Pheochromocytoma multisystem crisis successfully treated by emergency surgery: Report of a case

Pheochromocytomas are rare neuroendocrine tumors that produce symptoms through the excess release of catecholamines. The treatment of choice is a complete surgical removal after pretreatment with an α-blocker, to prevent dangerous hemodynamic fluctuations. Newell and colleagues defined the rare, fatal condition of catecholamine crisis, which includes multiple organ failure (MOF), severe blood pressure variability, high fever, and encephalopathy, as pheochromocytoma multisystem crisis (PMC). The indications for emergency surgery in this unstable state still remain controversial. This report presents the case of a 52-year-old female patient with PMC who successfully underwent a surgical resection. This case showed that early tumor removal may be the only means of halting the progression of this disease.     

Lemierre's syndrome: the link between a simple sore throat, sore neck and pleuritic chest pain

An unusual case of tonsillitis which showed progression to this rare syndrome despite treatment with intravenous antibiotics. Lemierre's syndrome is a rare condition characterised by a triad of: sepsis, thrombophlebitis of the internal jugular vein along with pleuropulmonary and/or distant metastatic abscesses. Diagnosis rests on a high index of suspicion and is confirmed by culture of Fusobacterium spp. from blood or infected sites. Radiological investigations are established aids for confirmation of diagnosis. Treatment is with appropriate antibiotics for at least 6 weeks and surgical drainage of abscesses if required. This case has been presented to highlight the mode of presentation, diagnostic tools employed and the management of the complications that featured in this condition.     

Lung Transplantation as Successful Treatment of End-stage Idiopathic Pleuroparenchymal Fibroelastosis: A Case Report

Pleuroparenchymal fibroelastosis (PPFE) is a rare condition, characterized by predominantly upper-lobe pleural and subjacent parenchymal fibrosis, the latter being intra-alveolar with accompanying elastosis of the alveolar walls that leads a clinical progression to respiratory failure. This condition may not be as rare as it seems to be, because nowadays the increasing awareness among specialists is raising the number of new diagnoses. Limited data are available about the prognosis, both for secondary and idiopathic forms. Nevertheless, the idiopathic form seems to be rapidly progressive and no treatment can control the disease, which is why management is challenging. Since the disease was characterized, PPFE cases have been reported in the literature, but most have been secondary rather than idiopathic. Of these, few have successfully undergone lung transplantation as a treatment of end-stage respiratory failure. We here report a successful case of a 38-year-old man affected by idiopathic PPFE who underwent bilateral lung transplantation after extracorporeal membrane oxygenation bridging for an abrupt transition to critical clinical conditions. After a complex postoperative course and a first year characterized by acute rejection, the patient is alive at 5 years with a good quality of life. Our experience confirms that lung transplantation would be a valuable treatment option in case of end-stage idiopathic PPFE cases.     

LIFE-THREATENING GLOSSOPHARYNGEAL NEURALGlA

Glossopharyngeal neuralgia is a rare condition that, on occasions, may be associated with cardiac dysrhythmia. This case report describes the case of an 86 year old woman whose glossopharyngeal neuralgia was complicated by life-threatening dysrhythmias and who was cured by posterior fossa rhizotomy.     

Life-threatening glossopharyngeal neuralgia.

Glossopharyngeal neuralgia is a rare condition that, on occasions, may be associated with cardiac dysrhythmia. This case report describes the case of an 86-year-old woman whose glossopharyngeal neuralgia was complicated by life-threatening dysrhythmias and who was cured by posterior fossa rhizotomy.     

Bilateral traumatic abducens nerve paralysis with cervical spine flexion injury

Bilateral traumatic abducens nerve palsy is a rare condition. We report a case associated with cervical spine flexion injury. This may be the first such case report, as no similar case was found in our review of the literature. The mechanisms of injury in this case are relevant to theories that explain hyperextension injuries.     

Tuberculosis of the Thyroid Gland. A Case Report

Tuberculosis of the thyroid gland is a very rare condition. A case of a 47-year-old female with a history of a euthyroid multi-nodular goitre is presented. The patient developed hoarseness due to right recurrent laryngeal nerve paralysis, confirmed with indirect laryngoscopy. On repeated ultrasound, volumetric progression of a dominant nodule in the inferior part of the right thyroid lobe was noticed. Fine needle aspiration was inconclusive. Diagnosis was ultimately based on histopathology of the surgical resection specimen.     

