Langerhan’s Cell Histiocytosis of the Lumbar Spine during Pregnancy: A Rare Case with Literature Review

Rully H Dahlan,
Farid Yudoyono,
Sevline E Ompusunggu,
Akhmad Y Pramatirta,
Agung B Sutiono
Ahmad Faried,
Muhammad Z Arifin

The Journal of Spinal Surgery

Background: Langerhan’s cell histiocytosis (LCH), previously known as histiocytosis X, is a reactive proliferative dendritic cells of unknown pathogenesis characterized by the proliferation of Langerhan’s cells and is extremely rare in the lumbar spines of adults. This condition is most common among young males under the age of 15 years old (with a peak incidence at 2-4 years old), and the most frequent site of these osteolytic bony lesions of the spine is the thoracic region.

Purpose: To highlight an interesting and rare presentation for Langerhans cell histiocytosis of the spinal cord in pregnant woman.

Study design: This is a case report of a single patient in whom a Langerhans cell histiocytosis was resected from the lumbal spine in pregnant woman with return to normal functioning.
Patient sample: A 26-year-old pregnant woman at 20 to 22 weeks presenting with acute cauda equina syndrome, a 1-month history of pain and numbness and paraparese of right limb had gradually progressed to involve all the lower limbs. Outcome measures: Frankel grading of neural function and Visual Analogue Score are included to evaluate the therapeutic effiiency.

Methods: Magnetic resonance imaging revealed the widespread involvement of an extradural contrast-enhancing mass in the lumbar spine of L2-L5.

Results: The patient underwent decompression and surgical resection of the tumor in a three-quarters prone position, fetal heart monitoring was performed by our obstetrician; there was no fetal distress during the surgery. The diagnosis was confimed by histological analysis. She entered spontaneous labor at 36 to 37 weeks and birthed a baby weighing 3000 gm. The child began crying immediately and had Apgar scores of 8 and 10 in the 1st and 5th minutes respectively. No residual disease or recurrence was noted at follow-up longer than 1 year.

Conclusion: Our case’s unique presentation involves LCH in an adult pregnant patient at the lumbar spine with no osteolytic JOSS caSe repOrt 1-6Lecturer, 7Professor 1-3Department of Neurosurgery, Faculty of Medicine, Spine Research Laboratory, Universitas Padjadjaran, Dr Hasan Sadikin Hospital, Bandung, Indonesia Department of Obstetrics and Gynecology, Faculty of Medicine, Universitas Padjadjaran, Dr Hasan Sadikin Hospital Bandung, Indonesia 5-7 Department of Neurosurgery, Faculty of Medicine, Universitas Padjadjaran, Dr Hasan Sadikin Hospital, Bandung, Indonesia

Corresponding Author: Farid Yudoyono, Lecturer, Department of Neurosurgery, Faculty of Medicine, Spine Research Laboratory Universitas Padjadjaran, Dr Hasan Sadikin Hospital, Jl Pasteur
No. 38, Bandung 40161, Indonesia, e-mail: 10.5005/jp-journals-10039-1019
activity. The management of pregnant women and maintenance of fetal well-being should be coordinated among the spinal neurosurgeon, obstetrician and anesthesiologist.

Keywords: Langerhan’s cell histiocytosis, Pregnancy, Cauda equina syndrome, Three quarter prone position.