Facial Swelling Research Articles

Staggered botulinum toxin-a injections into parotid and submandibular glands prior to four-duct ligation for pediatric sialorrhea.

This study aimed to assess the effectiveness of four-duct ligation following Botulinum toxin-A injections into the parotid and submandibular glands in pediatric patients with sialorrhea resistant to nonsurgical treatments. Prior research has individually explored either surgical or Botulinum toxin interventions; however, the safety and efficacy of the combined approach to these treatments have yet to be documented. Patients were assessed before surgery and 6, 12, and 24months post-operatively. Evaluations involved interviews with parents and caregivers, conducted face-to-face or by telephone, using the Drooling Severity and Frequency Scales. Additionally, metrics such as the daily count of bib changes and the hourly frequency of saliva wiping were recorded. Quality-of-life assessments were performed before and after the surgical procedures. All complications associated with the interventions were carefully recorded. The study group consisted of 25 participants, with a median age of 6years (range 2-17years). Each participant was diagnosed with a neurological, metabolic, or genetic disorder. Evaluations were conducted periodically, culminating in a final follow-up at 24months. The treatment demonstrated a 100% success rate at six months post-operation (25/25 patients), which slightly decreased to 96% at the 12-month mark (24/25 patients) and further to 84% by the 24-month follow-up (21/25 patients). Major complications were not observed. However, minor complications were reported in six patients (24%): three exhibited temporary facial swelling (12%), two encountered minor bleeding (8%), and one experienced excessive dryness (4%). The combination of Botulinum toxin-A injection 3 weeks prior to the four-duct ligation procedure demonstrated high success rates and substantially reduced the incidence of infectious and cosmetic complications. Consequently, this staggered approach to combination treatment is recommended for managing pediatric sialorrhea cases that are resistant to non-surgical interventions.

Pregabalin as an Effective Adjunct in Postoperative Care following Third Molar Removal

Background: The surgical removal of impacted mandibular third molar is probably the most commonly performed procedure in oral & maxillofacial surgery. This study aimed to evaluate the effectiveness of pregabalin as an adjunct to standard analgesia for managing postoperative pain, facial swelling, and trismus following mandibular third molar extraction.  Methods: A total of 136 patients undergoing bilateral extractions were included. Each patient served as their own control, receiving pregabalin with standard analgesia for one extraction and only standard analgesia for the other. Postoperative pain was assessed using the Visual Analogue Scale (VAS) at various intervals, while facial swelling and interincisal distance (IID) were measured on Days 1, 3, 7, and 14.  Results: VAS scores were higher in the control group; however, it was not statistically significant (P = 0.000) at any time interval. % facial swelling was significantly lower in the test group on postoperative day 1[2.32(1.77- 2.95) test; 2.32 (1.82-3.60) control; P = 0.009] and day 3 [6.67 (5.17-8.44) test; 7.49 (5.77-9.43) control; P = 0.004]. However, no significant differences were seen on postoperative day 7 and day 14. Similarly, no significant differences in mouth opening measurements were found.  Conclusion: Pregabalin is effective as an adjuvant therapy in reducing pain and facial swelling after third molar surgery. However, multicenter, randomized, double- blinded studies are recommended to confirm these findings.

Isolated fungal sphenoid sinusitis with unilateral lateral rectus palsy six years post lung transplant

Rhino-orbital-cerebral mucormycosis (ROCM) infections are rare and usually occur in patients with diabetes, malignancy, or organ transplantation. The most common presenting symptoms include facial pain and swelling, fever, and rhinorrhea. Mortality rates reach nearly 50%. Those with previous organ transplants typically present with sinus symptoms a few weeks to months after transplantation. A 75-year-old man presented with headache. His history was significant for bilateral lung transplantation in 2017. On his fourth presentation, he was admitted to the hospital for work up. Imaging showed small fluid levels within the right maxillary and sphenoid sinuses. Infectious work-up revealed no meningitis. On day three, the patient complained of diplopia on the right. His examination was significant for right lateral rectus palsy. Repeat imaging was performed and showed increasing fluid levels of the right maxillary and sphenoid sinuses, and the otolaryngology consultation service was consulted. Nasal endoscopy was significant for pink, vascularised mucosa with no obvious regions of pallor or necrosis. Endoscopic sinus surgery was performed. There were no findings suggestive of fungus but purulence of the right sphenoid grew Rhizopus. The patient’s nerve palsy did not resolve and progressed to the contralateral orbit. Repeat nasal endoscopy continued to show healthy mucosa while MRI showed enhancement worrisome for meningitis. To our knowledge, this is the only reported case of fungal sphenoid sinusitis resulting in meningitis and death six years after transplant. Current literature documents fungal infection in post-transplant patients up to four years after surgery, with most occurring within the first year. Suspicion for fungal infection should remain high in this patient population. Keywords: mucormycosis, lung transplant, sinusitis, lateral rectus palsy

