Background: Severe acute respiratory virus coronavirus-2 infection or coronavirus disease (COVID) is categorized into acute illness and late multiinflammatory syndrome in children (MISC). This has recently been challenged with recognition of presentations with mucocutaneous-enteric symptoms that display considerable overlap between the two. We recognized a similar overlap of manifestations when encountered the three cases of fever associated with unilateral parotitis. Clinical Description: The three patients were of different age groups ranging from 2 months to 7 years of age, all of whom presented with fever, unilateral swelling of face and neck consistent with the region of the parotid gland, and absence of other localizing symptoms or abnormalities on examination. All of them were positive for COVID antibodies, had negative COVID real-time polymerase chain reaction test, did not satisfy the diagnostic criteria of MISC, but had raised inflammatory markers. Since the workup for other common causes of acute parotitis was negative, a clinical diagnosis of post-COVID immune-mediated acute parotitis was kept. Management: All the three patients were managed with systemic steroids (oral or parenteral) and showed complete resolution of symptoms and normalization of laboratory parameters within a few days, a therapeutic response in alignment with an immune-mediated phenomenon. Conclusion: Acute unilateral parotitis with pyrexia may be a hitherto unreported late post-COVID manifestation that is immune mediated and shows an excellent therapeutic response to a short course of steroids.

Background: Foreign bodies inside the oral cavity are commonly encountered among children. One peculiar occurrence is when the tongue gets entrapped in foreign bodies such as bottles. This happens as children often insert their tongues into the bottle and apply oral suction to ingest the last few drops from the bottle. There is a paucity of data in Indian literature and no recommended guidelines for the management of such cases. Clinical Description: The patient, a 12-year-old boy had a history of inability to remove a plastic water bottle sipper from around the tongue while swallowing water quickly. He was initially asymptomatic and then he started developing progressive pain and swelling on the anterior part of the tongue. On examination, the plastic sipper was constricting the tongue circumferentially and the patient felt a sharp pain on maneuvering the bottle. Minimal discoloration of the anterior part of the tongue had set in 1 h after the presentation. Management: Lubrication with 2% lignocaine jelly and ice packs circumferentially followed by attempts of gentle traction and manipulation to remove the foreign body were unsuccessful. Consequently, the patient was shifted to the emergency operation theater where the patient was sedated with intravenous (IV) ketamine and midazolam after securing a nasopharyngeal airway with 100% preoxygenation. An orthopedic bone cutter was used, and a radial cut was given on the impacted end of the bottle, and the constricted part was removed as pressure on the tongue was released, followed by 100% oxygenation with bag and mask ventilation. Conclusion: Immediate intervention in cases of the entrapped tongue can prevent grave consequences such as airway compromise and tongue ischemia and necrosis. Mechanical removal can be done safely using heavy scissors or orthopedic bone under IV sedation after securing the airway in collaboration with the anesthesia team.

Background: Leukemic infiltration of the optic nerve is a neuro-oncologic emergency and also a sign of extramedullary central nervous system relapse. It presents a clinical dilemma in the early stages due to multiple differentials. Patients with leukemia receive radiation and chemotherapy are thus, susceptible to inflammatory, toxic, and infectious causes of optic neuropathy, besides infiltration with tumor cells. Clinical Description: A 15-year-old boy treated for acute lymphoblastic leukemia (ALL) and in remission for 7 months, presented with the unilateral decreased vision for 7 days. A structured evaluation was done, comprising visual acuity, color vision, field of vision, fundus, ophthalmoscopy, ultrasound b-scan, and magnetic resonance imaging of the orbit. The final diagnosis was leukemic infiltration of the optic nerve. Cerebrospinal fluid (CSF) analysis confirmed the presence of atypical lymphocytes. Management: The patient was diagnosed with extramedullary relapse of ALL. Since there are no standard guidelines, a literature review was performed, and the treating team decided to start the patient on stand-alone chemotherapy. Symptomatic resolution became apparent within 10 days. On follow-up, the optic nerve lesion resolved with residual gliosis in the surrounding retina. The CSF has become clear, and the patient is now considered to be in remission. Conclusion: It is important to use a structured clinical approach coupled with investigations to recognize the ocular involvement of ALL (especially in younger patients). There is a need for a regular routine ophthalmological examination in patients in remission for early detection of a relapse. There is a strong felt need for pediatric hemato-oncologists to plan research in this area to generate data so that recommendations for the management of extramedullary relapses are formulated.

