Roopa, a 38 years old lady was diagnosed to have a rare condition called Rosai-Dorfman Disease (RDD) at Vikram Hospital, She initially presented to our Neurosurgeons with c/o feeling unsteady while walking which worsened with movement of head for 2-3 months duration. She also had headaches & nasal block. MRI of the head and neck and biopsy from the nasal sinus was done by our ENT surgeon and the condition was diagnosed by our Pathologist as intracranial RDD. Patient was seen by Dr. Niti Raizada, Sr. Consultant, Medical Oncologist and started on chemotherapy.
Patient had been married for 12 years and had undergone 2 cycles of IVF but was unable to get pregnant. After she was diagnosed with RDD, she had given up hopes of pregnancy. Miraculously, patient discovered she was pregnant within three months of the chemotherapy and continued the pregnancy despite the risk of teratogenicity.
Elaborating on the criticality of the case, Dr. Shashikala Ksheerasagar, Consultant Obstetrician & Gynaecologist, Vikram Hospital, said, “Our team of doctors categorized this case as high risk pregnancy and handled such a case for the first time. The case was very challenging from the beginning as the patient had a spontaneous pregnancy following chemotherapy for RDD, the Obstetric team faced many challenges managing the patient in the presence of co-morbidities like Gestational Diabetes Mellitus (GDM), Anemia, Sub clinical hypothyroidism, Allergic bronchitis, Progressive symptom of RDD like diplopia (Visual disturbance double vision), and cerebellar ataxia (difficulty in walking) and Headaches.”
Roopa’s health continued to deteriorate where she had difficulty walking, visual disturbances & managing day to day activities like cooking, housekeeping and as pregnancy advanced, she was heavily dependent on her husband for performing even the smallest of tasks such as eating food, medication, etc. If condition was untreated, she would have headaches, severe visual disturbance and incapability to walk. She would have continuously raised intracranial pressures which could lead to herniation of brainstem and sudden death.
It was a challenge handling her, an elective C/S was done, and patient closely monitored post operatively. Post pregnancy Lactation was an issue as she had a delay in initiation of lactation. Two and a half months after delivery patient underwent radical stereotactic radiation for the intracanal lesion. Following radiotherapy, patient made a good recovery and was able to handle her baby, her household chores independently,” added Dr. Shashikala Ksheerasagar, Consultant Obstetrician & Gynaecologist, Vikram Hospital.
Dr. Mohan D. Mahendrakar, Consultant Pediatrician & Neonatologist, Vikram Hospital, Bengaluru said, “Patient delivered a baby boy and we found that baby was suffering from imperferforate anus, ASD (Atrial Septal Defect - hole in the heart) and PUJ obstruction on the left side. After diagnosis, the baby underwent colostomy on the very first day he was born.”
After a period of time as the baby grew older, an ANO-RECTO PLASTY was conducted by Dr. Deepti Vepakomma, Consultant Paediatric Surgery. Now the baby is more than a year old and has normal growth and development.
Dr. Niti Raizada, Sr. Consultant, Medical Oncologist & Hemato-oncologist, Vikram Hospital, Bengaluru, said, “Intracranial Rosai Dorfman Disease is extremely rare, and it is an uncommon benign idiopathic lymphoproliferative disorder. It commonly affects lymph nodes, extra nodal involvement of multiple organs, Bilateral painless cervical lymphadenopathy, fever, night sweats and weight loss. Exacerbation of RDD during pregnancy and the need to resort to radiotherapy using cyber knife due to aggravation of symptoms and remarkable recovery”. is what we have seen.
RDD in this lady responded very well to combination chemotherapy drugs - however they were discontinued when she conceived. Later post-delivery, disease evaluation suggestion that tumor had increased in size and causing many symptoms when additional local therapy was recommended.
Dr. Somesh Mittal, MD & CEO, Vikram Hospital Bengaluru also commented, “We are proud to have dedicated and specialized consultants who focus constantly on saving lives of patients at all stages of illness. Roopa’s case was highly risky and our consultants worked really hard to save her and also her child.