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Background: Child sexual abuse is highly prevalent in India, both among boys and girls; however, few studies have studied sexual abuse of male children. The present case report highlights the challenges of diagnosis and management of a young boy who presented with marked hyperactivity and sexual acting out behaviors subsequent to his repeated exposure to pornography. Clinical Description: H, a 5-year-old boy, presented with a 2 month history of hyperactivity, poor concentration, inappropriate touching, and self-stimulation. H also displayed sexual knowledge beyond that of what would be expected of his age and developmental level. The Child Sexual Behavior Inventory was administered that showed an unusually high score on the (sexual abuse specific items, sexual behaviors that are atypical for child's age and gender). Management and Outcome: The use of therapeutic interventions such as building a therapeutic relationship, environmental change, family counseling, and nondirective play therapy helped in remitting most of the child's behavioral difficulties. Conclusions: Parents need to be active participants in the digital lives of their children and exercise controls on what they view online. Since pediatricians are often the first points of contact for a child with aberrant behaviors, they need to be aware of the law on the protection of children against sexual offenses and the range of types of sexual offenses against children.

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Background: Atypical Mongolian spots are lesions that are aberrant in location (over the abdomen, back, legs, shoulders, or arms), extensive in distribution, persist beyond early infancy, and/or progressively increase in number. These are frequently associated with lysosomal storage disorders or neurocristopathies. Clinical Description: A 4-month-old girl presented with a common cold. Extensive aberrant Mongolian spots over the back, buttocks, lower limbs, and new onset grayish-blue macular spots over the abdomen prompted us to do a detailed evaluation to look for any underlying pathology. Other salient clinical findings included coarse facies, hepatomegaly, absence of significant developmental delay, bilateral macular cherry red spots, and ovoid-shaped vertebral bodies. The clinical phenotype was that of a lysosomal storage disorder possibly GM1 gangliosidosis. This was confirmed by deficient levels of ß-galactosidase. Management: The parents underwent genetic counseling. The infant was enrolled in an early intervention program. By the age of 6 months, the infant had not acquired any new developmental skills and new Mongolian spots did not develop. Subsequently, the family was lost to follow-up. Conclusion: Infants with aberrant Mongolian spots need to undergo an in-depth evaluation to identify underlying systemic disorders, even if they are asymptomatic. Diagnosis helps in providing supportive care to the child, specific therapy (if available) and the option of prenatal diagnosis in subsequent pregnancies.

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Background: Fibroadenoma is the most common breast lesion among pediatric female population. A giant fibroadenoma is rare, characterized by a rapidly growing tumor, with a mass >5 cm diameter in greatest dimension and/or weighing more than 500 gm. Phyllodes tumor range from benign to malignant. Both have similar presentation. Clinical Description: An 11-year-old girl presented with rapidly progressive, painless increase in the size of the left breast over 2 months. Local examination revealed a firm nontender mass involving the upper inner and outer quadrants of the left breast along with the retro-areolar region. The mass was mobile and measured approximately 11 cm × 10 cm. The overlying skin was normal, although with dilated veins and free from underlying mass. The clinical phenotype was suggestive a phyllodes tumor in view of rapid progression and large size. However, the ultrasonogram and fine-needle aspiration cytology favored a fibroadenoma. Management: It was decided to perform a “Nipple-areolar complex sparing lumpectomy” with deferment of reconstructive surgery. Adequate lump excision was achieved while maintaining proper cosmesis. The histopathological findings of the excision biopsy confirmed the final diagnosis of fibroadenoma. Conclusion: A large breast mass in a pediatric/adolescent girl poses a diagnostic dilemma to the treating surgeon. Proper evaluation is needed to differentiate between several benign breast masses from malignant ones. The definitive diagnosis is made histologically. Total excision of the lump with conservation of nipple and areola is indicated to make a definitive diagnosis and to relieve the compression of the normal breast tissue.

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Background: Neurodevelopmental disorders (NDD) are a major global public health problem, particularly affecting children from the lower- and middle-income countries (LMICs). In India, nearly 2.3 million children below 6 years of age have some developmental disability, of whom many live in rural and semi-urban areas with minimum access to early intervention services. We attempted to reach out to such a population at their doorstep with affordable care and management through home-based early intervention (HBEI) programs provided by local field level workers (hitherto referred as community therapy providers [CTP]). A group of local youth, with a short training on NDD and EI methods, have been providing regular, weekly therapy sessions to the afflicted children at the latter's residence, under constant virtual guidance and monitoring by specialists, with the help of a mobile application. The children were initially screened and assessed by our specialist team, who assigned the therapy program and demonstrated the techniques to the CTPs. Clinical Description: We are sharing a series of 8 cases, ranging from cerebral palsy to Global Developmental Delay and speech delay, who have received HBEI for 3–5 months, to demonstrate the impact of the program. Management and Outcome: The children have shown improvement in all domains with the intensive and regular services. Moreover, empathy, concern, and inclusion of parents in therapy sessions rejuvenated the families. Conclusion: Provision of HBEI through field workers may be a cost-effective solution to the formidable problem of childhood disability among the under-privileged rural community. The electronic tracking system has proved very useful in remote monitoring.