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) in a 15-Year-old male presenting with orofacial Swelling

BackgroundSubcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of non-Hodgkin lymphoma that primarily affects subcutaneous tissues. Its occurrence in the orofacial region is exceptionally uncommon, presenting diagnostic challenges due to symptom overlap with more prevalent orofacial condition.Case presentationThis report details the case of a 15-year-old male who presented with persistent left-sided facial swelling, initially misdiagnosed as facial cellulitis. Diagnostic complexity arose from the similarity of symptoms to common orofacial diseases. Comprehensive diagnostic approaches, including liquid-based thin-layer cytometry, immunohistochemistry, and advanced imaging, were pivotal in identifying SPTCL. The recurrence of symptoms following the cessation of dexamethasone treatment indicated hormone dependency. Surgical intervention and subsequent histopathological analysis confirmed SPTCL, with immunohistochemical profiling playing a critical role in the definitive diagnosis. The patient’s management involved a multidisciplinary approach, leading to a referral to a hematology specialist and subsequent favorable outcomes.ConclusionsThis case underscores the diagnostic challenges of orofacial lymphomas such as SPTCL and highlights the necessity for early, accurate pathological diagnosis. It emphasizes the role of advanced diagnostic techniques and the importance of a comprehensive, multidisciplinary approach in the management of such rare cases. This report contributes to the limited but growing body of literature on SPTCL in the orofacial region, providing insights for clinicians in similar future cases.

EVALUATION OF POSTOPERATİVE MORBİDİTY FOLLOWİNG OPERATIONS OF IMPACTED MANDIBULAR THIRD MOLARS WITH BONE RETENTION

This study is aimed to see how gender and whether tooth is placed on the right or left affected the length of the operation, edema, trismus, and discomfort after surgical extraction of an impacted mandibular third molar. This prospective study was conducted in the Inonu University. The study included 104 mandibular wisdom tooth with bone retention. There were 55 females and 49 males among the 104 patients, with 53 left and 51 right impacted mandibular third molar. The operations were performed by the same surgeon in the same operating room. The time between the incision and the suture was determined and recorded as the operation time for each procedure. Before surgery, on the 2nd postoperative day, and on the 7th postoperative day, the patients were compared in terms of facial swelling impacted mandibular third molar, discomfort, and trismus. IBM SPSS V23 was used to evaluate the data. The left impacted mandibular third molar extraction takes longer than the right impacted mandibular third molar extraction. Tooth 38, which have a longer operation time, are more prone to swelling, discomfort, and trismus. When tooths are extracted, right-handed surgeons cause less postoperative morbidity 48. We recommend that both right-handed, left-handed surgeons operate in clinics that conduct impacted tooth extractions.

Dual infection of maxillary sinus masquerading as a malignant lesion; A rare presentation

Primary sinonasal tuberculosis is a rare form of TB even in developing countries where TB is prevalent and invasive mycotic infection is more common in immunocompromised patients. It is unusual to have a dual infection with tuberculosis and mycosis in the maxillary region mimicking a neoplastic process and causing bone erosion. A 49-year old male presented with 2-year history of left-sided facial swelling. On microscopic examination, epithelioid cell granulomas, chronic inflammatory infiltrate, macrophages, giant cells, and focal necrosis were seen. Ziehl Neelsen staining for acid-fast bacilli with 20% H2SO4 was positive. Fungal hyphae were also seen in the sections examined. Special stains (Periodic acid-Schiff and Grocott’s methenamine silver stain) for fungal hyphae were positive. Mycosis and tuberculosis should always be considered in the differential diagnosis of maxillary lesions or sinusitis.

