Mixed connective tissue disease (MCTD) is a term involving the features of lupus systemic sclerosis, polymyositis, rheumatoid arthritis and high titre of anti ribonucleoprotein (RNP) antibodies, exact etiology is not known. It is characterized by microvascular damage, immune system activation leading to inflammation and excessive deposition of collagen in the skin, lungs, heart, gastrointestinal tract and kidneys. The females are being more affected especially after childbirth attributed to the hypothesis of microchimerism, the pathogenesis being a two way migration of fetal cells through the placenta. It cannot be cured completely but treatment with corticosteroids is helpful. ACE inhibitors are useful in renal involvement and hypertension. We had a case of mixed connective tissue disorder in a patient aged 28 years with 12 weeks of gestation for medical termination of pregnancy (MTP) and permanent sterilization. The complications are preeclampsia, preterm labor, fetal growth restriction, eclampsia, thrombocytopenia and infections like pneumonia, sepsis like syndrome and the maternal mortality rate is 325/100000. This is a unique case of MCTD wherein we had limited cutaneous disease like CREST-calcinosis, Raynaud's phenomenon, esophageal involvement, sclerodactyly and telangiectasia of a lesser degree. So early diagnosis and timely intervention is advocated to prevent complications.
ABSTRACT:The incidence of cervical fibroids is 0.5-1%. It is usually single; they are usually confined to supravaginal portion of the cervix. Rarely it becomes submucous and polypoidal. So it is usually subserous or interstitial. It can be anterior, posterior or central in position. We had different types of cervical fibroids of which we will describe a few. Usually cervical fibroids cause infertility, difficulty in labor, infections, metrorrhagia, menorrhagia, constipation, retention of urine and dyspareunia. The cervical fibroid distorts the shape of cervix and grows bigger. It pushes the uterus upward giving the appearance of lantern of Saint Paul's dome in a case of a central cervical fibroid. Most of the patients in the reproductive age get admitted for menorrhagia due to fibroid. Its growth is dependent on estrogen. It does not grow after menopause. KEYWORDS: Cervical fibroid, fibroid polyp, hysterectomy. CASE 1:A 52 year old housewife, completed the family, who had previous two LSCS section and sterilization done 20 years ago, got admitted with retention of urine for the past 12 hours. She gave history of burning micturition and frequent retention of urine for which catheterization was done a number of times outside.She had lower abdominal pain and post coital bleeding. She had irregular menstrual history. Foley's catheter was introduced. Bimanual examination showed cervix was replaced by huge mass 8×11 cm occupying the whole cervix and vagina. This was a central cervical fibroid wherein the uterus was found sitting on top of it.The uterus was palpable per abdomen as a soft globular mass2×3 cm suggestive of a fundal fibroid. Patient was anemic and 2 units of blood transfusion were given. Routine investigations were normal. Ultrasound(USG) report showed a small fundal fibroid 2×2 cm and a huge central cervical fibroid of size 8.3×11.3 cm (figure1). Patient was explained and consent for laparotomy was obtained.Laparotomy showed plenty of adhesions due to previous surgeries which were released .The bladder was found plastered with the mass. So a transverse incision was made at the level of the UV fold to push the bladder up. Uterine vessels supplying the fibroid were engorged and tortuous were carefully cut and ligated (figure 2).The uterus along with the central cervical fibroid shelled out without injuring the bladder, ureter and rectum. Pre-operative ureteric stenting could have helped in the surgery of shelling the cervical fibroid. Vault and abdomen closed after perfect hemostasis. The cut section of the specimen showed the uterus sitting on top of the cervical fibroid and had a small fundal fibroid of size 3×3cm.The specimen was sent for histopathology, because of the increased cellularity and mitosis, leiomyosarcoma was thought of initially by the pathologist. Later on, they confirmed it as a feature of highly cellular leiomyoma with hyaline and cystic degeneration (figure 2) Post-operative period was uneventful. Patient passed urine freely. She came for follow up. She had no complaints.
Leiomyoma is the most common estrogen dependent benign tumour of the uterus occurring in the reproductive age. It is composed of smooth muscle and fibrous tissue. Asymptomatic myomas can be present in 50% of cases. It causes mainly menstrual problems like menorrhagia, metorrhagia, dysmenorrhoea and also infertility. Nowadays it is very rare to get huge myomas because most of the patients for any vague, trivial complaints undergoes scan, which picks up even very small myomas. We are presenting a very huge fibroid causing hernia in a patient 48years old P3L3 who developed hernia, following puerperal sterilization done. She had a hernia repair done 20 years ago. Now she developed recurrent hernia due to the huge abdominal mass 22×22cms of a parasitic fibroid. She was taken up for laporotomy. The mass was removed in addition total hysterectomy with bilateral salpingo oophorectomy was done. This case is presented here because of its rarity and its management was quite tough-a Herculean task of course. KEYWORDS: Hernia, parasitic fibroid, Subserous fibroid, total abdominal hysterectomy with salpingo-oophorectomy. CASE REPORT:A 48years old lady P3L3 got admitted with the history of menorrhagia, abdominal pain and breathlessness since 6months. On examination she was found to be anemic. Uterus was palpable up to 24 weeks size and she had a separate huge mass 22×22cms size extending up to the umbilicus and the right lumbar region. She had a scar in the infra umbilical region with incisional hernia. Cervix was healthy.No significant medical history present. She was transfused with two units of blood. Other blood investigations were normal. X-Ray chest showed cardiomegaly with left ventricular hypertrophy. Intra venous pyelogram showed bilateral hydroureter. Ultrasound showed umbilical hernia, uterus had multiple fibroids-sizes: 20×17×10cms and a huge fundal fibroid of size 22×22cms. The right ovary was cystic. Computed tomography showed large heterogeneous multi loculated abdomino pelvic mass of size 22×20cms with internal degeneration and calcifications from the pelvis. MANAGEMENT:Patient was taken up for laporotomy. There were plenty of adhesions which were released. A huge subserous fundal fibroid, 24×24cms with a twisted pedicle almost getting detached was attached to the Fundus of the uterus on the right side. There were plenty of vessels from the omentum feeding the fibroid. The whole omentum was attached to the surface of the fibroid. It was very vascular. The clamps were applied on the twisted pedicle of the subserous fibroid as well as on the huge vessels which were feeding it.Thanks to the general surgeon, the task was made easy. Part of the omentum had to be sacrificed because it was adherent to the surface of the subserous fibroid. The uterus was enlarged to 24 weeks size which had intramural fibroids. Total abdominal hysterectomy with bilateral salpingo oophorectomy was done. Hernia repair was done meticulously with mesh.
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