Volume 3 Issue 10, October 2016

Editorial
Parulekar SV

Retroperitoneal Inflammatory Myofibroblastic Tumour Presenting As A Vaginal Swelling
Niphadkar M, Parulekar SV.

Retroperitoneal And Pelvic Fibromatosis
Parulekar SV

Unicornuate Uterus With Functional Rudimentary Non-Communicating Right Horn
Santoso BI, Hidayah GN, Surya R

Spontaneous Postpartum Paravaginal Hematoma
Singhal N, Madhva Prasad S, Gupta AS.

Intrauterine Foreign Body Or Osseous Metaplasia Of The Endometrium
Singhal N, Madhva Prasad S, Gupta AS.

Fraser Syndrome, a Typical Case Detected Antenatally
Chhatrapati A, Nandu KR, Nadkarni T, Jassawalla MJ.

Obstructed labor in a case of Prune belly Syndrome
Pardeshi S, Swaminathan G, Warke HS, Mayadeo NM.

Anticoagulant Induced Vaginal Bleeding in Case of Deep Vein Thrombosis- A Therapeutic dilemma
Pai K, Raut D, Samant PY.

Huge Recurrent Ovarian Endometrioma Managed Laparoscopically
Shah NH, Paranjpe SH, Khadkikar R.

Editorial

Parulekar SV

The retroperitoneal diseases are important because they can remain asymptomatic until late in the stage of the disease, can cause a lot of morbidity, are difficult to diagnose clinically, and surgical approach to their management can be quite challenging. Retroperitoneal fibrosis is one such disorder in which there is inflammation and fibrosis. It is generalized and spreading. It is of interest to the gynecologists when it involves the lower retroperitoneal space and obstructs the blood vessels and ureters running through it. It may be primary or secondary to tuberculosis, actinomycosis, histoplasmosis, exposure to drugs like ergotomines, methyldopa, hydralazine, bromocriptine, beta blockers, pergolide, and methysergide. It may also develop after trauma including surgery, and radiotherapy for cancer. These patients present with vague symptoms like poorly localized, dull pain in the back, flank, or lower abdomen, fever, edema feet, malaise, anorexia, nausea, vomiting, and loss of weight.  Diagnosis requires a high degree of suspicion and help of imaging techniques like ultrasonography, contrast enhanced computed tomography and magnetic resonance imaging. Definitive diagnosis is based on a biopsy which is not obtained easily. Treatment is with drugs, and is often not very satisfactory. Another retroperitoneal disorder of interest to a gynecologist is retroperitoneal and pelvic fibromatosis. It may be caused by trauma, exposure to drugs, and irradiation. It is often asymptomatic. It tends to be more localized than retroperitoneal fibrosis. It may present with gastrointestinal bleeding or obstruction, unilateral ureteric obstruction and hydronephrosis. Diagnosis requires imaging and biopsy. Its treatment is surgical excision. Drug therapy may also help. A third group of retroperitoneal disorders of interest to a gynecologist is tumors, which can be benign or malignant. They may be mesenchymal in origin (arising from fibrous tissue, skeletal muscle, fat, vessels and nerves), extragonadal germ cells, lymphoma, or adenocarcinoma. Common tumors include liposarcoma, leiomyosarcoma, fibrosarcoma, rhabdomyosarcoma, haemangiopericytoma, malignant peripheral nerve sheath tumor, and malignant fibrous histiocytoma. The sarcomas can become very large before they become symptomatic. The symptoms are vague and do not suggest the diagnosis. A retroperitoneal mass is not mobile on palpation, but that does not help much in clinical diagnosis because many large intraperitoneal masses are also fixed. Though imaging modalities help, a definitive diagnosis is possible only with a biopsy. Treatment is difficult and prognosis is often not good. We present two interesting cases in this issue – one of retroperitoneal inflammatory myofibroblastic tumor which presented as a vaginal mass, and another one of retroperitoneal and pelvic fibromatosis. I hope they prove of interest to our readers.

