Speakers

Local experience in successful separation of conjoined twins
1109 16:25-16:40
Interim Meeting of AASPN/303A
Conjoined Pyopagus Twins is a rare, congenital abnormality which represent around 10-18% of all conjoined twins. They are typically joined at the sacrum with face opposite to each other and may share terminal spine, spinal cord, gastrointestinal system, anus, perineal ragion, reproductive and genitourinary systems. Surgical separation of Conjoined Twins is challenging due to the complexity of anatomy and physiology requiring multidiscplinary team approach and detail preoperative workout. The success of surgery also depending on meticulous pre Operative planning and preparing for the unexpected senario. The authors will share the local experience in managing 2 cases of pyopagus in Hospital Kuala Lumpur, Malaysia who was operated in December 2005 at Pediatric Institute Hospital Kuala Lumpur and recently in October 2020 at Hospital Tunku Azizah ( Women & Children Hospital). The operative strategy and interesting intraoperative findings will be discussed in details . Intraoperative Neurophysiological Monitoring (IONM) were used in both cases. Case 1: 4 months, boys who were born via Elective Caesarian Section with combined weight of 4.245 kg. They are joined at the lumbosacral spine. Both twins have one leg with congenital Talipes Equino Varus and one normal leg each, on the ispilateral side and actively moving. Further imaging revealed shared spinal canal and fusion of Spinal cord with thecal sac begin at L2 vertebra ( Twin A) and L3 ( Twin B). Both have well developed external genitalia and separated anus. Surgical Separation was done successfully by combination of Neurosurgery and Plastic & Reconstructive Surgery team at 4 months of age on 17 October 2020. The surgery lasted for 16 hours. IONM contributed significantly in detemining the line of separation on the fused cord and transection of the sensory nerve roots which crossed over from the opposite twins and sharing similar exit foramina. Separation was done without any additional neurological motor deficit after surgery with immediate intraoperative improvement of wave following separation of the fused spinal cord. Fascia lata were used for reconstruction of dura defects reenforced with Tisseel glue while Fasciocutaneous flap in combination of regenerated template (Integra) and staged skin grafting were done for closure. One twin also have inguinal henia and underwent herniotomy about 1 month after separation by Pediatric Surgical team. Case 2: KH and KE born via LSCS with birth weight of 1.8Kg. Diagnosed Triplet during pregnancy with Pyopagus Conjoined Twin. The singleton triplet is alive and well. KH and KE were operated at 6 months old on 4th December 2005. They were fused at lower sacrum and cocyx with separated spinal cord. They have 4 lower limbs, 3 normal but KH has right CTEV. They shared a single anus but separated urethra and reproductive system, vagina closely opposed but separated. Surgical Separation was done with by combination of Pediatric Surgery and Neeurosurgery. Anus was dissected at midline and reconstructed while opposed vaginal was separated. KH developed chronic discharging sinus which resolved after several surgery and having continence to both urine and fecal. She also developed scoliosis on followup by Orthopedic Spine team. Not corrective surgery done yet. KE developed CFS leak and post operative day 1 and repaired. She also developed both bowel and urinaty incontinence and underwent bladder augmentation surgery by Urology on 27 March 2019. Both need long term followup with various specialties.

Pediatric endoscopic endonasal surgery for skull base lesion, technical challenges
1109 14:05-14:15
AASNS & WANS Joint Seminar/305
Surgical access to the intracranial skull base lesions has undergone substantial development with the indications of endoscopic endonasal surgery constantly expanding from lesion confined to sellar to the anterior midline skull base cisterns, posterior fossa and even to craniocervical junction through various route such as transphenoidal, transtuberculum, transplanum and transclivus approaches. Despite it’s clear advantages, there are certain limitations in performing surgical procedure especially in children due to restricted working space, small nostrils, narrow sinonasal corridor, unfavourable anatomy, lack of dedicated surgical instrumentation, limited range of maneuverability for microsurgical techniques and lack of flexible instruments to deal with laterally located lesions. The Sino nasal corridor must be expanded and optimized to have an adequate access to the skull base and facilitate the reconstruction of surgical defect in order to avoid Sino nasal complications, post operative CSF leakage and it’s associated morbidities. Some of the important considerations for this approach include locations of the lesions, involvement of the intracranial neurovascular structures, diagnosis, nature of the pathology, patient’s age and medical comorbidities and surgeon factors including training, experience and expertise. The utilization of the computer – assisted imaging navigation is becoming an important adjunct tool especially in case of repeated surgery or when normal anatomical landmarks of the nasal cavity and endonasal skull base altered or destroyed by diseases or previous surgery. It is useful during the preoperative planning and guide to safe resection and preservation of the critical functions, therefore ensure patient’s safety and improve outcome. Various cases of endoscopic endonasal surgery and it’s management strategies to overcome the technical challenges in children will be presented and discussed.