Pediatric groin disorder, Lecture for CI.pptx

Discusses the common pediatric groin and scrotal diseases.

1. Pediatric groin disorders: Management of acute scrotum, undescended testis and inguinal hernia

2. outline Intrduction Embroyolgy and pathophysiology Clinical features Workups Management Referrences

3. Acute scrotum is defined as acute scrotal pain with or without swelling and erythema. Early recognition and prompt management are imperative  permanent ischemic damage to the testis.

4. Testicular torsion Torsion of the testis results from twisting of the spermatic cord which compromises the testicular vasculature and results in infarction. There appears to be a 4-8-hour window before significant damage occurs once torsion develops testicular salvage declines significantly beyond six hours.

5. Two types of torsion occur: intravaginal and extravaginal. Intravaginal torsion is more common in children and adolescents. spermatic cord twists within the tunica vaginalis Abnormal fixation of the testis and epididymis within the tunica vaginalis - ‘ bell-clapper’ deformity and has an incidence as high as 12%

6. Extravaginal torsion occurs perinatally when the spermatic cord twists proximal to the tunica vaginalis

7. CLINICAL FEATURES sudden onset severe unilateraL pain in the testis lower thigh,or lower abdomen +/_nausea and vomiting. Past history of similar pain in 50%

8. Physical examination Scrotal swelling and erythema retracted up toward the inguinal region a transverse orientation and an anteriorly located epididymis The cremasteric reflex is often absent with testicular torsion

9. focal tenderness at the superior pole of the testis or along the epididymis is often found with a torsed appendix testis or epididymitis

10. workups Color Doppler u/s Sensitivity of 89.9%, a specificity of 98.8% Ultrasound should only be used when the diagnosis is equivocal because imaging studies will only delay scrotal exploration.

11. Management The diagnosis of testicular torsion is usually clinically apparent and managed by immediate scrotal exploration. manual detorsion can be attempted Detorsion is performed with a medial to lateral, ‘open book’ rotation—2/3 rd of cases prompt exploration and fixation remain mandatory because the detorsion may not be complete and torsion can reoccur.

12. Exploration is typically performed using a median raphe scrotal incision. The symptomatic hemiscrotum is entered and the testis delivered, detorsed If the testis is clearly nonviable, it should be removed to avoid potential damage to the contralateral testis from the formation of antisperm antibodies. Contralateral scrotum should be explored  orchidopexy

13. CONDITIONS MIMICKING TESTICULAR TORSION Torsion of Testicular Appendages The testicular appendage represents a vestigial remnant of the Müllerian duct, and the epididymal appendage is of Wolffian duct origin. Torsion of Testicular Appendages most common cause of an acute scrotum misdiagnosed as acute epididymitis or epididymo-orchitis. Blue dot sign Managed expectantly with NSAIDs

14. Epididymitis True bacterial epididymitis is rare in children, accounting for 10% to 15% of patients with an acute scrotum. bladder and urethra to the epididymis in a retrograde direction via the ejaculatory ducts and can be associated with UTI The scrotal pain and swelling typically have a slow onset, worsening over days Examination reveals induration, swelling, and tenderness of the hemiscrotum

15. If acute epididymitis is found on scrotal exploration, cultures should be obtained, but the contralateral side should not be opened to avoid spreading the infection.

16. TESTICULAR TRAUMA The diagnosis is made by taking a complete history, and paying close attention to factors suggesting sexual abuse. The injured testis is swollen and is markedly tender. The most common injury is a hematoma of the testis. Ultrasound should be obtained to evaluate for rupture of the tunica albuginea, which is an indication for operative repair

17. UNDESCENDED TESTIS

18. Undescended testis a testis that cannot be manipulated to the bottom of the scrotum without undue tension on the spermatic cord Sir John Hunter, the British anatomist, reported this condition in 1786. In 1877, Annandale performed the first successful orchidopexy.

19. incidence UDT occurs in approximately 3% of term male infants and in up to 33–45% of premature and/or birth weight <2.5 kg male infants. The majority of testes descend within the first 6 to 12 months such that at 1 year, the incidence is down to 1%. Testicular descent after 1 year is unlikely. The overall rate of secondary testicular ascent has been reported between 2–45%

20. location of UDT 2/3 rd to 4/5 th of cases are palpable Usually within the inguinal canal or distal to the external ring. Anomalies associated with UDT include patent processus vaginalis and epididymal abnormalities. Specific syndromes with higher rates of UDT include prune-belly syndrome gastroschisis bladder exstrophy

21. two phases of testicular descent The first transabdominal” phase at 8 to 15 weeks the testis (T) is held near the inguinal abdominal wall is controlled by testicular hormones, with insulin-like factor 3 the primary hormone possibly augmented by mullerian-inhibiting substance (MIS)

22. In the second (“inguinoscrotal”) phase at 28 to 35 weeks, the gubernaculum migrates by elongation toward the scrotum. This is controlled indirectly by testosterone acting on the genitofemoral nerve (GFN) in the dorsal root ganglia  to release calcitonin gene-related peptide (CGRP)

23. Two important hormones in testicular descent are insulin-like factor 3 (INSL3) and testosterone, both secreted by the testis anatomic factors are the gubernaculum testis and the cranial suspensory ligament (CSL).

