Background: Factor XIII deficiency is a rare disorder that may be congenital or acquired and is considered if a coagulation disorder is suspected, but the initial laboratory investigations are normal. Clinical Description: A 10-year-old boy presented with a scrotal swelling and pain following minor trauma. Local examination showed redness of overlying scrotal skin with tenderness on palpation. Management: Initial laboratory investigations for coagulation (prothrombin time/activated partial thromboplastin time/thrombin time) were normal. The diagnosis of factor XIII deficiency was clinched by abnormal clot solubility test. Child was treated with fresh-frozen plasma. Conclusion: Scrotal hematoma is a rare site for bleeding in factor XIII deficiency, which must be suspected if clinical suspicion of coagulation disorder is high and initial laboratory tests for coagulation are normal.

Background: Bell's palsy is the most common form of facial paralysis in children. Etiology of facial paralysis can be both congenital and acquired. Acquired facial paralysis in most cases is due to viral infections. Hepatitis C infection leading to Bell's palsy is an unusual complication which has not been reported until now. Clinical Description and Management: A 4 month's old male child was admitted with fever, vomiting, refusal to feed, and icterus. His investigations revealed anemia with neutrophilic leukocytosis and deranged liver function tests. Hepatitis C antibody test was reactive. On 5^th day of admission, right-sided Bell's palsy was noticed. There were no other signs of neurological deficit. Parental testing revealed hepatitis C antibodies in mother. The child was managed conservatively. He responded to treatment and symptoms subsided within 1 week of its onset. Conclusion: This case report highlights an unusual complication of hepatitis C infection in children. Prognosis in this case was good as the child recovered spontaneously.

Background: Henoch–Schönlein purpura (HSP) is the most common vasculitis seen in children that rarely involve central nervous system (CNS) (0.65%–8%). Patients with CNS vasculitis may present with symptoms such as headache, seizures, focal neurological deficit, or behavior abnormalities. We describe a case of HSP where the patient presented with developmental delay along with skin rash and renal involvement. Clinical Description: A 15-month-old boy presented with a history of developmental delay and skin rashes for the past 2 months. There was no significant antenatal or birth history. At the age of 15 months, the child could not sit without support, could not transfer objects from one hand to other, could only speak bisyllables, and could not wave “bye-bye.” On examination, the patient had normal vitals with equal volume peripheral pulses and normal blood pressure in all four limbs. Urine examination showed microscopic hematuria, and kidney functions were mildly deranged. Skin biopsy showed immunoglobulin A vasculitis. Magnetic resonance imaging brain was done which revealed multifocal areas of altered signal intensity in periventricular deep white matter in bilateral frontal and parieto-occipital lobes, appearing hyperintense on T2-weighted and fluid-attenuated inversion recovery sequences, showing evidence of diffusion restriction, suggestive of areas of acute ischemia. The findings were consistent with manifestations of CNS vasculitis. Management: The patient was managed conservatively and advised physiotherapy and follow-up for developmental delay. Conclusion: HSP can rarely involve CNS, however, developmental delay in HSP has never been reported before.

Background: Cerebral venous sinus thrombosis (CSVT) in children is a rare, obscure, but potentially fatal problem. The clinical presentation is varied, as is the multiple underlying causes. CSVT can be seen in patients with inherited or acquired prothrombotic risk factors, even in the absence of an underlying condition. The respective etiological roles of methylenetetrahydrofolate reductase (MTHFR) CG677T mutation and hyperhomocysteinemia in CSVT is still not clear. We present a case of CSVT following a minor head injury, in whom an MTHFR mutation was identified, but with initial normal homocysteine levels. Clinical Description: A 9-year-old boy presented with nonaccidental fall followed by persistent headache, projectile vomiting, bradycardia and hypertension suggesting features of raised intracranial pressure. Brain imaging (magnetic resonance venography) showed extensive dural venous sinus thrombosis. Prothrombotic workup revealed heterozygous CG677T polymorphism of the MTHFR gene with normal serum homocysteine, B12 and folate levels. Management: The child was given supportive management. Low-molecular-weight heparin was initiated followed by long-term warfarin. There were no neurological deficits at discharge. Six months afterward, there is persistence of thrombosis with partial recanalization in the affected cerebral sinuses. The serum homocysteine level is now marginally elevated. Conclusion: Available neuroimaging should be promptly instituted to establish CSVT. A targeted search for prothombotic risk factors should be undertaken but within the proper timeframe. Genetic mutations may be identified accurately in the acute phase, but other factors should be done after 4–6 weeks. The relationship between MTHFR polymorphisms and hyperhomocysteinemia with venous thrombosis is yet to be defined.

