Unilateral pulmonary vein atresia presenting with recurrent haemoptysis in a child: a case report.

BACKGROUND: Haemoptysis is an uncommon presenting symptom in children and is usually caused by acute lower respiratory tract infection or foreign body aspiration. We report a rare case of right unilateral pulmonary vein atresia (PVA) as the underlying aetiology of recurrent haemoptysis in a child. CASE PRESENTATION: A 4 years old girl presented with history of recurrent haemoptysis. Bronchoscopic evaluation excluded a foreign body aspiration but revealed right bronchial mucosal hyperaemia and varices. Diagnosis of right unilateral PVA was suspected on transthoracic echocardiography which demonstrated hypoplastic right pulmonary artery and non-visualization of right pulmonary veins. Final diagnosis was confirmed on cardiac CT angiography. A conservative treatment approach was opted with consideration for pneumonectomy in future when she is older. CONCLUSION: Rarer causes should be considered when investigating for recurrent haemoptysis in children. Bronchoscopy and cardiac imaging are useful tools to establish the diagnosis of unilateral PVA in our case.

A 4-year review of surgical and oncological outcomes of endoscopic endonasal transpterygoid nasopharyngectomy in salvaging locally recurrent nasopharyngeal carcinoma.

PURPOSE: To study the surgical and oncological outcomes of endoscopic endonasal transpterygoid nasopharyngectomy (EETN) in salvaging locally recurrent nasopharyngeal carcinoma (NPC). METHOD: This was a retrospective clinical record review study carried out at a tertiary centre from June 2013 until May 2017. A total of 55 locally recurrent NPC patients (rT1-rT4) underwent EETN performed by single skull base surgeon with curative intention with postoperative adjuvant chemotherapy but without postoperative radiotherapy. RESULTS: There were 44 (80.0%) males and 11 (20.0%) females, with mean age of 52.5 years. The mean operating time was 180 min (range 150-280 min). 85% (47/55) of patients achieved en bloc tumour resection. 93% (51/55) of patients obtained negative microscopic margin based on postoperative histopathological evaluation. Intraoperatively, one (1.8%) patient had internal carotid artery injury which was successfully stented and had recovered fully without neurological deficit. There were no major postoperative complications reported. During a mean follow-up period of 18-month (range 12-48 months) postsurgery, five patients (9.1%) had residual or recurrence at the primary site. All five patients underwent re-surgery. One patient at rT3 passed away 6 months after re-surgery due to distant metastasis complicated with septicaemia. The 1-year local disease-free rate was 93% and the 1-year overall survival rate was 98%. CONCLUSIONS: EETN is emerging treatment options for locally recurrent NPC, with relatively low morbidity and encouraging short-term outcome. Long-term outcome is yet to be determined with longer follow-up and bigger cohort study. However, a successful surgical outcome required a very experienced team and highly specialised equipment.

Cavernous sinus fungal infection: a rare case.

Intracranial fungal infection of the cavernous sinus is a condition that usually affects immunocompromised individuals and is rarely seen in immunocompetent individuals. It is a potentially life threatening condition which requires prompt treatment. Here we present a case of an immunocompetent patient with a fungal infection of the cavernous sinus.

3 Tesla magnetic resonance imaging noise in standard head and neck sequence does not cause temporary threshold shift in high frequency.

High acoustic noise level is one of the unavoidable side effects of 3 T magnetic resonance imaging (MRI). A case of hearing loss after 3 T MRI has been reported in this institution and hence this study. The objective of this study was to determine whether temporary threshold shift (TTS) in high frequency hearing occurs in patients undergoing 3 T MRI scans of the head and neck. A total of 35 patients undergoing head and neck 3 T MRI for various clinical indications were tested with pure tone audiometry in different frequencies including high frequencies, before and after the MRI scan. Any threshold change from the recorded baseline of 10 dB was considered significant. All patients were fitted with foamed 3 M earplugs before the procedure following the safety guidelines for 3 T MRI. The mean time for MRI procedure was 1,672 s (range 1,040-2,810). The noise dose received by each patient amounted to an average of 3,906.29% (1,415-9,170%). The noise dose was derived from a normograph used by Occupational Noise Surveys. This was calculated using the nomograph of L eq, L EX, noise dose and time. There was no statistically significant difference between the hearing threshold before and after the MRI procedures for all the frequencies (paired t test, P > 0.05). For patients using 3 M foamed earplugs, noise level generated by 3 T MRI during routine clinical sequence did not cause any TTS in high frequency hearing.

