SUPERFICIAL SOFT TISSUE SWELLING IN PAEDIATRICS.pptx

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1. SUPERFICIAL SOFT TISSUE SWELLING IN PAEDIATRICS by dr amanfo benaiah

2. CASE STUDY 10yr old boy who was referred from A shaman hospital on account of a painful swelling at the perineum for the past 2 weeks which was associated with pain upon sitting walking. The swelling was noted 8yrs ago at the perineum about the size of a pea but since increased in size over the past 8yrs extending into the scrotum with no associated symptoms up until 2 weeks prior to presentation. Child was seen at korle bu when the swelling was noted at 2yrs with investigation ordered but defaulted. Mother however noted prominent vasculature on the child’s right thigh his football matches or running and was not associated with symptom hence reluctance to follow up until 2 weeks prior to presentation Odq:fever -, cough-, trauma- , dysuria-,abdominal pain-,hematuria-,constipation-

3. Hx continued Past medical history: no previous admission, no hx of blood transfusion or surgery,no hx of asthma, hypertension,scd Drug hx : used otc painkillers, no hx of herbal medication use,no known food or drug allergies Pregnancy and birth history: unremarkable Immunization hx : up to date bcg scar noted deltoid area of right upper arm Family hx : 2 nd of 3 children to 41yr old teacher{father) and 35yr old petty trader(mother)

4. Hx continued Social hx : he is a primary school pupil, lives at ashaman with family, active health insurance, accepted blood transfusion On examination, young boy lying in bed in mild discomfort with hand over genitalia, mildly pale, afebrile(36.3 ),anicteric, not in any respiratory distress, hydration satisfactory Respiratory system: air entry adequate bilaterally with vesicular breath sound Respiratory rate: 18cpm

5. Hx continued Cardiovascular system: heart sounds S1+S2+0 pulse 90pm regular and of good volume Abdomen : soft full, moves with respiration, not distended, mild tenderness at the supra pubic region however bladder not palpable, a 4*3 cm reducible umbilical hernia noted ,inguinal lymphadenopathy noted(2.5cm non tender , not matted) A soft mass about 8* 5 cm mass noted at perineal raphe extending into distal third of the scrotum which was firm, warm to touch. No ulceration , soft and tender,not fluctuant. Both testes palpable with visibly prominent vasculature into the mass inferiorly otherwise normal looking external male genitalia

6. continued Dre : good anal hygiene,good sphincter tone, no masses palpable ,examining finger stained with stool Central nervous system: conscious and alert, ptosis of the right eye, normal tone and reflexes Impression: PERINEAL MASS ddx : hemangioma lyphangioma teratoma perineal ascess

7. Plan Admit Fbc , gxm , bue & cr , hb electrophoresis Usg Mri paracetamol Prep for surgery pending mri report

8. Usg presented on admission(10/06/24) –soft tissue malignancy Mri report

9. Intra –op findings are as follows Tortuous highly vascular 8*6cm mass with feeding vessels from the right ischiorectal region and tributaries from peribulbar urethra area Ebl-450ml Child was hemotransfused . Child currently doing on ward.

10. HEMANGIOMA

11. CONTINUED H emangiomas are benign vascular neoplasms that have a characteristic clinical course marked by early proliferation and followed by spontaneous involution. Hemangiomas are the most common tumors of infancy Infantile hemangiomas may be cutaneous or extracutaneous : Head and neck - 60% Trunk - 25% Extremities - 15

12. EPIDEMIOLOGY Hemangioma appears in neonatal period , usually within the first 2 weeks. Deep subcutaneous tumors or visceral hemangioma may not manifest until 2 to 3 months of life. Approximately 30% to 40% of hemangiomas are nascent at birth, presenting as a premonitory cutaneous mark—a barely visible pale area, telangiectatic , or macular red stain, or an ecchymotic spot. Approximately 80% of hemangiomas are solitary; 20% are multifocal Hemangioma is more common in females than males. The incidence is 10% to 12% in white infants and 23% in preterm infants who weigh <1,000 g. The frequency is lower in dark-skinned infants

13. EVOLUTIOI OF HEMANGIOMA Proliferating phase- 6 – 8months of infancy Involuting phase-reaches peak before first year. Continues until 5 to 10yr Involuted phase-regression complete in 50% children by age 5. 70% by age 7.

14. EVOLUTION OF HEMANGIOMA

15. Haemangiomas are either capillary ( portwine ) or cavern- (strawberry). Common in the head and neck The skin over a haemangioma is usually warm. Very rarely, present as pigmentary problems in Negroes. in Caucasians they present as reddish or purplish patches of the skin which blanch when compressed. They are not raised above the skin surface as they are intradermal, but the area may become large and the skin redundant.

16. Cavernous haemangiomas which may include arteriovenous communications can occur anywhere. They are elevated above the skin surface and may or may not be emptied by pressure. They are well-defined, lobulated, mulberry-like and of dark red or purple colour . They are honey-combs of large unequal spaces filled with blood and separated by fibrous strands. They may also involve deeper tissues such as muscle bone nervous system

17. SYNDROMES ASSOCIATED HEMANGIOMAS Sturge -Weber syndrome- ipsilateral glaucoma, intracranial haemangioma and focal epilepsy with varying degrees of mental retardation Von Hippel -Landau disease of associated cerebellar haemangioma , glaucoma and pancreatic disease may also occur. So are Maffucci's syndrome of haemagiomas associated with dyschondroplasia Kassabach-Mersitt ' s syndrome in which platelets are trapped and consumptive coagulopathy ensues associated with large cavernous

18. LUMBAR syndrome (Lower body IH and other cutaneous defects, Urogenital anomalies and ulceration, Myelopathy, Bony deformities,  Anorectal malformations and arterial anomalies, and Renal anomalies) PHACE syndrome includes features of Posterior fossa defects,  Hemangiomas , cerebrovascular Arterial anomalies, Cardiovascular anomalies including coarctation of the aorta, and Eye anomalies. The hallmark of PHACE syndrome is a large, segmental IH, characteristically located on the face, scalp, and/or neck.

19. DIAGNOSIS? TREATMENT?

20. LYMPHANGIOMA They are benign uncommon malformation of the lymphatic system They may superficial or deep, congenital or acquired Cysts or pockets of lymphatic fluid Contains clear straw coloured fluid unless infected or bleeding has occurred Lymphangiomas grow slowly. Sudden enlargement seen in infection, bleeding Spontaneous resolution uncommon cystic lymphagioma occurring in cervical area.

21. Common sites; neck, head( tongue), axilla, chest wall abdominal wall Internal organs involved in 10% mesentery commonly

22. Patient presents with disfigurement Mass Pain +/- fever Presure effects- respiratory distress, dysphagiaNeck - 85% unilateral Soft multiloculated mass Transilluminates

23. treatment Observation Sclerotherapy Surgical excision combination

24. Dermoid cyst Cyst beneath skin that is lined by ‘skin’ Congenital or acquired Commonly on face, neck, scalp. Others- intracranial, spinal, intra abdominal Present at birth but become obvious later. Rarely multiple

25. Site- lines of fusion, Ovid or spherical Smooth surface Fluctuate but not trans illuminate In subcutaneous tissue

26. Diagnosis clinical Treatment: surgical excision

27. Lipoma Benign tumour arising from fat Can be diffused or localised

28. features

29. Types; Neurolipomas Fibrolipoma Naevolipoma

30. diagnosis : mostly clinical Investigation : Doppler usg ct or mri ( abdominal or scalp lipoma ) Treatment: excision