Background: Congenital heart disease (CHD) is the most common of all birth defects. Interrupted aortic arch (IAA) is rare, attributing to 1% of all CHD and exhibits left sidedness in most cases. It usually presents in the first 2 weeks of life with heart failure and shock. Early recognition, preoperative stabilization with prostaglandin infusion, and timely surgery are necessary to prevent mortality. Single-stage repair has proven to have good outcomes. However, resource constraints in low- and middle-income countries can hinder diagnosis and intervention by single-stage repair due to a paucity of state of the art centers and inventory. Case Description: We present the experience of a low-resource center with two children with rare complex forms of IAA, both of whom underwent successful single-stage cardiac surgeries; a 6-week-old infant was admitted with difficulty in feeding and heart failure signs. Moreover, a 2-year-old boy was referred with failure to thrive, chronic respiratory problems, and feeding problems since early infancy. Both children were diagnosed with IAA with associated defects including patent ductus arteriosus by transthoracic echocardiography. Management: After stabilization with anticongestive medication, single-stage repair was attempted in both. The postoperative course was uneventful, and the postsurgical echocardiogram showed no residual defect. Both cases were discharged. They are under follow-up, are symptom free, have gained weight, and have not shown any need for reintervention yet. Conclusion: If preparative diagnosis is made correctly and surgery planned meticulously, the immediate and short-term outcome of single-stage repair can be favorable.

Background: Neurological manifestations following dengue fever are increasingly reported and include encephalopathy, encephalitis, neuro-ophthalmic manifestations or Guillain–Barre syndrome-like presentation. Herein, we expand the clinical spectrum of this neurological repertoire by describing two patients presenting with acute necrotizing encephalopathy of childhood (ANEC) following dengue fever. Clinical Description: Both patients presented with features of acute febrile encephalopathy following an acute febrile illness that proved to be dengue fever. Laboratory investigations revealed thrombocytopenia (patient 1), transaminitis and normal findings on cerebrospinal fluid analysis (patient 1 and 2). Both the patients tested positive for NS1 antigen and dengue serology. Neuroimaging revealed bilateral symmetrical thalamic lesions with cerebellar, midbrain, and cortical white matter lesions suggestive of ANEC. Management: Patient 1 received supportive treatment while patient 2 received pulse methylprednisolone therapy along with supportive treatment. Both the patients had complete clinic-radiological recovery. Conclusion: ANEC following dengue fever may have a relatively benign course, besides the fulminant course that has been described earlier in a single case.

Background: Paraneoplastic glomerulonephritis (GN) are defined as glomerular lesions not directly related to tumor burden, invasion, or metastasis but induced by products of tumor cells. They are usually associated with solid tumors of gastrointestinal tract and hematological malignancies. Nephrotic syndrome is a rare manifestation of paraneoplastic syndromes, especially in children and biopsy findings are usually those of minimal change disease (MCD) or membranous nephropathy. We describe a case of steroid-resistant nephrotic syndrome with focal nodular hyperplasia (FNH) of liver. To the best of our knowledge, this has not been reported previously. Clinical Description: A 9-year-old boy presented with features of nephrotic syndrome and had a significant hepatomegaly. Based on radiological features and histopathological confirmation, the child was diagnosed to have a hepatic tumor consistent with a diagnosis of FNH, and the renal biopsy was suggestive of MCD. Management: As FNH did not require a surgical intervention, the nephrotic syndrome was managed with prednisolone initially, but the patient failed to achieve remission and was subsequently started on oral tacrolimus with enalapril and low-dose alternate day prednisolone. Remission of proteinuria was achieved within 2 months, and the child has been in follow-up for the past 18 months and the liver mass has been stable. Conclusion: This report suggests a possible paraneoplastic GN (steroid-resistant nephrotic syndrome) with FNH of liver that responded to immunosuppression.