Background: Wolcott–Rallison Syndrome (WRS) is a rare autosomal recessive disorder characterized by permanent neonatal diabetes mellitus, skeletal dysplasia, hepatic dysfunction, and other systemic associations. Clinical Description: A 3-month-old infant with a history of fever and poor oral intake presented with severe dehydration, acidosis and 3+ urine ketones and was diagnosed to have sepsis and diabetic ketoacidosis (DKA). He also developed acute kidney injury (AKI) with blood urea 118 mg/dL and serum creatinine 1.5 mg/dL. Management: The child was ventilated, stabilized, and managed for DKA with fluids and insulin as per guidelines. AKI was managed with peritoneal dialysis. Genetic analysis revealed homozygous mutation in eukaryotic translation initiation factor 2-alpha kinase 3 gene consistent with the diagnosis of WRS. A close follow-up was kept with regular screening for other associated manifestations. Central hypothyroidism was detected first followed by skeletal dysplasia and chronic kidney disease. Growth retardation and developmental delay are also present. Conclusion: Neonatal diabetes cases need an early genetic work up and watchful follow-up for the manifestation of other possible associated features.

Background: Superior mesenteric artery (SMA) syndrome or Wilke's syndrome is a rare, atypical cause of upper gastrointestinal tract obstruction. It occurs due to acute angulation between SMA and aorta causing compression of the third part of the duodenum inbetween them leading to proximal intestinal obstruction. Clinical Description: SMA syndrome occurs mostly in patients who have significant weight loss. It occurs mostly due to the loss of the retroperitoneal fat pad, which serves as a cushion for the duodenum protecting it from compression between the two arteries. Causes include prolonged supine bed rest, spine surgery, an unusual high insertion of ligament of Treitz, other causes leading to weight loss, and congenital anatomical defects. Symptoms include weight loss, bilious vomiting, pain abdomen, postprandial nausea, early satiety, and anorexia. We report a 14-year-old female, a known case of pulmonary tuberculosis, who developed pneumothorax followed by acute gastric dilatation superadded with septicemia and upper gastrointestinal tract obstructive symptoms during the hospital course. Management: Detailed history and radio imaging such as barium studies or contrast-enhanced computerized tomography abdomen are required for diagnosis. Treatment includes conservative management such as gastric decompression, maintenance of electrolytes, nutritional rehabilitation via nasojejunal feeding, orally or parenterally. Treatment aims to restore the mesenteric fat pad to increases the angle between the two arteries. If it fails, surgery needs to be planned wherein duodenojejunostomy can be performed. Conclusion: An increased index of suspicion should be kept for this rare syndrome in children, especially in cases associated with significant weight loss. This may lead to timely diagnosis and saving a precious life.

Background: Mastocytosis is a heterogeneous group of disorders that is characterized by excessive proliferation and pathologic accumulation of mast cells in various body tissues. The mast cells also have abnormal morphology and aberrant expression of surface receptors. Clinical Description: A 4-year-old boy was brought with a history of generalized skin lesions since birth and abdominal distension for 3 years. The diagnosis had not been established to date. General physical examination revealed severe acute malnutrition, pallor, dental staining, facial hypertrichosis, polymorphous skin lesions (cicatricial alopecia, diffuse erythema, multiple plaques of variable diameter, skin-colored nodules, and hypertrophic irregular scars), and positive Darier's sign. He also had hepatosplenomegaly. The differentials considered were congenital erythropoietic porphyria, systemic mastocytosis (SM), multifocal Langerhans cell histiocytosis, and linear immunoglobulin A bullous dermatosis. The presence of mast cells on skin biopsy and elevated serum tryptase levels led us to suspect SM and perform bone marrow studies. The diagnosis of “aggressive” SM was initially made on the application of the diagnostic criteria but revised to “smoldering” SM with the emergence of Vitamin B12 deficiency as the probable cause of pancytopenia. Management: Management was planned by a multidisciplinary team: pediatrician, dermatologist, and hematopathologist. The parents were counseled about the nature, natural history, treatment options, and prognosis of the disorder. The child was provided with nutritional rehabilitation and medication for the cutaneous symptoms (selective histamine H1 receptor inverse agonist, H2-receptor antagonist, and application of topical tacrolimus and calamine lotion). Conclusion: The prognosis varies according to subtype. Careful correlation of clinical and laboratory investigations is required when applying the diagnostic criteria for staging.

