Melena as an unusual presentation of gastrointestinal stromal tumour, a case report.

INTRODUCTION: Gastrointestinal Stromal Tumors (GISTs) are a rare slow growing malignancy, accounting for less than 1% of all gastrointestinal (GI) tract tumors. These tumors are usually discovered incidentally by endoscopy, surgery or radiology. However on occasions they may present with significant symptoms including GI blood loss. This case report discusses an atypical presentation of a GIST in a 57-year-old female. CASE PRESENTATION: A 57-year-old woman presented to the emergency department following one episode of melena. This occurred on a background of two previous presentations with melena over a 10-year period. She had a preceding surgery for a Meckel's Diverticulum. She was admitted for monitoring and investigation. An emergency upper endoscopy showed no upper gastrointestinal pathology to account for the bleeding. Her condition deteriorated with development of hypovolemic shock, requiring blood transfusion. An urgent CT angiogram identified a large mass in the distal ileum. The patient underwent an emergency laparotomy, where a 9.1 cm tumor located on the distal one-third of the ileum was resected. Histopathology confirmed the mass was a GIST. The patient had a successful post-operative period and subsequent treatment with Imatinib. DISCUSSION: The majority of GISTs are found incidentally. This case report describes an unusual presentation of a GIST in which the tumor bled into the intestinal lumen causing significant melena and life threatening hemorrhage. CONCLUSION: We conclude that GIST should be considered as a possible differential in rare cases of GI bleeding where more common causes have been ruled out.

Entero-enteric fistula from the stump of an end-to-side ileocolic anastomosis mimicking cancer recurrence.

Enteric fistulae are a complex and technically frustrating complication of any bowel surgery. The constellation of associated non-specific symptoms often leads to extensive investigation and, in this case, suspicion of disease recurrence. A 71-year-old gentleman with a history of previous colorectal cancer presented with chronic diarrhoea, weight loss and left lower quadrant pain. Elective exploratory laparoscopy was performed to investigate possible disease recurrence due to elevated carcinoembryonic antigen levels and a positron emission tomography positive area within the mesentery. A jejunal-ileal fistula was found at laparotomy where the blind ileal stump of the end-to-side ileocolic anastomosis had fistulated into the jejunum. Resection of the affected jejunum was performed with end-to-end jejuno-jejunal re-anastomosis and stapling of the ileal stump. Specimen histology was negative for recurrence. Intestinal fistulae represent a diagnostic challenge. This is the first case report describing an enteric fistula mimicking cancer recurrence.

Retroileal colorectal anastomosis: an old technique, still relevant.

Retroileal colorectal anastomosis is an infrequently used technique providing tension-free anastomosis after extended left-sided colonic resection, which was first described by Turnbull in 1972. The authors describe the use of a retroileal colorectal anastomosis in a patient with chronic Candida albicans and Enterococcal retroperitoneal abscess. The patient's condition was most likely secondary to an anastomotic leak and colonic fistula post en bloc resection for recurrence of a left renal cell carcinoma. After failure of antimicrobial therapy and percutaneous drainage, surgical intervention was required. This involved resection of the existing colocolonic anastomosis and drainage of the retroperitoneal abscess. A retroileal colorectal anastomosis was then necessary to ensure a tension-free anastomosis. To the best of our knowledge, this is the first reported case of retroileal anastomosis used for this purpose. It highlights the importance of this technique in the surgeon's armamentarium.

Adhesions after laparoscopic and open ileal pouch-anal anastomosis surgery for ulcerative colitis.

