–However, transfusions for priapism have been associated with ASPEN syndrome, a syndrome characterized by the association of SCD, priapism, exchange transfusion, and neurologic events. The most serious event associated with this syndrome is cerebrovascular accident.

–Exchange blood transfusion:

•This is done using packed RBCs.

•This however may be associated with neurological complications including headaches, seizures and obtundation.

•These complications may be related to the rapid elevation of Hb to above 12 g/ dl and release of procoagulant and vasoactive factors from the corpora cavernosa.

•To ovoid this, the target of exchange RBCs transfusion should be a Hb <10 g/ dl and hemoglobin S <50 %.

•A regular simple packed RBCs transfusion was found beneficial for those with stuttering or repeated attacks of priapism. The aim is to reduce HbS level to less than 30 %.

–Other therapies reported in the literature include calcium-channel blockers, such as verapamil, diltiazem, and nifedipine; hyaluronidase; and anticoagulants.

23.9.8Complications of Priapism and Prognosis

•Most episodes of priapism resolve without complications.

•These are usually of short duration.

•Prolonged episodes of priapism on the other hand may lead to fibrotic changes in the corpora cavernosa and subsequently erectile dysfunction.

•These changes are time dependent and after 24–48 h of priapism irreversible changes develop leading to erectile dysfunction.

•It has been reported that SCA patients who present with priapism and seek medical advice within 12 h usually retain normal erectile function while all those who present beyond

36 h after the onset of priapism develop erectile dysfunction.

•If irreversible damage has been done, longterm management of erectile dysfunction may be treated with placement of a penile prosthesis to restore function depending on the preference of the patient.

•A severe and prolonged episode of priapism will result in cavernous smooth muscle necrosis, fibrosis, and ultimately penile shortening.

•If erectile function is unlikely to recover, immediate implantation of a penile prosthesis may, therefore, be considered to avoid the complications of penile fibrosis and shortening of the penis.

•The prognosis for erectile function in sickle cell patients is poor.

•Most authors report that one fourth to one half of patients become impotent after prolonged episodes of priapism.

•The prognosis may be better for prepubertal children.

•Acute cases have a much poorer prognosis than cases of intermittent priapism.

Further Reading

1.Adeyoju AB, Olujohungbe ABK, Olujohungbe ABK. Priapism in sickle-cell disease; incidence, risk factors and complications—an international multicenter study. Br J Urol Int. 2002;90(9):898–902.

2.Burnett AL, Bivalacqua TJ, Champion HC, Musicki B. Long-term oral phosphodiesterase 5 inhibitor therapy alleviates recurrent priapism. Urology. 2006;67:1043–8.

3.Chacrabarty A, Upadhyay J, Dhabuwala CB, Sarnaik S, Perlmutter AD, Connor JP. Priapism associated with sickle cell hemoglobinopathy in children: longterm effects on potency. J Urol. 1996;155:1419.

4.El-Banasawy MS, Dawood A, Farouk A. Low flow priapism: risk factors for erectile dysfunction. BJU Int. 2002;89:285–90.

5.Gbadoe AD, Atakouma Y, Kusiaku K, Assimadi JK. Management of sickle cell priapism with etilefrine. Arch Dis Child. 2001;85:52–3.

6.Kato GJ, McGowan V, McGowan V. Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease. Blood. 2006;107(6):2279–85.

7.Liu J, Al-Hothari MA, Mahboob FA. Non-surgical treatment of recurrent or stuttering priapism in sickle cell children. Saudi Med J. 2003;24:1143–5.

8. Mantadakis E, Ewalt DH, Cavender JD, Rogers ZR, Buchanam GR. Outpatient penile aspiration and epinephrine irrigation for young patients with sickle cell anemia and prolonged priapism. Blood. 2000;95:78–82.

9.Maples BI, Hagemann TM. Treatment of priapism in pediatric patients with sickle cell disease. Am J Health Syst Pharm. 2004;61:355–63.

10.Miller ST, Rao SP, Dunn EK, Glassberg KI. Priapism in children with sickle cell disease. J Urol. 1995;154:844–7.

11. Rogers ZR. Priapism in sickle cell disease. Hematol Oncol Clin N Am. 2005;19:917.

12. Saad ST, Lajolo C, Gilli S, Marques Junior JF, Lima CS, Costa FF, et al. Follow-up of sickle cell disease patients with priapism treated by hydroxyurea. Am J Hematol. 2004;77:45–9.

13.Salem EA, ElAasser. Management of ischemic priapism by penile prosthesis insertion: prevention of distal erosion. J Urol. 2010;183:2300–3.

14.Siegel JF, Rich MA, Brock WA. Association of sickle

cell disease, priapism, exchange transfusion and neurological events: ASPEN syndrome. J Urol. 1993;150: 1480–2.

15. Van der Horst C, Stuebinger H, Seif C, Melchior D, Martinez-Portillo FJ, Juenemann KP. Priapism: etiology, pathophysiology and management. Int Braz J Urol. 2003;29:391–400.

16. Wen C, Munarriz R, Mcauley I, Goldstein I, Traish A, Kim N. Management of ischemic priapism with highdose intracavernosal phenylephrine: from bench to bedside. J Sex Med. 2006;3(5):918–22.

24.1Introduction

•Cryptorchidism is derived from the Greek kρυπτός, kryptos, meaning hidden and ὄρχις,

orchis, meaning testicle.

•It is defined as the absence of one or both testes from the scrotum (Fig. 24.1).

•Undescended testis was first described in 1786 by Hunter and has been recognized for centuries.

•The first surgical orchiopexy was attempted in 1820 by Rosenmerkal.

