Immune Thrombocytopenic Purpura and Paradoxical Thrombosis: A Systematic Review of Case Reports.

BACKGROUND AND AIMS:  Immune thrombocytopenic purpura (ITP) is an acquired bleeding disorder characterized by autoantibodies against platelets. The clinical presentation is variable; the main symptom is bleeding, and many patients are asymptomatic; others have nonspecific symptoms like fatigue. Uncommonly, ITP can present with paradoxical thrombosis. The risk of thrombosis in ITP may be higher than expected, which makes the management of ITP more challenging. This review aims to evaluate patients with ITP who develop thrombosis and identify potential risk factors related to thrombosis in this category of patients. MATERIALS AND METHODS:  English literature was searched using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines for adults above 18 years with primary ITP who had infarctions or thrombotic events. Patients with secondary ITP were excluded. The search included articles published up to 20th October 2021. RESULTS:  A total of 73 articles were included. Seventy-seven patients with ITP had developed infarctions and various thrombotic events. Sixty-three patients had arterial events, and 14 patients developed venous thrombotic events. CONCLUSION:  Patients with ITP have low platelets, which predispose them to bleed; despite that, serious thrombotic complications can happen in these patients and are difficult to predict. Therefore, it is critical for physicians to understand that ITP is paradoxically a prothrombotic condition and to address preventive thromboembolic measures whenever possible.

New Phenotypic Feature in a Patient With a Rare Triplication of the 22q11.2 Region Presenting With Peters Anomaly, Congenital Heart Disease, and Global Developmental Delay: A Case Report and Literature Review.

The vulnerability of chromosome 22q11.2 region to rearrangement is due to several low copy repeat (LCR) sequences. These rearrangements are involved in syndromes that share similar phenotypic features. The rearrangements of the 22q11.2 chromosomal region are common, specifically, duplications and deletions associated with congenital anomalies and developmental disabilities disorders. However, the features associated with this chromosomal rearrangement remain largely unknown. We present, to the best of our knowledge, the third patient affected by triplication of the 22q11.2 chromosome region, who presents with Peters anomaly, global developmental delay, patent ductus arteriosus, and subaortic stenosis. This case highlights a new phenotypic feature associated with triplication of this genomic region.

Thoracic Spinal Cord Compression Secondary to Metastatic Papillary Thyroid Carcinoma: An Unusual Oncological Phenomenon.

Hematogenous spread is fairly an unusual feature for papillary thyroid carcinoma (PTC) in comparison to follicular thyroid carcinoma (FTC). Thoracic spinal metastasis with complicating cord compression is an even rarer manifestation of PTC that was reported in a limited number of cases in the literature. Herein we present a 65-year-old female with a history of PTC on current radiotherapy, status post attempted surgery due to significant tumor burden and intraoperative bleeding, presented with a one-week history of rapidly progressive bilateral lower extremities weakness. Physical examination revealed paraplegia of both lower extremities with areflexia and a sensory level equivalent to the upper thoracic vertebrae. Urgent imaging depicted destructive epidural lesions at T1-T3 vertebrae with thoracic cord compression. Emergent laminectomy and debulking of these lesions were undertaken. Histopathological examination confirmed metastatic PTC. The patient proceeded to further treatment with radiotherapy following her successful neurological recovery. Thoracic vertebral metastasis is an unusual oncological phenomenon of PTC. Metastatic PTC should be considered in patients with a current or remote history of PTC who present with thoracic cord compression. Our case demonstrates that multidisciplinary management is the key to achieving a better outcome for metastatic PTC with thoracic cord compression.

Primary Hepatic Lymphoma Presenting as an Acute Abdomen in a Young Female Patient: A Case Report and Literature Review.

We report a rare case of primary hepatic lymphoma (PHL) in a hepatitis B virus- (HBV-) infected young female patient who presented with right upper abdominal pain, nausea, and vomiting for a few days. The preoperative diagnosis was difficult due to the rarity of the disease and the presence of a solitary hypodense mass in the left lobe of the liver on contrast-enhanced computed tomography (CT) scan with a normal alpha-fetoprotein (AFP) and negative cytology. She underwent an uneventful extended left hemihepatectomy, and the surgical biopsy revealed a PHL-of diffuse large B-cell lymphoma (DLBCL) type-with negative resection margins. She received adjuvant combination chemotherapy and remained disease-free with normal serial radiology over a 2-year follow-up period.

Reconstructive Surgery for Mycetoma: Is There a Need to Establish an Algorithm?