Advanced cancer with situs inversus totalis associated with KIF3 complex deficiency: Report of two cases

Situs inversus totalis (SIT) is a relatively rare congenital anomaly, occurring at an incidence of 1 in 10 000–50 000 live births. Although there are some case reports of SIT with the presence of cancer, there are few reports on the relationship between SIT and cancer. However, the recent phylogenetic investigations of this condition suggest that this may be linked to the development and progression of cancer on the molecular level. The key elements are one of the intracellular motor proteins, the KIF3 complex, and the cell-adhesion factors N-cadherin and β-catenin. We herein present the cases of advanced gastric cancer and lung cancer with SIT, and review the relationship between SIT and the development and progression of cancer.     

Osteofibrous dysplasia of the ulna.

Osteofibrous dysplasia is a rare condition usually ffecting the tibiae and fibulae of males in the first two decades of life. Involvement at the ulna has also been reported in some cases. We describe an extensive, rapidly progressive lesion of the ulna in a three-year-old girl. The progression of the disease accelerated after a local marginal removal, and required extensive resection of the ulna with reconstruction using a fibular autograft. The particular features of this case are the change in the aggressive nature of the condition, which has not been previously reported. We believe that this is only the second recorded case of histologically proven involvement of the ulna.     

Superficial siderosis of the central nervous system: secondary progression despite successful surgical treatment, mimicking amyotrophic lateral sclerosis. Case report and review

Superficial siderosis of the central nervous system is a rare disorder with hemosiderin deposition in the spinal and cranial leptomeninges and subpial layer, mostly from repetitive subarachnoid hemorrhage. Progressive sensorineural deafness, cerebellar ataxia, and pyramidal signs comprise the typical clinical presentation. We describe a 47-year-old patient, who showed initial 2-year improvement after successful occlusion of an intradural bleeding source at T4. Secondary progression of symptoms without further bleedings was noted thereafter, with a clinical picture of amyotrophic lateral sclerosis. This case illustrates that the disease may progress secondarily even without re-bleedings, and that secondary progression might be due to a similar pathomechanism as in amyotrophic lateral sclerosis.     

Convergent radioulnar translocation with radial head fracture in a child: a case report and review of the literature

SUMMARY: Convergent type radioulnar translocation is an extremely rare form of injury occurring in a growing child. We report a case of convergent radioulnar translocation along with a radial head fracture in a young child. Open reduction of the elbow and internal fixation of the radial head fragment helped in achieving concentric reduction and good functional outcome at 2 years of follow-up. This case has been shown to highlight the diagnostic and management issues of this rare condition, which may be easily missed initially.     

Sewing hot and cold: successful replant of a hand in a patient who developed malignant hyperthermia intraoperatively.

We report a case in which a patient developed malignant hyperthermia (MH) during surgery to replant his hand. His condition was stabilised without the need to cease the procedure which continued for a further 6 h. MH and traumatic amputation of a hand are both rare emergencies. This case demonstrates that, MH may be controlled to allow a lengthy operation to be completed.     

Progressive post-traumatic ankle arthrosis treated with total ankle joint replacement: a case review

This article discusses the joint degeneration progression associated with post-traumatic arthrosis of the ankle. A representative case study of this debilitating condition was outlined, and treatment with total ankle joint replacement was presented. Although ankle arthrodesis continues to be a standard option following the progression of severe post-traumatic osteoarthritis, total ankle replacement is maturing as a viable option for this condition.     

Osteonecrosis of the jaws and bisphosphonates

Osteonecrosis of the jaws associated with bisphosphonate therapy was first identified in 2003 as a condition typified by exposed bone that does not heal after 8 weeks. Other signs and symptoms, such as pain and infection, may or may not be present. There is a strong need for consensus on a case definition for this condition. This condition has occurred primarily among cancer patients treated with the aminobisphosphonates zoledronic acid and pamidronate. The etiology of this condition remains unknown; however, oral disease and trauma appear to be important risk factors. This condition appears to be rare in metabolic bone disease and Paget’s disease, with an estimated prevalence of approximately 1 per 100,000 person-years. Thus, the benefits of bisphosphonate therapy appear to outweigh the risks. Recommendations have been established for the oral health management of patients with a history of bisphosphonate therapy.     