Maxillary Sinusitis of Odontogenic Origin- A Case Report and a Short Review of Literature

Maxillary sinusitis of odontogenic origin (MSOO) presents a diagnostic challenge due to its overlapping symptoms with other forms of sinusitis. We present a case of a 17-year-old male with MSOO, emphasizing varied treatment modalities. Clinical assessment revealed facial swelling and pain, prompting a differential diagnosis. Orthopantomogram and Cone Beam Computed Tomography (CBCT) confirmed odontogenic involvement. Surgical intervention included extraction, sinus lavage, and closure of oroantral communication. Postoperative recovery was successful. Discussion highlights the prevalence, diagnosis, and management of MSOO. Contemporary reports suggest odontogenic sources contribute to over half of maxillary sinusitis cases. Radiographs and CBCT aid diagnosis, delineating odontogenic involvement. Effective management includes dental evaluation and endoscopic sinus surgery, and early diagnosis is crucial to prevent complications. However, consensus on antimicrobial therapy and drainage routes is lacking. This case underscores the importance of interdisciplinary collaboration and tailored management strategies for MSOO.

Subcutaneous Dirofilariasis: A Zoonosis of Significance

Accidental inoculation of dirofilarial worm by mosquitos into humans cause a zoonotic disease called dirofilariasis. Common sites of involvement are lungs, eyes and rarely subcutaneous areas. This is a case report of a 60-year-old woman presented with facial swelling for the past 6 months. Ultra sonogram revealed a cyst with worm like structures inside, which was excised surgically and histopathology confirmed with diagnosis with demonstration of Dirofilaria worm. This case report aims at reminding clinicians not to overlook or misdiagnose rare diagnoses like dirofilariasis during evaluation of subcutaneous swellings.

12511 Rhabdomyolysis Related Acute Kidney Injury Presenting as First Manifestation of Autoimmune Hypothyroidism: A Case Report

Abstract Disclosure: A. Tiwari: None. S. Mantravadi: None. N. Doshi: None. A. Dabaja: None. C. Saad: None. Introduction: Autoimmune thyroiditis is the predominant cause of primary hypothyroidism, with an incidence of estimated 0.3-1.5 cases per 1000 people, with female to male predominance of 7-10[1]. Hypothyroid myopathy affects 79 % of the affected population. Its common presentation is fatigue and cramp but in rare instances rhabdomyolysis can occur with trivial triggers like alcohol, exercise2, Case Presentation: A 32-year-old male with no significant medical history, presented with generalized weakness, myalgia, and facial puffiness for last three months. The patient engaged in regular-intensity workouts and reported consuming 12 beers several times a week for past 5 years. Physical examination revealed facial puffiness and proximal muscle weakness. Initial Laboratory findings showed elevated creatinine (1.68 mg/dL, baseline 1.0 mg/dL in April 2020), CK (8113 U/L), AST (260 U/L), ALT (129 U/L). Urinalysis revealed hematuria and mild proteinuria. Serum electrolytes were unremarkable. Abdominal ultrasound showed diffuse hepatic steatosis with normal kidney appearance. This could not explain the reason for the Acute Kidney Injury and further workup showed TSH (>150 mIU/L) and TPO antibodies (197.9 U/mL). Autoimmune panel was unremarkable. Thyroid ultrasound done subsequently revealed a markedly heterogeneous gland. Treatment included parenteral crystalloids, low-dose prednisone which was empirically added for suspected autoimmune myositis and was later discontinued once the autoimmune panel was negative, and Levothyroxine at the recommended dose. At the time of discharge, his creatinine was reduced to 1.33 mg/dL and CK to 2,298 U/L. Discussion: Severe rhabdomyolysis is a rare but significant complication of autoimmune thyroiditis, potentially leading to acute kidney injury (AKI) if not promptly diagnosed and treated. Resident physicians often attribute symptoms like myalgia and generalized weakness in males to clear evident rhabdomyolysis if they find a cause like alcohol consumption and exercise, potentially overlooking the actual etiology. We recommend thyroid workup in such patients to promptly detect and manage underlying etiology predisposing to muscle damage and subsequent kidney injury. Our patient never had any Physician encounter as his prior medical history was unremarkable, however we suggest thorough family history and thyroid profile should be a part of outpatient workup as well for patients who show other risk factors for rhabdomyolysis like daily alcohol consumption, vigorous workout, consume medications like HMG CoA inhibitors, antipsychotics etc. Presentation: 6/3/2024

