Secondary nerve lengthening to obtain full knee extension in popliteal pterygium syndrome.

Microsurgical nerve lengthening was performed in two siblings presenting a popliteal pterigium syndrome with a knee flexion contracture of 80 degrees. After the first attempt for nerve lengthening and knee extension elsewhere, a repeated lengthening was required due to continuing tip-toe walking and recurrent knee contracture at the age of 3 years. An extensive external and internal interfascicular microsurgical neurolysis resulted in a lengthening of the nerves. A full length of leg procedure had to be performed, inclusive of Achilles tendon lengthening to obtain a complete extension of the knee and a 90-degree ankle flexion. Maintaining the leg in a fully extended position was obtained with a dynamic splinting in the first month after the operation. When timing the operation we have to consider the importance of adequate precision of the microsurgical neurolysis, down to the identification of the Fontana bands, and the adequate postoperative splinting.

Reconstructive surgical management of cryptomenorrhea because of complex uterovaginal malformations with duplicate uterus and complete vaginal agenesis.

OBJECTIVE: To describe the surgical reconstructive options in cases of cryptomenorrhea because of an obstructed functioning uterus with upper and lower vaginal agenesis. DESIGN: Assessment of operative, anatomic, and functional results after operative reconstruction over a 1-year follow-up period. SETTING: University hospital and referral center for pediatric and adolescent gynecology. PATIENT(S): Three adolescent girls, aged 15 to 18 years with primary amenorrhea, cyclic abdominal pain, hematometra, and complete vaginal agenesis. INTERVENTION(S): A combinative vaginal and laparoscopic approach to reconstruct a neovagina by human amniotic membranes and establish the uterovaginal continuity. MAIN OUTCOME MEASURE(S): Anatomic success was defined by a vaginal length ≥8 cm, and a width allowing the introduction of two fingers. Functional success was evaluated according to the restoration of menstrual cycle resolving of pain and patients'sexual satisfaction. RESULT(S): Neovaginal length was 9 to 12 cm and adequately wide. In all patients the uterovaginal continuity could successfully be restored. Satisfactory anatomic and functional results could be achieved, with no operative morbidity. Regular menstruation resumed with normal ultrasound findings during follow-up. CONCLUSION(S): In rare cases of an obstructed functioning uterus by complete vaginal agenesis, a combinative laparoscopic and vaginal surgical approach effectively restores anatomy and function, by an overall minimal operative morbidity. Human amnion provides excellent results in neovaginal reconstruction.

[Urinary incontinence in neurogenic defects and urogenital anomalies in childhood]. / Harninkontinenz bei neurogenen Defekten und Urogenitalanomalien im Kindesalter.

Dysraphic defects may cause neurogenic incontinence in childhood. Constipation and encopresis are often associated. Depending on the involved segment of the spinal cord hyperreflexia or atonia of the detrusor is observed. Similar findings, without anatomic correlation, can be seen in occult-neurogenic voiding dysfunctions. Therapeutic means aim at preservation of kidney function and the best possible continence. If the symptoms cannot be treated by anticholinergic drugs in a low-capacity, hypertonic bladder, augmentation by bowel segments or continent urinary diversion (e.g. Mainz I pouch) is performed. In the last years modalities of clean intermittent self-catheterization in high-capacity, atonic bladders could be enhanced by the development of new atraumatic catheter systems. Urogenital malformation e.g. proximal epispadias and exstrophic bladder can cause incontinence as well. Recently, new therapeutic concepts were introduced. Ectopic ureter (extraurethral incontinence) in girls or posterior urethral valves in boys as a reason for incontinence must not be forgotten.