Entrapment of the basilar artery within a clivus fracture: Case report and literature review

A review of the literature revealed that basilar artery (BA) entrapment is a very rare (17 cases published) and severe pathological condition, which often leads to death. We report the case of a 72-year-old man who presented with a longitudinal clivus fracture associated with a basilar artery entrapment. This entrapment was responsible for a basilar artery dissection, which led to an ischemic stroke in the pons. The patient was managed with medical treatment, mainly to avoid a progression towards an ischemic stroke. It consisted of heparin therapy followed by antiplatelet therapy, which finally resulted in a successful outcome. In BA entrapment most of the patients who had a favorable outcome received antithrombotic therapy. This suggests that antithrombotic therapy might be useful in the first line treatment of post-traumatic BA entrapment.     

Superior cerebellar aneurysm causing subarachnoid haemorrhage in a 17-year-old with alagille syndrome

Alagille syndrome is a rare autosomal dominant condition characterised by mutation in Jagged1 gene. Intracranial aneurysms may be seen in this condition and may present as subarachnoid hemorrhage. We describe the first case of superior cerebellar aneurysm rupture causing WFNS grade 1 subarachnoid haemorrhage in a 17-year-old girl. The clinical condition and management of this rare occurrence is discussed with a review of literature.     

Mucinous adenocarcinoma of the lung in association with congenital pulmonary airway malformation

Congenital pulmonary airway malformation (CPAM) is a rare developmental abnormality of the lung that has been associated with the presence of rhabdomyosarcoma, pleuropulmonary blastoma, and most commonly bronchioalveolar carcinoma (BAC) of the lung. Here, we report the case of an 8-year-old patient who developed KRAS mutation positive stage IV mucinous adenocarcinoma of the lung in association with CPAM. This case reflects the previously recognized progression of CPAM to malignancy and suggests that BAC arising in CPAM may take a more aggressive course than previously recognized.     

Pruritic urticarial papules and plaques of pregnancy: an unusual case

Pruritic urticarial papules and plaques of pregnancy is considered the most common pruritic skin condition seen in pregnancy, and its classic presentation and treatment options have been well described. However, the exact etiologic factor of this condition remains obscure. This article presents an unusual case to demonstrate a variation in presentation of this disease as well as a unique course of treatment. The unusual progression of this case may render new insight regarding the etiologic factor of this disease.     

Spontaneous rupture of the inferior vena cava: case report and literature review

Spontaneous rupture of the inferior vena cava is a rare clinical entity. Diagnosis of this condition, in the absence of any relevant history, is usually made at laparotomy. Only one such case has previously been reported in the literature. We report a case of spontaneous rupture of the inferior vena cava which was diagnosed following laparotomy for hypovolaemia and acute abdominal pain. This case highlights the fact that spontaneous rupture of the inferior vena cava may be a cause of massive intra-abdominal bleeding not associated with trauma or rupture of the abdominal aorta.     

Bilateral adrenal hemorrhage after colectomy for perforated diverticulitis: A case report

IntroductionBilateral adrenal hemorrhage can lead to acute adrenal insufficiency. This is a rare complication in the post-operative setting, and we present a case in which it developed after a colectomy for perforated diverticulitis.Presentation of caseThe patient is a 65-year-old female who presented with abdominal pain, nausea, emesis, and hematochezia, and CT scan showing sigmoid diverticulitis with peri-sigmoid abscess. After a failure of non-operative treatment, she underwent Hartmann’s resection, and her post-operative course was complicated by refractory tachycardia, hypotension, hyponatremia, and nausea/vomiting. Bleeding, hypovolemia, and sepsis were ruled out. A CT scan showed enlarged poorly defined adrenals bilaterally, suggestive of bilateral adrenal hemorrhage. Serum cortisol level was low and diagnostic of acute adrenal insufficiency. With intravenous steroid therapy (hydrocortisone), her vital signs, laboratory abnormalities, and diet intolerance all resolved. She was discharged on oral prednisone and continued long term.DiscussionBilateral adrenal hemorrhage is rare post-operatively and can lead to adrenal insufficiency. 15% of patients who die in shock have bilateral adrenal hemorrhage on autopsy, indicating the necessity of timely diagnosis and treatment of this condition. Corticosteroid therapy is the mainstay of treatment.ConclusionThis case study illustrates that post-operative delay of progression or worsening of condition, with no alternative explanation, can be due to acute adrenal insufficiency resulting from bilateral adrenal hemorrhage, and timely diagnosis and treatment of this condition is paramount for a favorable outcome.