients with nevus lipomatosus cutaneous superficialis. All patients had ill-defined hyperechoic masses in the dermis or from the dermis to the subcutis, and the posterior echoes were attenuated in seven patients. Color Doppler sonography revealed no blood flow to the lesions. Ultrasound images were created using the reflections of ultrasound waves at interfaces with different acoustic impedances. Therefore, it is assumed that, in nevus lipomatosus cutaneous superficialis, the ultrasound beam is scattered by ectopic mature adipocytes intermingled with collagen bundles, which are shown as hyperechoic masses. Furthermore, the scattering of the ultrasound beam is thought to reduce tissue penetration, which may attenuate the posterior echo.

Trigeminal Lipomatosis: A Rare Cause of Intractable Neuralgia.

Lipomatosis of nerve, earlier known as fibrolipomatous hamartoma is a rare condition which predominantly affects peripheral nerves, cranial nerve involvement being extremely uncommon. Preoperative consideration of this entity is of paramount importance as its inadvertent complete surgical resection may inevitably result in significant neurological deficit. We report a case of trigeminal lipomatosis in a young patient with trigeminal neuralgia.

Correlation of spinal epidural fat volume with body mass index: a longitudinal study.

PURPOSE: Spinal epidural lipomatosis is abnormal accumulation of normal fat in the epidural space with weight loss suggested as first-line therapy in select symptomatic patients. However, moderate to large longitudinal studies establishing concordant changes between body mass index and epidural fat are lacking. The purpose of this study was to longitudinally assess this relationship. METHODS: We performed an ancillary study of the Habitual Diet and Avocado Trial. Baseline and six-month abdominal MRIs were analyzed for 98 overweight or obese but otherwise healthy subjects. Dorsal epidural fat volumes in the lumbar spine were measured and correlated with changes in body mass index, changes in visceral fat volume, and demographic information. RESULTS: There was a linear relationship between body mass index changes and epidural fat volume changes with a one-point change in body mass index corresponding to a 45 mm3 change in dorsal epidural fat volume (p < 0.001, 95% CI 31.87 to 76.77) as well as between visceral fat volume changes and epidural fat volume changes (regression coefficient 0.51, p < 0.001, 95% CI 0.22 to 0.47). Age was inversely related with subjects older than 45.7 years tending to lose epidural fat (regression coefficient -0.22, p = 0.025, 95% CI -10.43 to -0.72). CONCLUSION: Changes in spinal dorsal epidural fat volume parallel changes in body mass index and visceral fat, supporting weight loss as initial treatment for uncomplicated obesity-associated spinal epidural lipomatosis.

Quantitative imaging predicts pancreatic fatty infiltration on routine CT examination.

PURPOSE: The purpose of this study was to assess the performance of quantitative computed tomography (CT) imaging for detecting pancreatic fatty infiltration, using the results of histopathological analysis as reference. MATERIALS AND METHODS: Sixty patients who underwent pancreatic surgery for a pancreatic tumor between 2016 and 2019 were retrospectively included. There were 33 women and 27 men with a mean age of 56 ± 12 (SD) years (age range: 18-79 years). Patients with dilatation of the main pancreatic duct, chronic pancreatitis, or preoperative treatment were excluded to prevent any bias in the radiological-pathological correlation. Pancreatic fatty infiltration was recorded at pathology. Pancreatic surface lobularity, pancreatic attenuation, visceral fat area, and subcutaneous fat area were derived from preoperative CT images. The performance for the prediction of fatty infiltration was assessed using area under receiver operating characteristic curve (AUC) and backward binary logistic regression analysis. Results were validated in a separate cohort of 34 patients (17 women; mean age, 50 ± 14 [SD] years; age range: 18-73). RESULTS: A total of 28/60 (47%) and 17/34 (50%) patients had pancreatic fatty infiltration in the derivation and validation cohorts, respectively. In the derivation cohort, patients with pancreatic fatty infiltration had a significantly higher PSL (P < 0.001) and a lower pancreatic attenuation on both precontrast and portal venous phase images (P = 0.011 and 0.003, respectively), and higher subcutaneous fat area and visceral fat area (P = 0.010 and 0.007, respectively). Multivariable analysis identified pancreatic surface lobularity > 7.6 and pancreatic attenuation on portal venous phase images < 83.5 Hounsfield units as independently associated with fatty infiltration. The combination of these variables resulted in an AUC of 0.85 (95% CI: 0.74-0.95) and 0.83 (95% CI: 0.67-0.99) in the derivation and validation cohorts, respectively. CONCLUSION: CT-based quantitative imaging accurately predicts pancreatic fatty infiltration.

Cervical syringomyelia with caudal thoracic epidural lipomatosis: case report and literature review.

Syringomyelia associated with epidural lipomatosis is a rare finding. Only three published cases of epidural lipomatosis associated with syringomyelia exist in the literature.We report the case of a 46-year-old woman who presented with progressive myelopathy over an 18-month period. Imaging revealed significant thoracic spinal cord compression secondary to epidural lipomatosis from T3 to T8 with cephalad cervical syringomyelia extending from C7 to T1. Imaging was unremarkable for Chiari malformation or a craniospinal space-occupying lesion. A T2 to T8 laminoplasty was performed, removing excessive epidural adipose tissue to decompress the thoracic spinal cord. Postoperatively, the patient reported symptom improvement with complete symptom resolution at 3 months. Follow-up imaging at 3-months demonstrated thoracic spinal cord decompression with mild syrinx reduction. At two-year follow-up the patient remained asymptomatic with unchanged imaging.Syringomyelia in the setting epidural lipomatosis is a rare finding.

Extensive Symptomatic Thoracolumbar Epidural Lipomatosis Treated With Minimally Invasive Hemilaminectomies: Technical Case Report.

BACKGROUND AND IMPORTANCE: Spinal epidural lipomatosis is a rare condition commonly associated with chronic corticosteroid use and obesity that involves deposition of adipose tissue in the epidural space of the spinal canal. This accumulation of adipose tissue may cause compression of the spinal cord and/or nerve roots and result in compressive symptoms such as myelopathy or radiculopathy. Spinal involvement is usually confined to either the thoracic or lumbar spine but can infrequently affect both regions. Depending on pre-existing conditions, treatment options include weight loss and discontinuation of exogenous steroid use, both of which have been shown to be effective therapeutic methods. Surgical decompression may be useful for appropriately selected patients in whom conservative therapy has failed or who experience acute neurological deterioration, although this is rarely indicated. CLINICAL PRESENTATION: In this study, we describe a patient receiving long-term corticosteroid therapy who presented with symptomatic epidural lipomatosis that involved the thoracic and lumbar spine. She was treated with decompression by continuous T3-L5 hemilaminectomies performed through 5 small incisions of alternating laterality. After surgery, the patient experienced clinical improvement and was able to return to her baseline. CONCLUSION: We illustrate a successful spinal decompression of extensive epidural lipomatosis through a less-invasive surgical approach using several small incisions to accomplish uninterrupted hemilaminectomies. This alternative approach to a standard continuous incision can be considered in cases of extensive spinal epidural lipomatosis in patients with multiple medical comorbidities in whom wound healing is believed to be an issue and for whom minimizing blood loss is crucial.