Intrasellar pituitary mucocele: diagnostic dilemma.

Isolated intrasellar pituitary mucocele following transsphenoidal sinus surgery is extremely rare. The clinical features resemble a pituitary tumor, therefore careful radiological interpretation is crucial to reach the correct diagnosis. We report a case of intrasellar mucocele who had transsphenoidal sinus surgery performed 15 years prior.

A new modified speculum guided single nostril technique for endoscopic transnasal transsphenoidal surgery: an analysis of nasal complications.

Abstract The endoscopic transnasal, transsphenoidal surgical technique for pituitary tumour excision has generally been regarded as a less invasive technique, ranging from single nostril to dual nostril techniques. We propose a single nostril technique using a modified nasal speculum as a preferred technique. We initially reviewed 25 patients who underwent pituitary tumour excision, via endoscopic transnasal transsphenoidal surgery, using this new modified speculum-guided single nostril technique. The results show shorter operation time with reduced intra- and post-operative nasal soft tissue injuries and complications.

A Rare Case of Complicated Sinonasal Meliodosis Mimicking Sinonasal Lymphoproliferative Disease.

Unilateral nasal obstruction with purulent nasal discharge is one of the presentations for sinonasal melioidosis. However, it may mimic nasal NK/T cell lymphoma. Both causing tissue destruction involving nasal septum, lateral nasal wall and palate. Here, we report a case of disseminated melioidosis involving sinonasal mimicking nasal lymphoma in a 32-year-old immunocompetent lady. She presented with prolonged fever, unilateral nasal blockage, painful facial swelling and knee pain. Clinical findings revealed extensive necrotic tissue and crusting involving right lateral wall of nasal cavity. Tissue and blood culture and sensitivity (C + S) grew Burkholderia pseudomallei. Recovery was complete after surgery and antibiotics.

A Rare Case and Literature Review of Endoscopic Endonasal.

We report a rare case of localized anterior clinoid mucocele (ACM) with acute visual loss and a literature review of the endoscopic endonasal surgery. We advocate that endoscopic endonasal approach is the best choice. To our knowledge there a limited report on localized ACM that treated via endoscopic endonasal approach.

A Rare Case of Cerebrospinal Fluid Rhinorrhea from Canal of Stenberg.

Background: A spontaneous cerebrospinal leak from Sternberg's canal with meningoencephalocele is a very rare clinical entity. Endoscopic repair of the defect is challenging and crucial in identifying the defect. The aim of this case report is to highlight the presence and management with endoscopic surgery in repairing Sternberg canal. Case: 40-year-old woman presents with spontaneous CSF rhinorrhea with no predisposing factors. CT imaging and MRI showed osteodural defect in the lateral recess of sphenoid with meningoencephalocoele lateral to the foramen rotundum. Endoscopic transethmoidal - transphenoidal - transpterygoid approach was used to repair the defect, and patient is well post-operative with least complication from the intervention surgery. Conclusion: Endoscopic approach proved to be the best and safest method in localizing the defect and closure of the leak. Angled scopes and image guided system were used to identify the precise location of the leak. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-022-03347-z.

An extensive cervical vagal nerve schwannoma: a case report.

Vagal nerve schwannoma is extremely rare. The majority of cases present with a slow growing neck swelling without neurological deficit. Magnetic resonance imaging is the gold standard investigation to establish a pre-operative diagnosis. We report a case of a 32-year-old man with an extensive right vagal nerve schwannoma involving the right jugular foramen and parapharyngeal space. The tumour was resected via a transcervical approach. Complete excision of the tumour is the key to prevent recurrence.