BACKGROUND: Emerging evidence suggests that a laparoscopic approach to colorectal procedures generates fewer adhesions. Even though laparoscopic ileal pouch-anal anastomosis (IPAA) is a lengthy procedure, the prospect of fewer adhesions may justify this approach. The aim of this study was to assess abdominal and adnexal adhesion formation following laparoscopic versus open IPAA in patients with ulcerative colitis. METHODS: A diagnostic laparoscopy was performed at time of ileostomy closure. All abdominal quadrants and the pelvis were video recorded systematically and graded offline. The incisional adhesion score (IAS; range 0-6) and total abdominal adhesion score (TAS; range 0-10) were calculated, based on the grade and extent of adhesions. Adnexal adhesions were classified by the American Fertility Society (AFS) adhesion score. RESULTS: A total of 43 patients consented to participate, of whom 40 could be included in the study (laparoscopic 28, open 12). Median age was 38 (range 20-61) years. There was no difference in age, sex, body mass index, American Society of Anesthesiologists grade and time to ileostomy closure between groups. The IAS was significantly lower after laparoscopic IPAA than following an open procedure: median (range) 0 (0-5) versus 4 (2-6) respectively (P = 0·004). The TAS was also significantly lower in the laparoscopic group: 2 (0-6) versus 8 (2-10) (P = 0·002). Applying the AFS score, women undergoing laparoscopic IPAA had a significantly lower mean(s.d.) prognostic classification score than those in the open group: 5·2(3·7) versus 20·0(5·6) (P = 0·023). CONCLUSION: Laparoscopic IPAA was associated with significantly fewer incisional, abdominal and adnexal adhesions in comparison with open IPAA.

Haematoma in a hydrocele of the canal of Nuck mimicking a Richter's hernia.

We report a haematoma in a hydrocele of the canal of Nuck in a 69-year-old female. She presented with a right-sided groin swelling, the differential for which included an irreducible inguinal hernia or haematoma given her aspirin and clopidegrel use. Successful treatment involved evacuation of the haematoma with excision of the sac. Despite a high index of suspicion for a haematoma, these swellings should ideally be explored given the potential for co-existence of a hernia.

Ruptured abdominal aortic aneurysm in a 12-month-old boy.

A rare case of abdominal aortic aneurysm in a 12-month-old boy is reported. The clinical presentation was one of acute catastrophic rupture. The morphologic and histologic findings suggested an idiopathic etiology.

A case of Fournier gangrene complicating idiopathic nephrotic syndrome of childhood.

A 10-year-old boy presenting with steroid resistant nephrotic syndrome developed Fournier gangrene of the scrotum. Antimicrobial drug therapy, intravenous albumin, excision of necrotic scrotum and left orchidectomy followed by skin grafting 3 weeks later led to an excellent cosmetic and medical result. Six months later he remains nephrotic on diuretic and angiotensin converting enzyme inhibitor medication.

Giant pharyngeal gland nuclei of Ascaris: unusual cytology suggests a novel pathway for transport of ribosomal proteins to the nucleus.

We describe the unusual giant pharyngeal gland nuclei of the parasitic roundworm Ascaris lumbricoides suum and attempt to reconcile the relationships among its uncommon, and in some instances unique, collection of constituents. The nuclei were studied by light and electron microscopy, Feulgen cytophotometry, nuclear size analysis and histochemical methods, including those for detection of DNA, RNA, acidic and basic proteins, lipids and carbohydrates. A highly active nucleus is revealed: an extensive system of intranuclear annulate lamellae (IAL); membrane-bounded, acidophilic bodies which contain non-basic proteins; an abundance of free nucleoli; and an exceedingly large chromocenter containing a core of DNA surrounded by nucleoli (an apparent nucleolar organizing region, NOR). The relationships among the various nuclear constituents suggest that the acidophilic bodies consist of nucleolus-related proteins imported from the cytoplasm to the NOR, and that the annulate lamellae mediate this transport. Incidental findings include rounded cytoplasmic invaginations into the nucleus, thread-like structures with dense cores and surrounding small granules found among concentrations of nucleoli, and bundles of 12 nm filaments closely associated with these nucleoli. The significance of such huge, morphologically complex and highly polyploid nuclei, derived from chromatin-diminished progenitor cells early in development, and the possible interrelationship of these phenomena, remain obscure.

Mirror image duplication of the hands and feet: report of a sporadic case with multiple congenital anomalies.

Mirror image duplication of the hands and feet is a rare entity. Based on 3 previous reports, findings include nasal abnormalities, dimelia of ulna and fibula, tibial hypoplasia and mirror image duplication of hands and feet. We report on a sporadic case in which mirror image duplication was associated with multiple congenital anomalies. Although these cases may represent variable expression of the same dominantly transmitted complex polysyndactyly syndrome, it is possible that mirror image duplication of the hands and feet is a manifestation common to a number of distinct clinical entities. During limb bud development, duplication and aberrant positioning of the zone of polarizing activity in relation to the apical ectodermal ridge may account for the anatomic abnormalities of the hands and feet in these patients.