Background: Severe and/or persistent anemia due to autoimmune hemolytic anemia (AIHA) secondary to vivax malaria is a rare association. Very few cases are reported worldwide, and even less from India. AIHA occurs when immunoglobulins and/or complements target red blood cell surface antigens resulting in hemolysis. Awareness of this complication helps in early recognition, investigation, and prompt initiation of treatment. Clinical Description: A 15-year-old boy presented with a history of high fever associated with chills for 2 days. Examination revealed the presence of severe pallor and icterus. Systemic examination was unremarkable. Investigations showed anemia (Hb: 3.6 g/dl) with reticulocytosis (6.6%), malaria (trophozoites of Plasmodium vivax and antigen test positive), and indirect evidence of hemolysis (indirect hyperbilirubinemia and elevated lactate dehydrogenase of 668 U/l). Management and Outcome: Treatment was started with intravenous artesunate and packed cells transfusion was planned. Cross-matching showed autoantibodies which raised suspicion of AIHA. Coomb's test was positive. Least incompatible packed red blood cells were transfused. A repeat peripheral smear was negative for malaria. Posttransfusion, the Hb levels rose to 8.3 g/dl before falling to 5.3 g/dl over the next 2 days. The direct antiglobulin test was positive. High doses of oral prednisolone resulted in progressive improvement in Hb levels to 8 g/dl within a week. He was discharged on oral prednisolone with daily oral folic acid supplements. The steroids were tapered when Hb reached 10.6 g/dl and continued for 3 months. Conclusion: If a patient with malaria (falciparum or vivax) exhibits persistent severe anemia, indirect evidence of hemolysis is found on investigations and/or autoantibodies identified on cross-matching, AIHA should be suspected and investigated accordingly.

Background: Cutaneous tuberculosis (TB) is a rare disease seen by the pediatrician on an outpatient basis. It has a varied presentation and is classified on the basis of the source of infection and host's immune response to mycobacteria. Lupus vulgaris (LV) is a paucibacillary manifestation of cutaneous TB. It can mimic other infectious skin diseases such as TB verrucosa cutis and chromoblastomycosis. Clinical Description: We hereby present a case report of an adolescent female with a serpiginous, nodular, and warty hyperpigmented skin lesion over her buttock. The lesion had started following incidental injury 7 years back as a papule and continued to expand despite multiple medications. Management: A skin biopsy was done which was suggestive of cutaneous TB, but the absence of systemic features confounded the diagnostic type. She was finally diagnosed as having LV after a detailed review with a dermatologist and pathologist. The patient responded well to antitubercular treatment. Conclusion: Although cutaneous tuberculosis is well described, it is often not recognized by the primary care physician. Diagnostic dilemmas may arise due to clinical-histopathological mismatch.

Background: Chylothorax is defined as the accumulation of chyle in the pleural space and is characterized by elevated triglyceride content in pleural fluid >110 mg/dl. Chylous ascites is the accumulation of chyle in peritoneal fluid characterized by triglyceride levels >200 mg/dL. Causes can be traumatic or nontraumatic; portal or nonportal; and congenital or acquired (inflammatory, postoperative, malignant, or infectious). Lymph duct abnormalities are a rare cause of concurrent occurrence of chylothorax with chylous ascites. Clinical Description: We report a 4½-year-old girl who initially presented a fever and was diagnosed to have right-sided pleural effusion. Since no other focus was identified, she was treated as a case of tubercular pleural effusion, following which her symptoms settled transiently. She thereafter had a recurrence of her symptoms in association with ascites and was managed as a case of relapse of tuberculosis (TB) with category 2 anti-tubercular therapy. Since her symptoms did not resolve, she was referred to our hospital for further management. Management: Clinical reasoning based on history and examination coupled with investigations ruled out cardiac, renal, hepatic, malabsorption, or nutritional pathologies of recurrent effusions. TB was ruled out. A lymphatic malformation was suspected. Therapeutic cum diagnostic paracentesis was done in a fed state, which indicated a chylous nature by the milky appearance and suggestive cytology and biochemistry. Lymphoscintigraphy confirmed the presence of a lymphatic duct abnormality. The child was managed with diet modifications, following which she improved within a week. Conclusions: The approach to recurrent effusions without any focus should be logical and sequential, as described above, to exclude the aforementioned common conditions. If workup for TB is repeatedly negative other less likely causes should be considered.

Background: Recurrent pneumonia is defined as at least two episodes of pneumonia in a year or three episodes during a lifetime, with clinical and radiological improvement in between. Clinical Description: A 5-year and 8-month-old boy presented with a history of three episodes of fever and fast breathing of variable duration over 8 months. In between, he had a persistent moist cough with intermittent fever, weight loss, and darkening pigmentation. He had three prior admissions for the same, with X-rays showing multilobar pneumonia. Routine investigations were normal. Tuberculosis workup was negative. Computerized tomography showed changes in consolidation without any evidence of structural abnormality. The child became asymptomatic with short courses of antibiotics and nebulization during these admissions. He was referred to us for further evaluation, and we reviewed his history, examination, and medical records. Management: The darkened complexion was suggestive of Addisonian pigmentation, but serum electrolytes were normal. However, very low levels of morning cortisol and high adrenocorticotrophic hormone were suggestive of adrenal insufficiency. Retrospective history revealed dysphagia with nocturnal cough suggesting aspiration. Barium swallow confirmed achalasia by the presence of a dilated esophagus with distal narrowing. The clinical phenotype was suggestive of Triple A (AAA) syndrome with Addison's disease, alacrimia, and achalasia. A positive Schirmer's test confirmed alacrimia and established the clinical diagnosis. He was started on replacement hydrocortisone and later taken up for Laparoscopic Heller Myotomy with fundoplication. On follow-up, his appetite improved, his cough subsided, he had adequate weight gain, and the pigmentation had decreased. Conclusion: Achalasia should be considered a differential in recurrent pneumonia. AAA syndrome has isolated glucocorticoid deficiency. Therefore, hyperpigmentation in the presence of normal electrolytes should not preclude considering the possibility of adrenal insufficiency.