8014 Cushing’s Syndrome: Two Sides of The Spectrum

Abstract Disclosure: M. Aguilera: None. A. Vu: None. J. Shakil: None. R. Al-Ward: None. Introduction: Cushing’s syndrome (CS) is a rare condition with an annual incidence of 2-8 per million. Endogenous CS is mainly due to corticotroph adenoma of the pituitary followed by excessive glucocorticoid production from adrenal and ectopic sources. CS has significant morbidity and mortality hence the need for timely diagnosis. The varied presentations often pose great challenges in identifying the source of hypercortisolism. We present two unusual cases of CS with distinct clinical presentations and atypical laboratory profiles. Case 1: 25-year-old male with no medical history presented with 9 months of facial puffiness, weight gain, fluid retention, hypertension and blurred vision. Physical examination revealed round facies, bilateral supraclavicular fat pads and wide abdominal striae. Laboratory workup showed markedly elevated ACTH 537 pg/mL [7.2-63 pg/mL], serum cortisol 8.5 ug/dL [6.2-19.4 ug/dl], 24-hour urine free cortisol (UFC) 30 [5-62 ug/24H] and normal late-night salivary cortisol (LNSC). Repeat workup a month later showed serum cortisol of 56ug/dL, ACTH 874 pg/dL, 24-hour UFC of 11880ug/24H and elevated LNSC. Pituitary MRI showed a large heterogenous 3.1 cm partially cystic sellar mass with suprasellar extension. Due to significantly elevated ACTH and short duration of symptoms, we suspected an ectopic source. Due to initial negative testing, except for high ACTH level, a silent corticotroph adenoma was also considered. The patient underwent transsphenoidal resection resulting in significant decrease of cortisol and ACTH postoperatively. Pathology showed a corticotroph adenoma with Ki-67 index of <1%. Case 2: 47-year-old woman with medical history of obesity, polycystic ovary syndrome and a stable 1 cm lung nodule since 2020, presented with fluid retention, worsening acne and resistant hypertension over a 1-year period. She required hospitalization for hypertensive emergency and hypokalemia. Laboratory data showed morning serum cortisol 28 ug/dL, ACTH of 90.1 pg/mL, 24-hour UFC 346 ug/24H, elevated LNSC on two occasions and cortisol 24.3 ug/dl after a 1mg dexamethasone suppression. MRI did not show a pituitary mass. PET dotatate scan revealed a tracer avid 1 cm right upper lobe pulmonary nodule. She underwent a right upper lobe lobectomy, resulting in a significant decrease in cortisol and ACTH levels on postoperative day 1. Pathology confirmed a typical carcinoid tumor. Discussion: High ACTH levels and a short symptom duration often point to ectopic production, paraneoplastic syndromes or corticotroph carcinomas. Cushing's disease typically exhibits mildly elevated ACTH levels, but our cases deviate from this norm, complicating source identification. This atypical presentation highlights the complexity of Cushing's syndrome, challenging conventional diagnostic paradigms. Presentation: 6/1/2024

A Challenging Case Of Giant Maxillary Ameloblastoma: Navigating Rarity And Complexity In Diagnosis And Treatment

Background: Ameloblastoma is a rare, aggressive, benign odontogenic tumor that most commonly occurs in the jaw. Maxillary occurrences are rarely reported. Case Presentation: We report the case of a 61-year-old male presenting with right-sided unilateral nasal obstruction, characterized by a mass occupying the entire right nasal fossa upon anterior rhinoscopy. A computed tomography (CT) scan, combined with magnetic resonance imaging (MRI), revealed a locally advanced mass centered on the right maxillary sinus. The patient underwent wide excision of the mass, and histopathological analysis confirmed the diagnosis of ameloblastoma. Discussion: Naso-sinus ameloblastomas are rare, benign, and slow-growing tumors, accounting for approximately 0.11% of nasal and sinus tumors. More common in men, they typically present between the ages of 20 and 30. Symptoms include nasal obstruction and epistaxis, with occasional facial swelling or headaches. These tumors often originate in the posterior maxillary region and extend into the maxillary sinuses and nasal cavity. Diagnosis is facilitated by imaging, where the tumors appear as solid, radio-opaque masses. Radical surgical resection is the preferred treatment, as it is essential for preventing recurrence. The prognosis is generally favorable, with no documented cases of malignant transformation or metastasis, and recurrences are rare when appropriate surgical management is performed. Conclusion: Medical imaging plays a crucial role in diagnosis and allows for the evaluation of tumor dissemination and anatomical involvement. The treatment of maxillary ameloblastoma typically involves surgical excision, with the complexity of management often linked to the extent of tumor spread.