Radiographic imaging studies in pediatric chronic sinusitis.

BACKGROUND: The diagnosis of chronic sinusitis is dependent on the radiographic evidence of sinus disease. METHODS: We evaluated the performance of radiographs and computed tomographic (CT) scans for the examination of the paranasal sinuses of 91 patients of both sexes, ranging in age from 2 to 17 years, who had chronic upper respiratory tract symptoms for at least 3 months. The CT scan findings were categorized as no disease; minimal disease, and mild, moderate, and severe sinusitis. RESULTS: Fifty-eight patients (63%) had chronic sinusitis: CT scan abnormalities were minimal in 17%, mild in 19%, moderate in 21%, and severe in 43%. There was a statistically significant correlation between rhinorrhea (r = 0.25, p = 0.01), cough (r = 0.27, p = 0.009), and the severity of sinus abnormality as determined by CT scan. Clinical presentation in the mild, moderate, and severe sinusitis groups (p < 0.05) was significantly different from that of the no disease group, whereas the minimal disease group had subclinical presentation (p = 0.11). Clinically significant chronic sinusitis often occurred at multiple sites: 44% of patients had pansinusitis, 50% had disease involvement of at least two sinuses, and 6% had disease in a single sinus. When sinus radiographs were compared with CT scans (n = 70 cases), radiographs could not identify minimal disease. For clinically significant sinusitis, sinus radiographs detected disease in 1 of 5 (20%) frontal sinuses, 0 of 12 (0%) sphenoidal sinuses, and 17 of 31 (54%) ethmoidal sinuses. With the minimal criteria of 40% to 50% opacification or fluid level filling of the maxillary antrum, radiographs detected disease in 37 of 49 (75%) cases. The sensitivity and specificity for a Waters view to confirm clinically significant chronic sinusitis without specifying the sites and severity were acceptable at 76% and 81%, respectively. When limited sinus CT scans were compared with full CT evaluation (n = 49 cases), limited studies detected 5 of 5 (100%) frontal, 9 of 11 (82%) sphenoidal, 14 of 19 (73%) ethmoidal, and 39 of 40 (97%) cases of maxillary sinusitis. The overall agreement was 88%. CONCLUSIONS: A single Waters view is an acceptable part of the initial evaluation of pediatric chronic sinusitis; however, a limited CT scan is a better alternative.

Microcephalic osteodysplastic dysplasia.

We present two patients with a distinct facial phenotype, short stature, brachydactyly, clubfoot deformities, cataracts, microcephaly, and normal intelligence. Similar radiographic abnormalities of the spine, long bones, hands, and feet were noted. These patients are similar to 2 males previously described by Saul and Wilson [1990: Am J Med Genet 35:388-393]. These 4 patients appear to have a unique skeletal dysplasia characterized by microcephaly, distinct facial phenotype, multisystem abnormalities, and short stature of postnatal onset.

Trichothiodystrophy and associated anomalies: a variant of SIBIDS or new symptom complex?

Trichothiodystrophy is characterized by sparse, short, sulfur-deficient hair. Numerous symptom complexes have been described in which the hair abnormality represents a constant feature. We report a boy with trichothiodystrophy, ichthyotic skin changes, onychodystrophy, chronic neutropenia, osteosclerosis, hypothyroidism, nystagmus, growth and mental retardation, and microcephaly, who developed a progressive encephalopathy with ataxia and optic atrophy at 2.5 years of age. In addition to a deficient cystine level identified on a hair sample, a disturbance in the composition of other amino acids was present. Although features were reminiscent of osteosclerosis, ichthyosis, brittle hair due to trichothiodystrophy, impaired intelligence, decreased fertility, and short stature (SIBIDS) and could represent a variant of this disorder, findings in our patient may reflect a new trichothiodystrophy symptom complex that carries a poor prognosis for survival beyond childhood.