Retroperitoneal Inflammatory Myofibroblastic Tumour Presenting As A Vaginal Swelling

Author Information

Niphadkar M*, Parulekar SV**

(* Third Year Resident, ** Professor and Head, Department of Obstetrics and Gynecology, Seth G S Medical College & KEM Hospital, Mumbai, India.)

Abstract

Inflammatory myofibroblastic tumor is of unknown etiology. It present at various sites, in relation to important structures. There are only six cases related to female genital tract reported in literature. We present a case in which the tumor was projecting into the vagina. This is the first case of this type in the world literature.

Introduction

Though called inflammatory, IMT is a neoplasm. It is multifocal and has a 35% chance of recurrence and metastases.[1,2] Its most common site is the lungs. It can also occur in the urinary bladder, kidney and ureter.[3,4,5] We present case in which the tumor was closely related to the urinary bladder, uterus and vagina and presented as a vaginal mass. It is the first case of its type in the world literature.

Case Report

A 34 year old female, para 2 living 2, previous full term normal deliveries, came to the gynecology clinic with the complaints of a painless vaginal swelling and dragging sensation in the pelvis. She also brought a MRI report which read as follows:

‘A well encapsulated T2 hyperintense lesion with multiple fibrotic strands within seen in the lower abdomen from L3 upwards and extending into the pelvis up to the right ischiorectal fossa. It is causing displacement of the uterus, cervix and the upper two thirds of the vagina to the left and insinuating into the right lateral fornix. No intrauterine/ intravaginal component noted. Presacral extension of the lesion is seen which is heterogenous. Similar heterogeneity is seen in the lower pelvic anterior component. Both ovaries are seen separately from the lesion. MRI findings are of a fairly large fat containing pelvic lesion with significant inferior extension. Liposarcoma is to be considered.’

Figure 1. MRI Appearance of the tumor.

On clinical examination, a mass protruding from the right lateral vaginal wall was seen. Its surface was smooth and covered with normal vaginal epithelium. On per vaginum examination, the mass was firm, painless and compressible. It was found to be extending upwards under the right obturator fossa and behind the right pubic ramus closely related to it. It was closely related to the right side of the uterus, which was displaced to the left.  Rectal examination showed the rectal mucosa to be free, and the mass to be on its right side. No obvious swelling over the back seen.

Figure 2. Speculum examination showing the mass projecting into the vagina from the right side.

Her Pap smear was normal. An ultrasonographic scan was obtained, which showed 10.3x7.2 cm sized soft tissue mass near the right adnexa going towards the right paravesical region up to the anterior abdominal wall, displacing the bladder to the left. It was isoechoic to fat.

The patient was referred to gastrointestinal surgery department for the further management of the retroperitoneal tumor. A computed tomography (CT) showed a 20x14x13 cm mass extending up to right ischiorectal fossa, presacral region and anterior abdominal wall. Fat planes were maintained around the mass. Multile mesenteric lymph nodes measuring less than 1 cm in diameter were seen. A CT guided biopsy was obtained. However the sample was inadequate and no diagnosis could be made, though angiomyoliposarcoma was suspected. So she was referred to an oncology center for neoadjuvant radiotherapy and chemotherapy and was to be scheduled for surgery later. She underwent an exploratory laparotomy and excision of the mass without any prior radio- or chemotherapy. The mass could be excised completely, including the part projecting into the vagina. The urinary bladder was opened accidentally during the surgery. It was repaired after placing a DJ stent in the right ureter. The patient made and uneventful recovery. She was prescribed tamoxifen 20 mg q12h for 9 months. The DJ stent was removed after 1.5 months. She was well 3 months after that.

Histopathological examination of the mass showed a nonencapsulated spindle cell tumor with fibrosis in the background and fat entrapment. Nuclear  pleomorphism  and  atypical  mitoses were  absent. Immunohistochemistry showed the tumor cells to be diffusely positive for SMA, focally for Desmin, and negative  for beta catenin. A diagnosis of inflammatory myofibroblastic tumor was made.

