A massive abdominal wall desmoid tumor occurring in a laparotomy scar: a case report.

INTRODUCTION: Desmoid tumors are benign but locally aggressive tumors of mesenchymal origin which are poorly circumscribed, infiltrate the surrounding tissue, lack a true capsule and are composed of abundant collagen. History of trauma to the site of tumor origin is elicited in up to 1 in 4 cases and they most commonly develop in the anterior abdominal wall and shoulder girdle but they can arise in any skeletal muscle. The clinical behavior and natural history of desmoid tumors are unpredictable and management is difficult with many issues remaining controversial, mainly regarding early detection, the role, type and timing of surgery and the value of non-operative therapies. CASE PRESENTATION: We report a case of a 23 year old male referred from a district hospital to a national referral hospital in Kenya, after developing a huge abdominal wall desmoid tumor following laparotomy for a blunt abdominal injury fourteen months earlier. The tumor was successfully excised and the abdominal wall defect reconstructed using a vicryl/prolene mesh and a unilateral groin flap. The patient had a non-eventful recovery and was discharged through radiotherapy clinic. CONCLUSION: Wide margin tumor excision alone is a reasonable option in the management of desmoid tumors.

Persistant pre-eclampsia post partum with elevated liver enzymes and hemolytic uremic syndrome.

The spectrum of complications with pre-eclampsia, which may include AFLP (acute fatty liver of pregnancy) as well as the HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets), is resolved by early delivery. However, the ravages of HUS/TTP (hemolytic uremic syndrome/thrombotic thrombocytopenic purpura) require therapy usually by plasma exchange. Overlap between these two groups of syndromes has occurred on rare occasions and usually requires the therapy of the predominant or more dangerous or threatening form. Such overlap can be appreciated and then treated successfully without residual morbidity. The index case is presented and an extensive review of the two groups of syndromes is provided.

Predictable recovery from myasthenia gravis crisis with plasma exchange: thirty-six cases and review of current management.

Adult, acquired, idiopathic, autoimmune myasthenia gravis has a well-characterized IgG anti-acetylcholine striated-muscle receptor antibody. Removal by plasma exchange is effective, established therapy to augment anti-cholinesterase and immunosuppressive therapy and is the treatment of choice for myasthenia gravis crisis. We report 36 consecutive patients referred and accepted for plasma exchange, 32 of whom were in or entering myasthenia crisis, over a 10 year period. An average of 7.8 (range 1 to 16) plasma exchange procedures were done, with uniform, significant improvement, including extubation of 13 in myasthenic crisis and discharge from hospital in all. We conclude that this is the best treatment for myasthenia gravis crisis in hospital. From recent cases, most, if not all, crises can be prevented by IVIgG or plasma exchange as out-patients with use of corticosteroid and or azathioprine.

Swelling of collagen intracanalicular implants.

BACKGROUND: Absorbable intracanalicular collagen implants are used to attempt to determine if permanent closure of the tear drainage system will diminish or alleviate the signs and symptoms of dryness. The rate of swelling of the collagen implants was measured in vitro in this study. METHODS: Collagen implants were submersed in saline, Refresh Plus artificial tears or Tobrex antibiotic solution on a hanging drop microscope slide. The diameter of the collagen rod was measured over time using videotaping and image processing. RESULTS: The 0.3- and 0.4-mm diameter collagen implants swelled approximately 55% to 65% in Unisol 4 Preservative Free saline and Refresh Plus artificial tear solution, with the majority of the swelling occurring over the first 30 minutes. The 0.4-mm diameter implants took slightly longer to reach the fully swollen state than did the 0.3-mm implants. They swelled somewhat less in Tobrex Ophthalmic Solution. CONCLUSIONS: Collagen implants swell approximately 60%, with the majority of the swelling occurring in the first 30 minutes. Thus, it will take approximately 30 minutes after insertion of the implants for the full occlusive effect to occur.

Characteristics of 73 patients, 1984-1993, treated by plasma exchange for Guillain-Barre syndrome.

Acute Guillain-Barre syndrome (GBS) is a demyelinating polyneuropathy which responds readily to plasma exchange (PEX). According to the North American Acute GBS PEX study there is a 50% or more reduction in the recovery time if PEX is initiated early in the course of the disease. Demyelinating antibodies are usually IgM. IgA antibodies require prolonged PEX. Patients with predominant IgG antibodies have chronic inflammatory demyelinating polyneuropathy (CIDP), which requires an even longer course of PEX, over weeks to months or years. We reviewed records of 73 patients with the initial diagnosis of GBS treated with PEX. Among these patients, 55 had classic GBS, three had the Miller-Fisher variant, two had CIDP, and 13 had demyelinating-like polyneuropathies associated with other conditions including malignancy, vaccine-related myelitis, steroid-induced myopathy, polymyositis, botulism, gram-negative sepsis, Sjogren's, and AIDS. Hughes grading system was used. Patients were graded 3 to 5, with grade 3 patients being unable to walk 5 m without support, grade 4 patients being bed or chair bound, and grade 5 patients being ventilator dependent. Of 60 unassociated (GBS) demyelinating cases receiving a mean of 6.5 PEX procedures, 13 (21%) were intubated early in the treatment, with four (6%) remaining ventilator dependent post-PEX. Of 51 non-intubated patients, 15 became ambulatory post-PEX. Patients with the Miller-Fisher variant showed improvement within 6 hours of PEX initiation. We did not investigate correlation of GBS with infection; however, we did observe a rise in CMV titer among 15% of the 58 patients with acute GBS. Considering our results we believe that intensive PEX on a daily basis for a few days is necessary for severely affected individuals. We advise five to nine procedures at consultation unless early, rapid recovery occurs.