Seckel Syndrome and Pregnancy: The Importance of a Multidisciplinary Team Meeting

Sunday, June 16, 2013

Title: Seckel Syndrome and Pregnancy: The Importance of a Multidisciplinary Team Meeting

Ryman Hall B4 (Gaylord Opryland)
Sue Ellen Abney-Roberts, MSN, RNC, C-EFM , Perinatal Services, Georgia Regents Health System, Augusta, GA
Frankie Parks, BSN, RNC , Perinatal Services, Georgia Health Sciences Medical Center, Augusta, GA

Discipline: Advanced Practice (AP), Childbearing (CB)

Learning Objectives:
  1. Describe Seckel Syndrome’s physical features and their potential impact during the intrapartum period
  2. Formulate a facility specific plan designed to coordinate multidisciplinary meetings for high risk obstetric patients
  3. Critique the benefit of a multidisciplinary approach to improve patient outcomes
Submission Description:
Background: Seckel syndrome is an extremely rare autosomal recessive genetic disorder that is a form of primordial dwarfism. It was first described in 1960 by Dr. Sekel.  Typically, intrauterine growth restriction is first identified in utero with a subsequent diagnosis of dwarfism. Physical features associated with this syndrome are microcephaly,  unusual facial features including large eyes, a narrow face, micrognathia and a protruding nose similar to a bird’s beak. These physical features cause a significant anesthesia risk. Patients may have varying degrees of mental retardation.

Case: Patient is a single 24 year old G1P0 with Seckel Syndrome. She lives with a family member and is developmentally delayed. She was referred to our Academic Medical Center for prenatal care at 23 weeks gestation. Of note, patient is 3 feet 10 inches tall and weighed 71 pounds at her initial visit. A referral was made to the ENT Clinic due to a history of respiratory arrest during general anesthesia as a child requiring a tracheostomy. Septal deviation was noted with complete obstruction on the right. Her cesarean section under regional anesthesia was scheduled and plans were made for an awake intubation if general anesthesia was required. She could open her mouth .5 cm pre-operatively.

 Patient presented to L&D at 35 and 36 weeks to rule out labor. Nursing was unaware of this high risk patient and was concerned about plans for her delivery. After these triage visits, a plan of care for delivery (OB, ENT and Anesthesia) was placed in her chart by Maternal-Fetal Medicine.  Unfortunately nursing was not included in development of this plan and therefore no plans were made for her postpartum care with a trachesotomy, a skill none of our staff was comfortable with. Patient arrived for a clinic visit  at 37 4/7 weeks gestation in active labor and was sent to L&D. Delivery was done in the Adult Operating Room under general anesthesia with an awake tracheostomy prior to the C/S. Patient went to Medical ICU post-operatively then was transferred to the ENT Unit on Day #2 after the tracheostomy was decannulated. Mom and baby discharged on postop day #3.

 Conclusion: This is an example of the importance of having nursing representation when multidisciplinary plans of care are being established for high risk obstetrical patients. Care planning for high risk patients should include potential complications of postpartum care including possible ICU admission and training for staff.
 
Keywords: Seckel Syndrome, multidisciplinary, dwarfism
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