Coronavirus Disease-19-associated Mucormycosis: A Single-center Observational Study of 69 Patients.

We studied the clinical presentation, risk factors, complications, and in-hospital outcomes of patients with coronavirus disease 2019 (COVID-19)-associated mucormycosis (CAM). A retrospective study was done on 69 COVID-19 patients with microbiologically proven mucormycosis admitted over a period of seven months from March 2021 to September 2021. All 69 mucormycosis patients (46 males, 23 females) had reverse transcriptase-polymerase chain reaction (RT-PCR)-confirmed COVID-19 infection. Their mean age was 52.8 years, with mucormycosis developing in 51 patients (73.9%) within 30 days of COVID-19 infection; 7 (10.1%) were positive on admission. Rhino-orbital-cerebral mucormycosis (ROCM) was the most common (40.3%), followed by rhino-orbital (37.3%) and sinonasal (22.4%). Diabetes mellitus was present in 98.6% of patients. Common symptoms of mucormycosis were facial pain, headache, facial swelling, and vision loss. During COVID-19, 88.4 and 52.5% received immunosuppressive treatment and zinc sulfate, respectively; 34.7% needed intensive care unit (ICU) admission. The mortality rate was 26.1%. On multivariate logistic regression analysis, the presence of chronic kidney disease, leukocytosis, ophthalmoplegia, oral/palate ulceration, current need for invasive ventilation, and past duration of oxygen therapy and zinc supplementation were significantly associated with mortality. Patients with current COVID-19 infection had severe disease with increased need for intensive care (57.1 vs 14.5%) and higher mortality (57.1 vs 22.6%) compared to mucormycosis patients with previous COVID-19 infection. Rhino-orbital-cerebral, rhino-orbital, and sinonasal were the most common presentations in cases of mucormycosis, with a mortality rate of 26.1%. COVID-19 coinfection predisposes patients with mucormycosis to severe disease with higher mortality.

S2987 Perforated Sigmoid Diverticulitis Presenting With Facial and Neck Swelling

S2987 Perforated Sigmoid Diverticulitis Presenting With Facial and Neck Swelling

CASE REPORT ON DRESS SYNDROME INDUCED BY ANTITUBERCULOSIS DRUGS

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe adverse drug reaction characterized by an extensive skin rash in association with visceral organ involvement, lymphadenopathy, eosinophilia, and atypical lymphocytosis. We report a rare case of DRESS Syndrome secondary to anti tubercular treatment (ATT). A 53 year old male with pulmonary tuberculosis (TB) presented with fever, facial puffiness, bilateral pedal edema, itchy exfoliative rashes over face, thorax, abdomen, and extremities for 15 days after about 4 months of starting FDC of ATT. On examination there was hypotension (BP-80/60 mm Hg) and bilateral diffuse symmetrical exfoliative dermatitis over face, chest, abdomen, all 4 extremities sparing flexor aspect of thighs and legs. Investigations revealed eosinophilia, leucocytosis (predominantly lymphocytosis), raised alkaline phosphatase and acute kidney injury. FDC was withheld, skin biopsy was performed and the patient was managed as DRESS syndrome fulfilling diagnostic criteria with oral steroids (prednisolone 1mg/kg/day) and other supportive medications. The patient improved gradually with improvement in most of the symptoms and progressive return of laboratory parameters towards normal level. Individuals diagnosed with pulmonary TB who are undergoing treatment with FDC of ATT have a heightened susceptibility to DRESS. The timely identification and cessation of the offending agent can effectively mitigate mortality.