24. The gubernaculum is thought to help anchor the testis near the internal inguinal ring as the kidney migrates cephalad Under the influence of INSL3,the gubernaculum undergoes two phases: outgrowth and regression Outgrowth  rapid swelling by the gubernaculum  dilating the inguinal canal and creating a pathway for descent.

25. CLASSIFICATION OF UNDESCENDED TESTES

26. Palpable or non palpable UDT Nonpalpable testes account for approximately 20% of all undescended testes. About 40% of the nonpalpable testes are intra-abdominal, 40% are inguinal, and 20% are atrophic or absent (vanishing testis syndrome).

27. Retractile testis Transient retraction of the testis out of the scrotum is a normal reflex occurs as a result of low temperature or stimulation of the cutaneous branch of the genitofemoral nerve (inner thigh). retractile reflex is weak or absent at birth, and the scrotum is often pendulous. Later in childhood, when androgen levels are low, cremasteric contractility is significantly increased and the cremasteric reflex more pronounced. After 10 years of age, the reflex becomes less pronounced as androgen levels rise with the onset of puberty.

28. ACQUIRED UNDESCENDED TESTES Acquired cryptorchidism is caused by failure of the spermatic cord to elongate in proportion to body growth patients with cerebral palsy, in whom acquired cryptorchidism approaches 50% in postpubertal boys with severe spastic diplegia Secondary or Iatrogenic Undescended Testis Secondary UDT is an uncommon complication of inguinal hernia repair, orchiopexy, or hydrocelectomy.

29. ETIOLOGIES The etiological factors of UDT can be traditionally grouped as: anatomical anomalies of the testis, epididymis and vas deferens improper attachment of the gubernaculum, patent processus vaginalis and inguinal hernia (hernias are found in 90% of UDT), anomalies of the inguinal canal. hormonal : deficient GnRH production or insensitivity of GnRH or LH receptors, deficient androgen production or insensitivity of androgen receptor, deficient INSL3 production or insensitivity of INSL3 receptor, deficient CGRP production (disorder of genito-femoral nerves) or insensitivity of CGRP receptor.

30. 3-Genetic androgen receptor gene mutations 5α-reductase gene HOXA10 gene mutations heterozygous mutations of Insl3 and Lgr8 genes (chromosome 19)

31. Diagnosis History Physical examination Diagnostic workups

32. EFFECTS OF UDT ON fertilty Risk of malignancy Tempreture variation Endocrine effect Germ cell dev.t Inguinal Hernia Torsion of a Cryptorchid Testis Increased Trauma Risk

33. treatment Hormonal surgical

34. Hormonal therapy Hormone therapy is based on the premise that the undescended testis is caused by deficiency of the hypothalamicpituitary-gonadal axis postnatal treatment can induce the required migration of the gubernaculum Therapy has been tried with testosterone, hCG, and luteinizing hormonereleasing hormone (LHRH).

35. doses caused precocious puberty. In the past 10 to 20 years, hCG Success rates for treatment range from 10% to 50%. Children older than 4 years and those with bilateral undescended testes near the scrotal entrance or retractile testes respond most favorably to hCG.

36. Acquired undescended testes with severe retraction or secondary ascent may respond to hCG treatment at levels of 100 IU/kg intramuscularly twice a week for 3 to 4 weeks. LHRH can be given as a nasal spray at 100 mg in each nostril six times a day for 3 to 4 weeks.

37. Surgical Treatment Treatment of cryptorchidism is based on the assumption that early intervention will prevent secondary degeneration of the testes caused by high temperature. Studies showing early degeneration of the germ cells in the first 6 to 12 months through to macroscopic atrophy in school-age children all suggest that undescended testesundergo progressive degeneration after birth Orchidopexy is recommended at 6 to 9 months. This is because the first signs of damage to the testes are identified at about 6 months of age

38. complications of Orchidopexy Failure of testis to reach scrotum Secondary atrophy of the testis Retraction of testis out of scrotum Occlusion of vas deferens Hemorrhage Wound infection

39. Inguinal hernia

40. 1-5% of all children develop inguinal hernia 10-30% in premature infants; due to lung disease M:F is 5:1 (in prematures 1:1) Right side twice as common as left side

41. Embryology and Anatomy

42. Clinical presentation Most are asymptomatic except for inguinal bulge Manuevers: having the. Child raise the head while supine; blowing up baloon; standing the child upright; cell phone documentation A good history is also reliable DDX: retractile testis, hydrocele, prepubertal fat In 12-17% present with incarceration Young age and prematurity are risk Irreducible mass, intermittent abd pain, vomiting Abdominal distension is late sign

43. Management Hydrocele with PPV: wait at least one year of age Incarceration: if not gangrenous - attempt reduction; All inguinal hernia - urgent but not emergent