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Management of a Pregnant Patient with Incontinentia Pigmenti
Abstract Number: SUN-61
Abstract Type: Case Report/Case Series
Patient History: A 29 year-old female G1P0 with a history incontentia pigmenti (IP) presented for induction of labor for oligohydramnios at 39.4 weeks. Her history was also notable for leg-length discrepancy, asthma, obesity, bicornuate uterus, and features of IP such as striated skin, scoliosis, mild mental retardation, and teeth irregularities.
Perioperative Course: Labor analgesia was requested and after multiple attempts a combined spinal-epidural was placed at the L3-4 interspace which functioned well throughout labor. Vaginal delivery was complicated by retained placenta. Twenty five minutes after delivery urgent manual extraction of placenta and suction D&C was required. This was attempted under neuraxial anesthesia with N2O and ketamine supplementation. However the patient was unable to tolerate the procedure and general anesthesia was induced. Extubation was complicated by airway obstruction and bronchospasm.
Discussion: Incontentia pigmenti, also known as Bloch-Sulzberger syndrome, is an X-linked dominant condition caused by a mutation in the NEMO gene with an estimated incidence of 0.7/100,000. IP is a genodermatosis that primarily affects the skin, eyes, hair, teeth, musculoskeletal and central nervous systems. The diagnostic feature is a full body rash that progresses through several stages and results in a characteristic swirling pattern. Key anesthetic concerns for patients with IP relate to oral and CNS abnormalities.
Dental anomalies include conical teeth, anodontia, and soft palate hypoplasia which may make airway manipulation more challenging. In this particular case, airway abnormalities combined with asthma and obesity may have contributed to respiratory compromise. IP affects the CNS and is associated with hemiplegia, hemiparesis, seizures, and mental retardation. Underlying disorders will affect the choice of anesthetic agents and the latter may impede communication with the patient and affect care. In this case, mental retardation contributed to the need for GETA. Musculoskeletal abnormalities also occur, including hemivertebra, congenital dislocation of the hip, and kyphoscoliosis. In this case, the presence of scoliosis made placement of a regional anesthetic challenging.
Less frequently, cardiovascular anomalies are present and may include atrial septal defects, ventricular endomyocardial fibrosis, and primary pulmonary hypertension. Given the rarity of the disorder, very few cases have been described in the literature and the purpose of this case is to highlight anesthetic challenges that may occur in the parturient with IP.
Chung W, Lee D, Change S, et al. A case of incontinentia pigmenti associated with multiorgan abnormalities. Ann Dermatol. 2009; 21: 56-59.
Fusco F, Paciolla M, Conte M, Pescatore A, et al. Incontinentia pigmenti: report on data from 2000 to 2013. Orphanet Journal of Rare Diseases. 2014; 9:93.