Von Hippel-Lindau (VHL) syndrome is characterized by hemangioblastomas involving the brain, spinal cord and cystic lesions of intra-abdominal organs such as the liver, kidney and pancreas.1 To our knowledge there have been no reports of these lesions involving the airway. Presence of cystic lesions in the airway could result in an unanticipated difficult airway. Inadvertent injury to a vascular epiglottic cyst resulting in airway bleeding is a dreaded complication that makes a difficult airway even more difficult to secure. CASE REPORT Our patient, a 43 years old gentleman, a diagnosed case of VHL syndrome, presented with headache, giddiness and gait unsteadiness. He was previously operated upon twice for cerebellar hemangioblastomas. Clinical examination and imaging revealed recurrence of the cerebellar hemangioblastoma requiring excision. Ultrasound of the abdomen revealed multiple cysts in pancreas and kidneys. Biochemical investigations were negative for pheochromocytoma. Airway assessment revealed adequate mouth opening, modified Mallampati score of 2 and restricted neck extension. In operating room, under standard monitoring, patient was induced with Propofol and Fentanyl. Intubation was attempted after …show more content…
The pathogenesis of VHL syndrome involves mutation in VHL gene located in the short arm of chromosome 3 which encodes a messenger RNA.1 This eventually leads to abnormalities in protein complexes involved in regulation of senescence, oxygen-sensing pathway, microtubule stability and orientation, cilia formation, assembly of collagen (type IV) and fibronectin in extracellular matrix and in tumor suppression. Although not described previously, the pathology can theoretically involve airway mucosa as it also contains epithelial cells, cilia, type IV collagen and
Justin is the registered nurse that has been given the handover for Kelly Malone’s postoperative care in the surgical unit. Kelly Malone is a 49 female patient who has had a septoplasty and a right ethmoidectomy. Justin is working with Kelly to identify Kelly’s needs in order for Kelly to be discharged from the hospital. Kelly’s postoperative observations were a temperature of 36.2 degrees celsius; heart rate of 68 beats per minute; respiratory rate of 18 breaths per minute, blood pressure of 111 systolic over 73 diastolic millimetres of mercury; oxygen saturation at 93 percent of room air and a self-rated pain score of two out of ten. Kelly has a history of ‘not being able to breathe well through her nose’ and a history of disturbed sleep.
A second lesion measuring 25cm in size in the left lower lobe was noted and a prevascular mass measuring almost 5cm, likely to be an enlarged lymph node was als noted. A PET scan has confirmed intense avidity of the left upper lobe and prevascular lesions with likely central
Here is a case presented with distension of abdomen with ascites, bilateral pedal oedema, macrocephaly, left half facial edema, left half hypertrophied tongue, dental anamolies. acanthosis nigricans, acrochordons and syndactyly consistent with a diagnosis of Hennekam syndrome. The diagnosis of Hennekam is
He has 2+ radial pulses, normal sensation throughout his hands, grossly. Less than two second cap refill. No swelling of the hand or the wrist itself. He also has several papules and one vesicular lesion on his upper abdomen and chest wall. Nothing on the right arm.
Epithelioid Hemangioendothelioma is a rare form of cancer that affects the blood vessels of an individual and has an unknown etiology. It is considered as a low-grade cancer because it grows and spreads slowly. However, it can become malignant. It typically starts in soft tissues like the liver or the lung, and can spread to nearby or distant organs. Epithelioid Hemangioendothelioma affects about 0.1 percent of the American population with an overall 5-year survival rate of 55% after a standard primary radical treatment.
Other imaging test includes a positron emission tomography (PET) and an M-iodobenzylguanidine (MIBG), which detects the tiny radioactive compounds that are taken up by the tumor. If Pheochromocytoma is left untreated can cause a number of critical conditions such as a stroke, heart disease, kidney failure, and even damage the nerves of the eye. When the Pheochromocytoma is assisted with other disorders the cancerous cells brake of and spread to other parts of the body and metastasize. It will metastasize in other areas of the body but mainly directs itself to the liver, lings or the lymph system. It is a lot easier to find the tumors using images, which is why they are mostly found when the patient is getting other screenings done.
The weakened breathing results in constant blowing of the nose, stuffiness, and an incapability to strongly smell both satisfying scents and unsatisfying stenches. Although weakened breathing is similar to the encounter of uncomfortable pain, it is also unique from the other negative effects. In contrast, this consequence is more of a long term consequence that can potentially remain for many years after the procedure. This is due to an inflammation in tissues in the nose that block the nasal canals. In this case, the doctor prescribes nasal sprays (typically Nasacort or Zetonna) to shrink the inflamed tissues.
MULTIPLE MYELOMA 72 year old african american male presents to your office with persistent pain in his left upper arm over the last few weeks. Pain is non-radiating and worse at night. You have seen this patient four weeks ago for the same complaint and prescribed acetaminophen. He also states that he feels exhausted lately. The patient denies any history of fever, weight loss, weight bearing or trauma.
Increasing doses of propofol, up to 420mg, were administered
At present it is treated symptomatically and therapeutically. It is important to identify which of the features that the individual with 22q11.2DS presents. Potentially serious defects such as cardiac abnormalities can be spotted early and be corrected with surgery. Similarly, a hypoplastic thymus can be replaced with a transplant from a donor to restore optimum immune function. If infections are still contracted, then antibiotics can be taken.
h-1 in group P and increase the concentration of sevoflurane to1.5% - 2% in group S. 4 Intervention was considered necessary if SPO2 < 94%, EtCO2 > 45 mmHg, apnea (cessation of spontaneous respiration for 20 seconds), significant bradycardia or significant arrhythmia. In case of apnea or suspected airway obstruction, the MRI examination was interrupted, the patient was taken out of MRI unit and the airway patency was assessed. When the scan completed, propofol infusion or sevoflurane inhalation was discontinued; the child is then transferred to the post anesthesia care unit (PACU) while breathing supplemental oxygen by face mask and monitored by pulse oximetry. After recovery from anesthesia, the child was transferred from PACU to the ambulatory unit (modified Aldret score ≥8 /10) .
Even though it is a life-saving procedure, it involves heavy blows and may lead to serious injuries at times. Once this procedure is successfully done, the victim should always be seen by a doctor to assess any internal damage due to the thrusts. Choking victims also need medical attention if coughing persists or they have a feeling that something is still inside their throats. References • https://www.nlm.nih.gov/medlineplus/ency/article/000047.htm • http://www.nhs.uk/chq/Pages/2301.aspx •
Tracheostomy tube has come out. It isn’t going back in. Patient cannot breathe.
As far as the assessment, airway involvement, see if there are any singed hairs or eyebrows, look at the nose and mouth if there is redness or burns because of a lot of carbonaceous material, are there any change in the patient’s voice inflection that they have stridor already. These are all the clues that make our case to immediately rapid sequence intubation to the patient to secure an airway to prevent the patient from inhalation injury. But if you decide to wait on the airway you are risking it for the patient’s life. For pediatrics, it’s more difficult than an
• During conscious sedation policies were not followed properly. It is required to have vital signs, continuous pulse ox. and ECG monitoring. This needs to be done pre and post procedure. • Post sedation procedures were not followed accurately.