•Annandale in 1877 performed the first successful orchiopexy.

•In 1899, Bevan published the principles of testicular mobilization, separation of the processus vaginalis, and repositioning of the testis into the scrotum.

•It is considered the most common birth defect of the male genitalia.

–It is estimated that about 3 % of full-term and 30 % of premature infant boys are born with at least one undescended testis.

–It is also estimated that about 80 % of undescended testes descend by the first year of life and the majority of them descend within the first 3 months of life.

–This makes the true overall incidence of undescended testes around 1 %.

•About 75–80 % of undescended testes are unilateral.

•Cryptorchidism can affect one or both testes and approximately 10 % of cases are bilateral.

For unilateral cases the left testicle is more commonly affected

•In 80–90 % of cases, an undescended testis can be felt in the inguinal canal; in a minority the testis or testes are in the abdomen or nonexistent (truly “hidden”).

•It has been estimated that the incidence of undescended testis in premature male newborns is about 30 %.

•The incidence of undescended testes in fullterm male newborns is 3–5 %.

•This incidence decreases to 0.8 % at 3 months of age as some of these undescended testes will descend to the scrotum spontaneously.

•In the United States, the prevalence of cryptorchidism ranges from 3.7 % at birth to 1.1 % from age 1 year to adulthood.

Fig. 24.1 A clinical photograph showing undescended right testis

•Internationally, the prevalence of cryptorchidism ranges from:

–4.3–4.9 % at birth

–1–1.5 % at age 3 months

–0.8–2.5 % at age 9 months

•Siblings of boys with undescended testes are at increased risk for cryptorchidism, with a reported incidence of up to 10 %.

•Cryptorchidism is identified in 1.5–4 % of fathers and 6.2 % of brothers of patients with cryptorchidism.

•Heritability in first-degree male relatives is estimated to be 0.67.

•True undescended testicles rarely descend into the scrotum spontaneously after 4 months of age.

•Factors that Predispose to cryptorchidism include:

–Prematurity

–Low birth weight

–Small size for gestational age

–Twinning

–Maternal exposure to estrogen during the first trimester

•Sometimes undescended testes are found incidentally during routine herniotomy in patients

with testicular feminization syndrome (Figs. 24.2 and 24.3).

•Early diagnosis and management of the undescended testicle are important to preserve fertility and improve early detection of testicular malignancy.

•The recent improvements in surgical technique, including laparoscopic diagnosis and treatment of undescended testes are likely to improve outcomes.

•According to the guidelines published by the American Urological Association in May 2014:

–Imaging for cryptorchidism is not recommended prior to referral, which should occur by 6 months of age.

–Orchiopexy is the most successful therapy to relocate the testis into the scrotum.

–Hormonal therapy is not recommended.

–Successful scrotal repositioning of the testis may reduce but does not prevent the potential long-term issues of infertility and testis cancer

–Appropriate counseling and follow-up of the patient are essential

•Undescended testes are known to be associated with complications which include:

–Reduced fertility

–Increased risk of testicular germ cell tumors

–Undescended testes are known to be associated with inguinal hernia which can be complicated by irreducibility and strangulation

–Psychological problems when the boy grows up

–Undescended testes are also more susceptible to testicular torsion and subsequent infarction

–Undescended testes are more prone to trauma

•It has been shown that men who have had an undescended testis have:

–Lower sperm counts

–Poorer quality of sperms

–Lower fertility rates than normal men

•The likelihood of subfertility increases in those with bilateral undescended testes and increasing age at the time of orchidopexy.

•The risk of subfertility can be reduced by early orchidopexy.

•This is one reason why orchidopexy should be done early as early as 6 months of age and not later than 2 years of age.

•An increased incidence of epididymal abnormalities in undescended testes also contributes to infertility.

•It has been well documented that men with a history of undescended testicle have a higher- than-expected incidence of testicular germ cell cancers. The incidence of testicular cancer among men with an undescended testis is approximately 1 in 1,000 to 1 in 2,500. This is significantly higher than the risk among the general population (1:100,000).

•Orchidopexy does not reduce the risk of testicular cancer, but it makes it easier to diagnose it early through testicular self-examination.

•About 20 % of testicular tumors in men with unilateral cryptorchidism occur on the side with the normally descended testicle.

•Undescended testes have also an increased risk of testicular torsion. Torsion of an intraabdominal testis may present as an acute abdomen.

•Undescended testes are also known to be associated with an inguinal hernia (Patent processus vaginalis) which is repaired at the time of orchidopexy. If an overt hernia is present in association with undescended testis, hernia repair with orchidopexy should be done at the time of diagnosis. This is to avoid the risk of irreducibility and strangulation.

•The classification of undescended is based on the physical and operative findings of undescended testes:

–True undescended testicles: This can be

•Intra-abdominal (nonpalpable)

•Peeping testis at the internal ring

•Canalicular or inguinal testes

•Undescended testes at the upper scrotum

–Ectopic testicles

–Retractile testicles

•About one half of nonpalpable testes are found to be intra-abdominal, while the rest represent absent (vanishing) or atrophic testes.

•The vanishing testicle is thought to be caused by intrauterine testicular torsion.

•Sometimes tissue in the scrotum may be palpable and it feels like an atrophic testis. This should not be taken for granted and sometimes this tissue represents the gubernaculum or a dissociated epididymis and vas deferens, and may coexist with an intra-abdominal testis.

•The presence of bilateral nonpalpable testes in a phenotypically male newborn should be taken seriously. The possibility of a genetic female with congenital adrenal hyperplasia and in an older child testicular absence must be kept in mind. These patients should be evaluated including:

–Pelvic ultrasound

–Karyotyping