Successful treatments for Kartagener syndrome with completely immotile sperm or azoospermia

Kartagener triad syndrome consists of dextrocardia, bronchoectasia and chronic bronchitis. It is often associated with completely immotile sperm with structurally abnormal tails. However, we observed that about half of Kartagener syndrome cases show obstructive azoospermia with normal epididymal spermatozoa with structurally normal flagellum collected easily with MESA (Microsurgical Epididymal Sperm Aspiration). We report successful cases of Kartagener syndrome with completely immotile sperm or obstructive azoospermia. Retrospective case series. Viability of completely immotile sperms was investigated with eosin-negrosin test. We conducted HOST to choose the viable spermatozoon when the positive ratio was over 30%. This test is based on the principle that viable spermatozoa have intact membranes which shows swelling of the cytoplasmic space and curling of the sperm tail. The spermatozoon with HOST positive was available for use in ICSI. In the case of lower positive ratio (<30%), MESA was conducted. Scrototomy is usually performed under general (Propofol + Fentanyl citrate) or loco-regional (cord block) anesthesia (10ml of 1% Lidocaine + Anapeine in equal quantities). High quality sperm is retrieved from whitish regions and low quality sperm from yellowish regions at the head of epididymis through insertion of a glass pipette with a sharp angle. It is easy to collect a large volume of sperm from the white regions. We summarized clinical outcome of Kartagener syndrome in Table. Kartagener syndrome is now regarded as one type of primary ciliary dyskinesia (PCD) which is accompanied with ultrastructural or functional defects of cilia (flagellum) and higher probability of conception by selecting the available spermatozoon.Tabled 1Successful treatments for Kartagener syndrome with immotile sperm or azoospermia (*: Brothers)Viability with eosin-negrosin test (%) / HOST (positive, negative)Sperm Count (x100000) / Motility (%) / FSH (mIU/ml)2PN fertilization (%)Embryo transfer (n)Clinical outcomeTEM of flagellumImmotile ejaculate sperms1 *65 / positive500 / 0 / 5.466.7 (8/12)Blastocyst 5AA(1)Relivery ♂ (17y, normal sperm)Abnormal2 *55 / positive500 / 0 / 6.370.0 (7/10)Blastocyst 4AA(1)Relivery ♂ (14y)Abnormal345 / positive100 / 0 / 7.542.9 (3/7)Blastocyst 1BA(1)No pregnancyAbnormalObstructive azoospermia4Motile250 / 40 / 6.766.7 (4/6)Blastocyst 4AA(1)Relivery ♀ (10y)Normal5Motile250 / 40 / 6.775.0 (3/4)Blastocyst 3AA(1)Relivery ♀ (6y)Normal6Motile50 / 70 / 6.550.0 (4/8)Blastocyst 1AA(1)No pregnancyNormal Open table in a new tab