Hypothyroidism and Transudative Ascites: Highlighting a Little-known Association

Hypothyroidism is a frequent condition in medical practice and clinical forms with ascites are exceptionally rare. After a review of the literature we found that the exudative nature of the fluid is the main feature associated with hypothyroidism, however no case of hypothyroidism associated with transudative ascites has been reported. We report a case of transudative ascites associated with hypothyroidism in a post thyroidectomy setting. This 72-year-old patient underwent total thyroidectomy 3 years ago, without supplementation with synthetic thyroid hormone. He had been treated in cardiology for compensated ischaemic heart disease for 4 months. He presented with apathy, significant physical asthenia and a hoarse voice. His general condition was altered, with a rounded, puffy face and infiltrated eyelids. The feet were oedematous. Haemodynamic constants revealed arterial hypotension. Ascites aspiration yielded a sterile, pauci-cellular, citrine-yellow, transudative fluid. After ruling out renal, glomerular and hepatic causes, the ascites persisted despite optimised treatment of his heart disease, making cardiac ascites unlikely. Signs of hypometabolism and myxedema, together with a very high TSH (TSHus = 54.26 microgr/L) and disappearance of ascites after thyroid hormone supplementation, supported the hypothesis of transudative ascites associated with hypothyroidism. Ascites associated with hypothyroidism is rare and the transudative nature of the fluid is exceptional; it is generally included in myxedema. Hypothyroidism is rarely manifested by ascites but can be considered after ruling out common causes. Hormonal treatment allows complete regression of ascites within a few weeks and constitutes a therapeutic test.

Oral cavity squamous cell carcinoma complicated by internal jugular vein thrombosis

Head and neck squamous cell carcinoma (SCC) encompasses various cancers from the upper aerodigestive tract. Initial presentation often includes regional nodal involvement (40% in stage IVA or B) and distant metastases (up to 10%). Facial edema is a frequent clinical issue with multiple causes, such as allergic reactions, trauma, venous thrombosis, infections, and both benign and malignant conditions. An extremely rare condition is tumor thrombosis of the internal jugular vein (IJV), typically reported in differentiated thyroid cancer cases. We present a diagnosed case of SCC of the left lower alveolus in a 54-year-old female who presented with diffuse facial swelling and stridor two years post-diagnosis. Further evaluation through clinical examination and imaging studies revealed partial thrombosis of the right IJV. The patient underwent an emergency tracheostomy and was treated with intravenous heparin, which effectively managed the local symptoms. This case report highlights the clinical features, diagnostic methods, treatment modalities, and favorable prognosis associated with the long-term effects of untreated head and neck cancers.

An unusual case of Anasarca-Rapunzel syndrome

Rapunzel syndrome is a rare form of gastric trichobezoar extending into the intestine, often associated with malnutrition and protein-losing enteropathy. A 12-year-old female presented with epigastric abdominal pain, facial puffiness, bilateral lower limb swelling, and anorexia. She was a known case of sickle-thalassemia double heterozygous state but had never received a blood transfusion. Family history revealed parental separation and harsh paternal behavior. The patient was undernourished but otherwise developed normally. On examination, she was lethargic, exhibited generalized anasarca, severe anemia, signs of congestive cardiac failure, and patchy hair loss on the scalp. Abdominal examination revealed moderate ascites, hepatosplenomegaly, and a firm, mobile, non-tender epigastric mass (10×8 cm), suggestive of gastric bezoar. Laboratory findings showed microcytic hypochromic anemia with macro-ovalocytes in the context of sickle-thalassemia heterozygosity. The child required multiple blood and serum albumin transfusions for severe anemia and hypoproteinemia with anasarca. Hepatic and renal causes of anasarca were excluded. Cardiovascular and respiratory systems were normal. Contrast enhanced computed tomography (CECT) abdomen confirmed the gastric bezoar. After stabilization, exploratory laparotomy and trichobezoar evacuation were performed. Psychiatric counseling and diversion therapy were provided. The child was discharged in stable condition, with follow-up showing significant improvement in nutritional status, mood, and general well-being. This case highlights the unusual presentation of Rapunzel syndrome with generalized anasarca in a pediatric patient with underlying sickle-thalassemia, emphasizing the importance of multidisciplinary management and follow-up.

Clinical profile and management of pediatric hereditary angioedema in resource-constrained settings: our experience from a single centre in North India.