Background: Syndromes in which some manifestations occur later in life are difficult to diagnose in childhood. A systematic clinical approach may prove to be extremely useful. Gorlin syndrome (GS) is characterized by jaw keratocysts and/or basal cell carcinomas usually appearing in the second decade of life. Clinical Description: A 9-month-old girl, second born to a nonconsanguineous couple, presented with delay in attaining milestones and spinal deformities. There was no significant antenatal, perinatal, neonatal, or family history. She had no neck control, vocalization, or social smile. Fixation and tracking of objects and startle response to sound was present. Salient examination findings were macrocephaly, frontoparietal bossing, flat occiput, hypertelorism, broad nasal bridge, right preauricular pit, palmar pitting, and kyphoscoliosis. X-ray chest revealed bifid vertebra, hemivertebrae, and bifid ribs. Echocardiogram, ultrasound abdomen, magnetic resonance imaging brain, and brainstem-evoked response audiometry were normal. Management: A systematic clinical approach was used that included the following steps: (1) history, examination, and identification of overt anomalies; (2) searching for concealed anomalies; (3) synthesizing information; (4) literature/database search using good search handles; (5) critically analyzing the differential diagnoses; and (6) surveillance for associated morbidities of established diagnosis. We arrived at a clinical diagnosis of GS after applying diagnostic criteria and excluding differential diagnoses. Appropriate intervention was started. The child is under annual follow-up. Conclusion: A systematic clinical approach including an effective search strategy with good handles helps in arriving at a clinical diagnosis in children with dysmorphism.

Background: Hypoparathyroidism is a condition characterized by hypocalcemia and hyperphosphatemia due to insufficient secretion of parathyroid hormone (PTH). The common causes of hypoparathyroidism are postsurgical and autoimmune. We describe a case of hypoparathyroidism precipitated by mumps. Clinical Description: A 17-year-old boy was brought to us with altered sensorium following 2 episodes of generalized tonic-clonic seizures within the last 4 h. He required mechanical ventilation, but severe laryngospasm made intubation difficult. He had been suffering from a febrile illness that appeared to be mumps for the preceding 4 days. The only salient biochemical abnormality was hypocalcemia and hyperphosphatemia. Neuroimaging and cerebrospinal fluid examination were normal. Hypoparathyroidism was suspected and confirmed by low PTH levels. He also developed secondary hypothyroidism. Management and Outcome: The child had no symptoms of hypocalcemia in the past. He was started on intravenous calcium and calcitriol. He showed dramatic improvement in sensorium with normalization of serum calcium levels. He was extubated after a week. At discharge, there were no neurological sequelae and the sick euthyroid syndrome had resolved. Viral serology for mumps was reactive by the 3^rd week of illness. Conclusion: Although viral infections have been described as triggers for other endocrinopathies, we could not find any association with hypoparathyroidism. An exhaustive literature search we could not find any similar case reports of hypoparathyroidism during or following mumps. Whether mumps infection had any role in the pathogenesis of the hypoparathyroidism or only unmasked an underlying endocrinopathy remains uncertain.

Background: Inferior vena cava (IVC) malformations are rare developmental anomalies and are usually asymptomatic. Agenesis of IVC is an uncommon form of IVC malformation, and symptomatic neonatal presentation is unusual. We describe an unusual case of symptomatic venous thrombosis in the neonatal period after central venous cannulation, which was later found to have agenesis of IVC. Clinical Description: An extreme preterm (28 weeks) baby developed deep vein thrombosis of the femoral and external iliac vein after femoral venous catheterization and required anticoagulation therapy. Later, during the hospital stay, he was noted to have developed dilated abdominal veins over the flanks and paraspinal region, with the direction of flow below upward 2 months after the resolution of the thrombus. Management and Outcome: Ultrasonography (USG) color Doppler ruled out portal hypertension or persistence of IVC obstruction. Computed tomography (CT) venography resolved it to be the case of agenesis of the infrarenal portion of IVC. No consensus guidelines exist for neonatal management and it is usually conservative. Conclusion: IVC developmental anomalies including agenesis of IVC are very rare and are usually an asymptomatic condition, but can cause symptoms due to venous congestion in the lower limb or pressure from enlarged collaterals and rarely associated with deep venous thrombosis usually seen in young adults. Neonatal presentation is extremely rare. Diagnosis is by USG color Doppler and CT venography. Neonatal management guidelines are lacking and the risk of anticoagulation must be cautiously weighed against the risk of hemorrhage.