Background: Acute hemorrhagic edema of infancy (AHEI) is an immune complex-mediated leukocytoclastic vasculitis. Despite its worrisome appearance, it has a benign disease course with rare systemic involvement. Clinical Description: A 25-month-old male patient was brought to the pediatric outpatient clinic with a pink oval-shaped plaque-like rash all over the body and edema on the lower extremities and left auricle. The child was diagnosed as a case of coronavirus disease 2019 (COVID-19) 5 days before the present complaints. History and physical examination were otherwise unremarkable. Because of the patient's age, the purpuric appearance of lesions, distribution pattern of the rashes, localized edema, and no end-organ involvement, a possibility of AHEI, triggered by COVID-19, was considered and the patient was evaluated for the same. Management and Conclusions: The patient recovered in 15 days with no end-organ involvement. He was advised to continue regular follow-ups to look for long-term complications. AHEI is a benign condition which may occur in children following COVID-19 infection. It is essential to recognize the condition to avoid unnecessary investigations and treatment.

Background: Neonatal purpura fulminans (PF) is a rare disorder characterized by the formation of dermal microvascular thrombosis associated with disseminated intravascular coagulation (DIC). It can be caused by inherited protein C or protein S deficiency or severe sepsis with DIC due to organisms such as Streptococcus pneumoniae and Gram-negative bacteria. Clinical Description: A preterm boy of 31-week gestation and weighing 1480 g was delivered by cesarean section. There were no risk factors for sepsis. He presented with respiratory distress after birth, was shifted to the neonatal intensive care unit (NICU), was diagnosed as respiratory distress syndrome, and was managed as per standard protocol. Management: On the 6^th day of life, the neonate developed pulmonary hemorrhage, multiple purpura on his upper and lower extremities, and shock. Raised D-dimer (>400 ng/ml), increased prothrombin and activated partial thromboplastin time, and thrombocytopenia (6000/μL) were indicative of DIC. The blood culture isolated Elizabethkingia meningoseptica. Meningitis was ruled out. Supportive care included fresh frozen plasma and platelet transfusion, antibiotics as per drug sensitivity, and granulocyte colony-stimulating factor. The baby improved and the lesions healed with scarring. Protein S and protein C deficiency was excluded on follow-up. On follow-up, at corrected age of 6 months, the baby was developmentally normal. Three additional cases were identified in the unit around the same time, however outbreak investigation could not identify origin of the pathogen. Conclusion: We could not find any earlier publications of neonatal PF due to E. meningoseptica septicemia. This organism is a cause of sepsis and meningitis in preterm babies and outbreaks in NICU settings. Early identification, meticulous assessment, and prompt specific antimicrobial treatment are important for survival.

Background: Scrub typhus is a mite-borne infection caused by the bacterium, Orientia tsutsugamushi. It is re-emerging in many parts of South East Asia, particularly in rural India. Although no age group is immune to this infection, scrub typhus in neonates is rarely suspected and reported. Here, we report a neonate with scrub typhus who was initially misdiagnosed as “late-onset neonatal sepsis.” Clinical Description: A 26-day-old exclusively breastfed infant presented with fever, vomiting, loose stools, abdominal distension, and refusal of feeds for 3 days. Examination revealed an irritable, febrile, and pale infant. She had tachycardia and facial puffiness. On abdominal examination, generalized distension with hepatosplenomegaly was noted. Blood investigations were suggestive of lymphocytic leukocytosis, thrombocytopenia, toxic granules in peripheral smear, and elevated C-reactive protein. Management: The infant was promptly started on empirical antibiotics for “late-onset sepsis.” However, in view of poor response, other possible differential diagnoses were considered. Careful reexamination revealed a necrotic ulcer covered by a yellow scab with erythematous rim on the left lower eyelid. Based on the clinical presentation and an eschar-like lesion, scrub typhus was suspected. The neonate was started on oral azithromycin and immunoglobulin M (IgM) enzyme-linked immunosorbent assay (ELISA) testing for scrub typhus came back positive. Fever subsided immediately within 48 h and the infant was discharged after 7 days. Conclusion: Acute febrile illness due to scrub typhus can affect newborns. A high index of suspicion is required for early diagnosis. Timely treatment leads to prompt clinical response and reduced complications.