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Background: Kikuchi Fujimoto disease (KFD) is a rare, benign self-limited disease characterized by prolonged regional lymphadenopathy associated with or without systemic signs or symptoms. It is a rare diagnosis in children. Due to the lack of pathognomonic clinical symptoms/signs, KFD poses a significant challenge to the clinician. Its diagnosis is confirmed by lymph node biopsy. Clinical Description: A 12.5-year boy presented with chronic cervical lymphadenopathy of 6-week duration, associated with mild-moderate fever, pain, and weight loss. He had raised erythrocyte sedimentation rate, leukopenia, lymphopenia, and thrombocytopenia. Mantoux test was positive. He was managed as a case of tuberculous lymphadenopathy till the lymph node biopsy confirmed the diagnosis of KFD. Management: The child recovered without medications. There has been no recurrence or relapse in 1.5 years of follow-up. Conclusion: This case report highlights the importance of considering the diagnosis of KFD in children presenting with persistent or chronic lymphadenopathy.

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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.

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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.

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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.

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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.

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Background: Mucormycosis is a highly fatal infection that affects immunocompromised individuals. Treatment is difficult and mortality is high when associated with complications. It is rare as a presenting feature of diabetes mellitus (DM) in children. Clinical Description: We describe a child who presented with extensive rhino-orbital invasive mucormycosis and subsequently diagnosed as Type 1 DM. She further developed cavernous sinus thrombosis and internal carotid artery thrombosis known to be associated with very high risk of mortality. Management: Aggressive medical management with posaconazole and liposomal amphotericin B for 12 weeks and early debridement of orbito-cerebral lesions led to complete resolution. Conclusion: Saving children with complicated extensive invasive mucormycosis is possible with aggressive prolonged antifungal therapy and early debridement of lesions.

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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.

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Background: Clinicians must be aware of phenotypic variability in neurofibromatosis type 1 (NF 1) presentations. There is perhaps a limited understanding on progression of NF 1 in prepubertal years and the subsequent threat to vision. Progressively increasing myopia may go unnoticed under a severely ptotic eyelid that gathers attention, due to a disfiguring mass in patients with NF 1. High myopia may result in recalcitrant amblyopia if not tackled early with multidisciplinary management. Clinical Description: A 12 year old girl had a history of progressively increasing left upper eyelid ptosis due to an upper eyelid mass, first noticed at the age of 1 year and eventually resulted in severe ptosis by the age of 5 years. However, this went unnoticed until she was diagnosed with NF 1 at the age of 12 years. Best corrected visual acuity was 6/6 (Plano) in the right eye (OD) and counting finger 2 m with − 15.0 diopter spheres in the left eye (OS). Peripheral fundus examination was normal in both eyes. Levo elevation and abduction were limited OS. Hypotropia, pulsatile proptosis, and depression of the globe were clinically attributable to enlargement of orbital tissues and lid problems. Contrast enhanced computed tomography scan revealed plexiform NF with extraconal extensions. Axial length was 21.94 mm OD and 28.92 mm OS. B scan ultrasound revealed a posterior staphyloma OS. Management: The patient underwent a debulking surgery of the eyelid mass which on histopathological examination confirmed plexiform NF. Surgery resulted in a cosmetic reduction in ptosis; however, any intervention was relatively too late to rehabilitate the left eye. Conclusions: Eye care certainly has its regional differences. High axial myopia may result in low VA and recalcitrant amblyopia that may go unrecognized and comes with management challenges to the attending ophthalmologist and allied specialties dealing with these cases of NF 1.

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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.

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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.

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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.

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Background: Sibling bullying is repetitive aggressive behavior that is motivated by the desire to dominate and incite distress on the victims. It is a hidden epidemic, with nearly 50% of children being victimized sometime in their lives. Since this is associated with adverse consequences for victims and abusers, it requires prompt recognition, evaluation, and management. Clinical Description: We present three families of sibling bullying. Comprehensive individual in-depth interviews (individual and group) were conducted by a team of professionals. The goal was to understand the psychological impact on the children, the family dynamics, and underlying psychosocial issues. Common factors identified were inadvertent positive reinforcement of bullying by parental inability to set limits to the abuser's aggression, failure to model respectful communication, and lack of using appropriate conflict resolution strategies. Management and Outcome: Drama therapy was used as the primary modality of intervention. This involved putting families in hypothetical situations in which they reenacted a bullying incident. Family members played various roles interchangeably in multiple sessions. The role-plays and ensuing discussions created awareness of the adverse effects of sibling bullying on the entire family. This coupled with instruction on positive disciplining and use of conflict resolution and anger management strategies resulted in a gradual decline in bullying. Conclusions: Sibling bullying can be effectively managed by strategies that promote positive interpersonal relationships. Pediatricians need to recognize cases of sibling bullying and consider referral to mental health professionals for evaluation and appropriate management.

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