Background: Teratomas are totipotent tumors commonly seen in gonadal and extragonadal region. Gastric teratomas are very rare and account for <1% of the cases. Conventionally, the treatment of gastric teratoma is open excision of the tumor. We present a rare variant of a gastric teratoma managed laparoscopically. Clinical Description: An 8 months' old male was noted to have pallor and history of blood-tinged spit-ups. The child was worked up for the cause of anemia with an upper gastrointestinal disorder as the first differential. The child had significant anemia with a hemoglobin level of 6 g/dl and was transfused packed red blood cells sequential imaging with an abdominal ultrasound, contrast-enhanced computerized tomographic scan, and magnetic resonance imaging identified the presence of a gastric teratoma with both an intragastric and extragastric component (dumbbell variant). He was referred to the pediatric surgery services for further management. Management: The infant underwent a gastroscopy to further define the intragastric component of the mass. This revealed a sessile submucosal polypoidal lesion with multiple areas of erosions in the region of the fundus. A biopsy from this suggested a teratoma. The child underwent a total laparoscopic excision of the lesion and recovered well. The histopathology of the tumor was consistent with a mature cystic teratoma (Grade 0). The postoperative period was uneventful. Conclusion: Laparoscopic complete surgical excision of the dumbbell variant of gastric teratoma in this child was curative, reduced morbidity, and hospital stay compared to usual open surgery.

Background: Internal hernias involve prolapse of any viscus through normal or abnormal mesenteric or peritoneal aperture and constitute <1% of all cases of hernia. Pericecal hernia is one form of internal hernia rarely reported in the pediatric population. Clinical Description: We describe the case of a 10-year-old boy with underlying tetralogy of Fallot, who presented with third episode of severe abdominal pain, requiring hospital admission with tenderness in the umbilical and right iliac quadrants, voluntary guarding but no organomegaly or abdominal distension, and preserved bowel sounds. Management: Baseline investigations (complete blood count, C-reactive protein, erythrocyte sedimentation rate, blood culture sensitivity, and stool and urine microscopy) and serum amylase and lipase were normal. Ultrasound showed mesenteric lymphadenopathy but no pancreatitis or appendicitis. Computed tomography (CT) demonstrated circumferential wall thickening in distal ileum with clustering of distal ileal loops, posterior and lateral to the cecum, extending into right paracolic gutter, suggestive of pericecal hernia. Child received conservative management and improved. Definitive surgical reduction was deferred due to negative parental consent. Conclusion: To conclude, internal abdominal hernia is rare in children with nonspecific clinical presentation. CT should be considered when underlying etiology of acute abdomen is not found on the basis of history, examination, and baseline investigative workup. Early intervention helps in reducing the morbidity and mortality to great extent.

Background: Air leak syndrome (ALS) can be seen in sick neonates on positive pressure ventilation. Tension pneumopericardium is uncommon, but life-threatening if not promptly identified and treated. Needle decompression can be life-saving. Recurrent tension pneumopericardium is difficult to treat and ideally indicates the placement of a pericardial drain under fluoroscopic guidance. We describe an alternative technique of percutaneous drain placement using intrapericardial saline infusion when the transport of the neonate to a catherization laboratory was not feasible. Clinical Description: A neonate with hyaline membrane disease and persistent pulmonary hypertension of new-born on high-frequency mechanical ventilation developed recurrent tension pneumopericardium that caused hemodynamic compromise. Management: Emergency needle decompression was performed twice. Further recurrence necessitated pericardial drain placement under echocardiographic guidance. Rapid exit of air from the pericardial cavity made advancement of the guidewire challenging. A small amount of sterile saline was injected in the pericardial space to assist advancement following which placement of the pericardial drain was successful. Subsequently, the neonate had an uneventful course and was discharged after 3 weeks. Conclusion: Prompt recognition and management of ALS can be life-saving. Urgent needle decompression and pericardial drain placement can be performed under fluoroscopic (ideally) or echocardiographic guidance by trained personnel. Instillation of saline in the pericardial space may assist with pericardial drain placement.

