Hump on head: Rare case of bi-frontal fibrous dysplasia.

Background: FD is relatively rare in the craniofacial region, accounting for only 20% of all cases. Currently, two general subtypes of FD are recognized: monostotic and polyostotic. The monostotic form is more frequent, accounting for 75% to 80% of fibrous dysplasia cases. Case Description: An 18-year-old male presented with the complaint of bony-hard swelling over the forehead for 8 years. Radiology showed an expansile osseous lesion involving frontal bones. The patient underwent bi-frontal craniectomy with gross total resection of tumour mass with titanium mesh cranioplasty. His postoperative period was uneventful and was discharged on the seventh postoperative day. Conclusion: The cases of monostotic skull fibrous dysplasia should be treated by resection of the affected bone and cranioplasty. However, a more conservative re-contouring may be carried out in cases with multifocal involvement or when the excision is considered risky due to proximity to the major venous sinuses.

A Case of Accidentally Found Gastrointestinal Stromal Tumor in a 57-Year-Old Candidate of Gastric Bypass Surgery.

INTRODUCTION: GIST tumors are the most common mesenchymal tumors in the alimentary tract but are rather rare compared to other GI tract tumors and are usually found incidentally. Studies about cases of GIST tumor in bariatric surgery are scarce and there is no specific guideline for treatment. Most of previous cases were managed by sleeve gastrectomy due to their location which were usually in the body and greater curvature. METHODS: This patient had a GIST on lesser curvature and therefore underwent laparoscopic Roux-en-Y bypass. The follow-up was done with imaging studies and CT scan since the location of the tumor was no longer accessible to endoscopy after the gastric bypass. RESULTS: The post-operative course was uneventful and after 2 years the patient is still in good health and had no other complications. The patient has been followed up by imaging studies. DISCUSSION: There are pros and cons to this compared with sleeve gastrectomy. The most important advantage for this approach is that we have the stomach preserved; thus, our operation has the reversibility potential in case of any complication which requires revision operations. However, on the flip side, the limitation of such intervention is that we are not able to use the EGD to monitor and follow up our patient. Based on the relevant literature, for following up and monitoring the postoperative condition of these patients with relatively small GISTs, imaging studies specially abdominal computed tomography scan (CT scan) would be highly beneficial. It is also worth of mentioning that performing a EGD before a bariatric surgery, which is well noted in IFSO position statements and is well explained and discussed in other literature, had a major role in diagnosing this patient's GIST tumor and is proved to be beneficial and essential, once again.

X-linked agammaglobulinemia: a survey of 33 Iranian patients.

In order to determine the clinical and laboratory features of X-linked agammaglobulinemia, the records of 33 male patients with XLA were reviewed during 22 years (1980-2002) in the Iranian referral center of primary immunodeficiency disorders. The patients' ages ranged from 20 to 360 months (median 113 months). The median age at the onset of the disease was 8 months and the median age of diagnosis was 48 months, with a median diagnosis delay of 33 months. Almost all of the patients presented common infectious diseases, which were: pneumonia, otitis, diarrhea, sinusitis, and arthritis. During the course of illness, infections in the respiratory tract, gastrointestinal tract, central nervous system, and musculoskeletal system were seen in 93.9%, 75.8%, 33.3%, and 21.2% of XLA patients, respectively. The most common complications of these patients were chronic infections in 75.8% of them, including: chronic otitis media, chronic sinusitis, chronic diarrhea, and bronchiectasis.