BACKGROUND: Mycetoma is a chronic, progressive, disfiguring, and destructive disease. It caused by a variety of microorganisms including fungi and higher bacteria. It is primarily an infection of the skin and soft tissue, most frequently affecting the lower extremity and the hand and spread through fascial planes and lymphatics. METHODS: Current medical and surgical management are still inadequate and the recurrence rate is high with severe disabilities. RESULTS: This review describes some reconstructive techniques that were performed to address essential aspects with regard to mycetoma surgical management that include coverage of large skin and soft-tissue defects left after local excisions, enhancing the rate of chronic mycetoma wound healing, and preservation or restoration of functional status of the affected limbs. CONCLUSION: These applied techniques-which had acceptable preliminary outcome-have to be considered by the surgeons dealing with mycetoma to improve the functional and cosmetic outcomes and to minimize tremendous morbidities and disabilities that are associated with this neglected disease.

Congenital heart disease in a cohort of Sudanese patients with cleft defects

Background and objectives:Cleft defects are among the most visible congenital defects worldwide and congenital heart disease (CHD)being one of the most common associated anomalies. This study aimed to assess the prevalence of CHD among a cohort of Sudanese patients with cleft lip and/or palate.Patients and Methods:All patients who presented to Soba University Hospital with cleft lip and/or palate from March 2009 toMarch 2015 were included.Results:Out of 381 patients included; 168 patients (44%) had cleft lip and palate (CLP), 156 (41%) had cleft lip (CL)and isolated cleft palate (CP) was found in 57 patients (15%). Facial dysmorphic features were identified in 57 (15%) and cardiac defects in 42 (11%) patients. Ventricular septal defect (VSD) was diagnosed in 16 patients (38%), and ASD in 12 (30.9%). Other macroscopic anomalies were identified in 57 patients (15%)and were associated with CHD (P<0.001). Significant association was found between the type of cleft andCHD (P<0.002), as cardiac defects were maximally observed among CP cases (21%) followed by CLP cases (13%) then CL cases (5.12%). Significant association was also found between facial dysmorphicoccurrence and CHD (P < 0.001).Conclusion:CHD is a common anomaly in cleft population. The pattern of CHD is consistent with the literature withVSD being the most frequent. The cardiac defects are most prevalent in CP group. Echocardiography isjustified for screening of CHD due to the relatively higher incidence of CHD among clefts patients than ingeneral population

Congenital heart disease in a cohort of Sudanese patients with cleft defects

Background and objectives: Cleft defects are among the most visible congenital defects worldwide and congenital heart disease (CHD) being one of the most common associated anomalies. This study aimed to assess the prevalence of CHD among a cohort of Sudanese patients with cleft lip and/or palate. Patients and Methods: All patients who presented to Soba University Hospital with cleft lip and/or palate from March 2009 to March 2015 were included. Results:Out of 381 patients included; 168 patients (44%) had cleft lip and palate (CLP), 156 (41%) had cleft lip (CL) and isolated cleft palate (CP) was found in 57 patients (15%). Facial dysmorphic features were identified in 57 (15%) and cardiac defects in 42 (11%) patients. Ventricular septal defect (VSD) was diagnosed in 16 patients (38%), and ASD in 12 (30.9%). Other macroscopic anomalies were identified in 57 patients (15%) and were associated with CHD (P<0.001). Significant association was found between the type of cleft and CHD (P<0.002), as cardiac defects were maximally observed among CP cases (21%) followed by CLP cases (13%) then CL cases (5.12%). Significant association was also found between facial dysmorphic occurrence and CHD (P < 0.001). Conclusion: CHD is a common anomaly in cleft population. The pattern of CHD is consistent with the literature with VSD being the most frequent. The cardiac defects are most prevalent in CP group. Echocardiography is justified for screening of CHD due to the relatively higher incidence of CHD among clefts patients than in general population

Surgical treatment of mucocele of the appendix: a systematic review and case report.

INTRODUCTION: Appendicular mucocele is a rare well-described clinico-pathological occurrence. It denotes an obstructive dilatation of the appendicular lumen by mucinous secretions. CASE REPORT: A 60-year-old patient presented with right lower abdominal pain and nausea for 2 years. Abdominal CT scan suggested a diagnosis of a appendicular mucocele. Following informed consent, surgical exploration revealed a cystic mass arising from the body of the appendix with inflamed walls with no evidence of perforation. Simple appendectomy was performed as the caecum and the mesenteric nodes were free of pathological involvement. The final diagnosis of mucinous cystadenoma was confirmed by histopathology. Postoperative course was uneventful. The patient was in good health during a four years regular follow-up. DISCUSSION: Appendicular mucocele is a rare disease with vague symptoms. Abdominal imaging is an important diagnostic tool, but histopathology is the standard for definitive diagnosis. Surgery for benign appendicular mucoceles has an excellent long-term prognosis.