Hereditary angioedema (HAE) is a rare genetic disorder. The pattern of HAE is different in children as compared to adults. There is limited literature from developing countries where all first-line treatments are either unavailable or not easily accessible.Data of children with HAE were retrieved from medical records of patients registered in the Pediatric Immunodeficiency Clinic at our institute.Of the 206 patients with HAE, 61 were diagnosed before the age of 18years. Male: female ratio was 1.1:1. Median age at onset of symptoms and diagnosis were 6.2years (range 1-17years) and 10.7years (range 1.5-18years) respectively. Median delay in diagnosis was 4.9years (range 0-16years). The commonest presentation was facial swelling (51/61) followed by swelling of extremities (47/61). Laryngeal edema and abdominal symptoms were reported in 28/61 and 31/61 patients respectively. Abdominal attacks were found to be less common in children as compared to adults. Most patients in our cohort received fresh-frozen plasma (n = 5/61) as on-demand therapy. Long-term prophylaxis included attenuated androgens (n = 25/61) and tranexamic acid (n = 23/61). Median duration of follow-up was 2242 patient months. One patient died on follow-up in this cohort.This is the largest single-centre cohort of pediatric HAE from resource-constrained settings. Facial attacks were more common, and there were significant delays in diagnosis when the age of onset of symptoms was younger. Gastrointestinal symptoms were less common in children than adults. HIGHLIGHTS: One of the largest single-centre cohorts of pediatric HAE and the only one from resource-constrained settings. There were significant delays in diagnosis when the age of onset of symptoms was younger. Abdominal attacks were found to be less common in children as compared to adults.

The Dermatologist's Role in Rehabilitation: A Novel Multidisciplinary Treatment Approach Combining Ablative Fractional Laser Resurfacing and Physical Therapy in Managing a Patient with Scar Contracture of the Neck

Objective: To employ a multidisciplinary approach combining ablative fractional laser resurfacing concurrently with physical therapy to improve functional and cosmetic deficits associated with traumatic scarring status-post parotid cancer treatment. Methods: A 65-year-old female presented to dermatology with scar contracture of the right neck secondary to multiple surgeries and 36 rounds of adjuvant radiation therapy for stage III adenoid cystic carcinoma of the parotid gland. Severe scar-related complications included impaired physical strength, limited range of motion of the neck, and activity intolerance. The patient also reported difficulty with swallowing and limited ability to open her mouth, resulting in a 30-pound weight loss. Ablative fractional laser resurfacing treatments and laser-assisted delivery of triamcinolone in combination and in parallel with physical therapy sessions were initiated. Results: By month five of combination treatment, the patient had objectively decreased scar fibrosis and swelling, decreased facial swelling composite scores, increased range of motion of the neck, and increased right shoulder strength. Subjectively, the patient expressed increased satisfaction in scar appearance, decreased pain, and increased tolerance to activities such as lifting and reaching. Conclusion: Ablative fractional laser resurfacing is a promising adjunct to established interventions for improving functional and cosmetic deficits and quality of life in patients with scar contractures due to its safety profile and efficacy.

Adult-Onset IgA Vasculitis Complicated by Kidney Failure at Disease Onset in a Nepalese Patient: A Case Report

Introduction: Immunoglobulin A (IgA) vasculitis, previously known as Henoch-Schönlein purpura, is an immune complex-mediated small vessel vasculitis primarily affecting children. While rare in adults, it can present with more severe manifestations, particularly involving the kidneys. This case report details the presentation and management of adult-onset IgA vasculitis with significant renal involvement.  Case Report: A 43-year-old male with a history of bipolar disorder presented with facial swelling, shortness of breath, and decreased urine output following an upper respiratory infection. Initial investigations revealed elevated blood pressure and renal impairment. Despite supportive treatment, his condition worsened, leading to a referral to a tertiary care center. He exhibited symptoms consistent with IgA vasculitis, including joint pain, rash, and nephrotic-range proteinuria. The diagnosis was confirmed through a skin biopsy and 24-hour urine collection. The patient was treated with intravenous methylprednisolone, oral prednisone, and an ACE inhibitor. His renal function improved with this regimen.  Discussion: Adult-onset IgA vasculitis can present with severe kidney involvement, including nephrotic-range proteinuria and elevated serum creatinine, which are associated with poorer outcomes compared to pediatric cases. The patient's management, involving glucocorticoids and an ACE inhibitor, aligns with current treatment recommendations for significant renal involvement. Long-term prognosis in adults remains challenging, with a higher risk of end-stage kidney disease compared to children. Vigilant monitoring and tailored treatment strategies are crucial for improving outcomes.  Conclusion: This case underscores the potential severity of adult- onset IgA vasculitis and highlights the importance of early diagnosis and aggressive management to mitigate long- term renal complications. Ongoing research is necessary to refine treatment approaches and enhance outcomes for adults with this condition.