Review of ear, nose and throat foreign bodies in Sarawak General Hospital. A five year experience.

OBJECTIVE: Ear, nose and throat foreign bodies are common in ENT clinical practice. This study was designed to establish the local data of otorhinolaryngeal foreign bodies in term of prevalence among paediatric and adult groups, the clinical features, types of foreign body at different sites, and laterality of foreign bodies. METHOD: This study was carried out at ENT department, Sarawak General Hospital, Malaysia, from 1st January 2005 to 31st December 2009. A total of 1084 cases were included and statistically analyzed. RESULT: Ear foreign bodies showed the highest incidence which was consisted of 480 (44.3%) cases, followed by nose in 270 (24.9%) cases, pharynx in 251 (23.2%) cases, esophagus in 57 (5.3%) cases and laryngo-tracheobronchial tree in 26 (2.4%) cases. Otorhinolaryngeal foreign bodies occurred more frequently in 0-10 year old age group which constituted 651 (60.1%) cases. The descending order of frequency for foreign body sites in adult was pharynx (17.2%), ear (12.8%), esophagus (3.1%), nose (1.7%) and laryngo-tracheobronchial tree (1.1%). The type of foreign bodies varies with age group and site of foreign body lodgement. In general, common foreign bodies in both adult and children were food related, with the additional of small objects such as plastic toy in paediatric group. CONCLUSION: Otorhinolaryngeal foreign bodies were found more frequently in children. The types of foreign body were different from age group and sites of foreign body lodgement. The local food constituted the highest incidence of ear, nose, and throat foreign bodies with additional of plastic toys in paediatric group.

Infrasellar craniopharyngioma of the posterior nasal septum: a rare entity.

OBJECTIVE: To report an unusual location of infrasellar craniopharyngioma in a peadiatric patient. CASE REPORT: A six-year-old boy presented with persistent bilateral nasal obstruction for one year. Clinical examination revealed a posterior choanal mass arising from septum and the finding was confirmed by paranasal sinuses computed tomography scan. He then underwent wide local excision. Histopathological examination confirmed the diagnosis of craniopharyngioma (adamantinomatous type). There were no signs and symptoms of recurrence after a year of followup. CONCLUSION: Infracranial craniopharyngioma without sellar involvement is extremely rare. Persistent nasal obstruction without endocrine dysfunction is the common presentation. Radiological imaging is important to diagnose and assess the extent. The mainstay of treatment for infrasellar craniopharyngioma is surgery. Regular follow up is mandatory.

Cervical schwannoma: report of four cases.

Extracranial schwannomas in the head and neck region are rare neoplasms. The tumours often present as asymptomatic, slowly enlarging lateral neck masses and determination of the nerve origin is not often made until the time of surgery. Preoperative diagnosis maybe aided by imaging studies such as magnetic resonance imaging or computed tomography, while open biopsy is no longer recommended. The accepted treatment for these tumors is surgical resection with preservation of the neural pathway. We report four cases of cervical schwannomas that we encountered at our center during four years of period. The clinical features, diagnosis and origin, management and pathological findings of these benign tumors are discussed.

Endolymphatic sac tumour.

We present a case of a papillary tumour at the cerebellopontine angle in a 41-year-old man. He presented with left-sided facial and ear pain associated with dizziness, nystagmus and hearing loss. CT scan of the temporal bone showed a destructive tumour at the left cerebellopontine angle. Surgical excision was performed and the diagnosis of the endolymphatic sac tumour was made. Endolymphatic tumour is a low grade adenocarcinoma that originates from the endolymphatic sac. The definitive diagnosis requires a combination of clinical features, radiological finding and pathological correlation.

Pharyngeal Lipoma-A Potentially Life Threatening Presentation.

Pharyngeal lipoma of the upper aero-digestive system is extremely rare. It is typically benign, slow growing and symptoms would depend on its size and location. Surgical intervention is often needed especially for large tumour with impending airway obstruction. Here we present a case of potentially life threatening presentation of pharyngeal lipoma.