Background: Child sexual abuse (CSA) is highly prevalent in India and a major public health challenge. CSA victims are at a greater risk for developing a range of negative behavioral and psychological outcomes across their life span. In this case report, we highlight the diagnosis and management of a boy who sexually victimized his classmate subsequent to his trauma of being sexually abused by a peer. Clinical Description: A 10-year-old child, was referred for sexual acting out behaviors, wherein he had attempted anal penetration of a classmate. History revealed multiple stressors including the death of his mother, history of sexual abuse by an older boy, prolonged exposure to digital sexualized content, father's remarriage, adjustment problems with stepmother, relocation, and a new school. A comprehensive psychosocial evaluation was conducted including the administration of a risk assessment tool, the Juvenile Sex Offender Assessment Protocol-II. Management: The mainstay of management was psycho-education that focused on guiding his parents on the importance of intensive parental supervision, academic support, restricting the use of the Internet, and promoting healthy friendships. The child was encouraged to have healthy extracurricular interests, especially outdoor physical activities. Regular follow-up for over a year revealed significant behavioral improvement and no repeat of sexual offending behavior. Conclusion: High-risk children need to be provided with supportive family services and linked to child welfare organizations for long-term intervention. Health-care professionals can play a pivotal role in the prevention of inappropriate sexual behaviors and the promotion of healthy development among youth.

Background: Thrombotic thrombocytopenic purpura (TTP) is a rare disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, acute renal failure, and central nervous system impairment. Clinical Description: Although most cases of TTP are idiopathic, there are some case reports describing its association with certain infections. We report a rare case of TTP associated with tuberculosis which was confirmed by ADAMTS13 levels. Management: This child was managed with high-dose plasma infusion. Conclusion: To the best of our knowledge, this is the first case in a pediatric patient with this association and treated successfully by high-dose plasma infusion.

Background: Congenital long QT syndrome (cLQTS) are heritable disorders due to genetic mutations causing prolonged corrected QT (QTc) interval that may result in fatal arrhythmias. Clinical Description: A well 20-day-old, exclusively breastfed boy had an episode of unresponsiveness with no other symptoms. There was a history of four previous unexplained infantile deaths after similar complaints. General and systemic examination was normal. The differential diagnoses were neonatal apnea, seizures, and inborn errors of metabolism. Management: Sepsis, hypoglycemia, and electrolyte imbalance were ruled out. Electrocardiography (ECG), chest radiograph, and echocardiograph were normal. Ultrasonogram of the cranium ruled out structural abnormality and bleed. Electroencephalogram was normal. First-line metabolic investigations were normal. On day 28 of life, he had a recurrent episode of apnea. ECG evaluation during this episode revealed a prolonged QTc interval. Suspecting cLQTS, Trio Whole Exome Sequencing for mutations in the cLQTS susceptibility genes was performed in the proband and parents. A heterozygous variation, c.290C>T; p.Thr97Met in TRPM4 was identified in the symptomatic neonate and asymptomatic mother, suggesting autosomal dominant inheritance. The baby was started on oral propranolol, but succumbed at 8 weeks. The mother was referred for cardiac management and parents counseled about possible prenatal diagnosis in subsequent pregnancies. Conclusion: cLQTS should be suspected in neonatal apnea, when no other cause is appreciable. A normal ECG does not exclude cLQTS. If there is a strong suspicion, repeat ECGs and appropriate genetic testing should be done. Patients should be managed according to standard guidelines.

Background: Children with acute febrile respiratory illnesses are commonly seen in the emergency department. Apart from the initial stabilization, they need to be assessed for the underlying cause, and clinicians should formulate differential diagnoses to start treatment. Radiological investigations including x-ray and ultrasonography are often used to narrow down the initial possibilities based on history and examination. A child presenting with an opaque hemithorax is one such scenario. Clinical Description: A 3-year-old boy presented with a short duration of fever, cough and respiratory distress. Examination showed rightward mediastinal shift with dull percussion note and reduced breath sounds over the left hemithorax, along with congested neck veins, stridor and left sided wheeze. Chest x-ray showed an opacified left hemithorax and ultrasonography suggested moderate pleural effusion. Management: Complicated pneumonia with parapneumonic effusion (or empyema) was considered, but a diagnostic pleural tap was dry. The possibilities were revised to include cystic space occupying lesions with mediastinal compression. Computed tomography of the chest confirmed a mass lesion with predominantly cystic components. The patient underwent surgical excision, and histopathology showed pleuropulmonary blastoma. Conclusion: Although respiratory infections are common in childhood, clinical evaluation and judicious use of imaging modalities can uncover less common conditions. Although bedside ultrasonography is a handy tool in the emergency department, it is not infallible.