Endoscopic Endonasal Approach in Clival Chordoma Surgery: Case Series.

Chordomas are rare and slow-growing locally destructive bone tumors that can develop in the craniospinal axis. It is commonly found in the sacrococcygeal region whereas only 25-35% are found in the clival region. Headache with neurological deficits are the most common clinical presentations. Complete surgical resection either via open or endoscopic endonasal approaches are the main mode of treatment. Here, we report a series of 5 cases of clival chordomas which was managed via endoscopic endonasal approaches in our center. A retrospective analysis of patients who had undergone endoscopic endonasal resection of clival chordoma in Sarawak General Hospital from 2014 to 2018. A total of 5 cases were operated on endoscopically via a combine effort of both the otorhinolaryngology team and the neurosurgical team during the study period from year 2013 to 2018. From our patient, 2 were female and 3 were male patients. The main clinical presentation was headache, squinting of eye and nasopharyngeal fullness. All our patient had endoscopic endonasal debulking of clival tumor done, with average of hospital stay from 9 - 23 days. Pos-operatively, patients were discharged back well. Endoscopic endonasal resection of clival chordomas gives good surgical resection results with low morbidity rates and therefore can be considered as a surgical option in centers where the surgical specialties are available.

A caroticocavernous fistula without vascular injury following endoscopic transsphenoidal excision of a tuberculum sellae meningioma-A case report.

Caroticocavernous fistulae can occur following transsphenoidal surgery even without evidence of carotid artery injury. A role of vascularized flap reconstruction may be contributory.

Spontaneous orbital haematoma in a scurvy child: A forgotten diagnosis.

Spontaneous unilateral orbital haematoma in children is not common and very rarely caused by scurvy. Scurvy is a clinical syndrome with a spectrum of clinical manifestations due to severe prolonged vitamin C deficiency leading to impairment of collagen synthesis over skin, bone, teeth and blood vessels. This paper presents a unique case of a 7- year-old girl with learning difficulty who presented with spontaneous right proptosis due to scurvy. Imaging studies suggestive of intra- orbital extraconal haemorrhage. The child was treated with a higher than recommended dose of vitamin C initially in emergency situation. She responded well and discharged without complication. Spontaneous orbital haematoma due to scurvy is very rare with less than 10 cases published in literature. The present case should raise the awareness regarding this forgotten disease and importance of balance nutrition amongst children.

Adenoid Cystic Carcinoma of the Nasopharynx: A Case Series.

Adenoid cystic carcinomas are tumors that are more commonly seen in the salivary glands than in the nasopharynx. Nasopharyngeal adenoid cystic carcinomas are rare and cases that are reported in the literatures are few. Treatment is mainly by surgical resection. Here, we report 2 cases of nasopharyngeal adenoid cystic carcinoma.

Management of sphenoid lateral recess encephalocoeles.

PURPOSE OF REVIEW: Sphenoid sinus lateral recess encephalocoeles (SSLRE) are rare occurrences and pose unique challenges due to limited surgical access for endoscopic endonasal repair and also the lack of consensus on optimal perioperative managements specifically in the spontaneous cases, which are also believed to be a variant of idiopathic intracranial hypertension (IIH). Endoscopic endonasal approaches have largely replaced the transcranial route and the techniques are continuously being refined to reduce the neurovascular morbidity and improve outcome. RECENT FINDINGS: Transpetrygoid is the most utilized approach with modifications suggested to limit bone removal, exposure and preservation of the neurovascular structures as dictated by the extent of the lateral recess. As more experience is gained, extended transphenoidal techniques were also successfully used for access. Lateral transorbital is a new approach to the lateral recess investigated in cadavers. IIH treatment is still controversial in the setting of SSLRE, but it appears rationale to evaluate, monitor and treat if necessary. SUMMARY: SSLRE management should be tailored to the specific anatomical variances and cause. Modifications of techniques have been described giving different options to access the lateral recess. Successful repair for spontaneous SSLRE may require treatment of IIH if present, but the long-term outcome is still unclear.