The unusual presentation in this case could be easily confused with a vulval lipoma. Like majority of the patients with retroperitoneal tumours, our patient never had any urinary symptoms or any other pressure symptoms. The interesting part was the extension of the tumour from the retroperitoneal compartment to the anterior pelvic compartment thus presenting as a swelling in the vagina.

Discussion 

Retroperitoneal tumors always present as large asymptomatic masses, nearly 50% are >20 cm at the time of diagnosis.  Very rarely will they present pain, swelling and edema due to impingement on the bone or neurovascular bundle. Our patient had a large mass, which was associated with a little dragging pain. IMT is usually arising from an important organ which can be damaged during removal of the tumor. In our case the urinary bladder was injured. But despite its location in the retroperitoneum passing close to the uterus and ureter in the right broad ligament and close to the bladder, the ureter was not involved and was not injured during the surgery. The vaginal extension of the mass could be removed entirely by the abdominal approach. Based on the MRI and USG report, the tumor in this case was initially thought to be a lipoma, which could have been a lipoma, angiolipoma, fibrolipoma, myelolipoma or spindle cell lipoma. It could also have been a liposarcoma. IMT is a not common condition and is not thought of in the differential diagnosis of retroperitoneal masses. In the case presented it turned out to be IMT. A preoperative diagnosis is important in management of the tumor. The features are quite distinctive.[6,7,8]

The diagnosis could have been made preoperatively by obtaining a biopsy of the vaginal mass quite easily. However the surgeons were reluctant to do so, fearing a breach in the surface of the mass and metastases. 

Surgery remains the mainstay for the treatment of IMT. Medical treatment may be useful as adjuvant therapy. Prolonged follow up is essential to detect a recurrence or metastases early.[9]

References

1. Biselli R, Boldrini R, Ferlini C, Boglino C, Inserra A, Bosman C. Myofibroblastic tumors: neoplasias with divergent behavior. Ultrastructural and flow cytometric analysis. Pathol Res Pract. 1999;195:619–632.
2. Freeman A, Geddes N, Munson P, Joseph J, Ramani P, Sandison A, et al. Anaplastic lymphoma kinase (ALK 1) staining and molecular analysis in inflammatory myofibroblastic tumors of the bladder: a preliminary clinicopathological study of nine cases and review of the literature. Mod Pathol. 2004;17:765–771.
3. Tambo M, Kondo H, Kitauchi T, Hirayama A, Cho M, Fujimoto K, et al. A case of inflammatory myofibroblastic tumor of the retroperitoneum. Hinyokika Kiyo. 2003;49:273–276.
4. Esmer-Sanchez D, Rangel D. Inflammatory pseudotumor of the retroperitoneum. Rev Gastroenterol Mex. 2002;67:97–99.
5. Coffin CM, Watterson J, Priest JR, Dehner LP. Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinic pathologic and immuno-histochemical study of 84 cases. Am J Surg Pathol. 1995;19:859–872.
6. Facchetti F, De Wolf Peeters C, De Weber I, Frizzera G. Inflammatory pseudotumor of lymph  nodes: immunohistochemical evidence for its fibrohistiocytic nature. Am J Pathol 1990;137:281–289.
7. Coyne JD, Wilson  G, Sandhu D, Young RH. Inflammatory pseudotumour of the urinary bladder. Histopathology 1991;18:261–264.
8. Ramachandra S, Hollowood  K, Bisceglia M, Fletcher CD. Inflammatory pseudotumour of soft tissues: a clinicopathological and immunohistochemical analysis of 18 cases. Histopathology 1995;27:313–323.
9. Gleason BC, Hornick JL. Inflammatory myofibroblastic tumours: Where are we now. J Clin Pathol. 2008;61:428–37.

Citation

Niphadkar M, Parulekar SV. Retroperitoneal Inflammatory Myofibroblastic Tumour Presenting As A Vaginal Swelling. JPGO 2016. Volume 3 No. 10. Available from: http://www.jpgo.org/2016/10/retroperitoneal-inflammatory.html

Retroperitoneal And Pelvic Fibromatosis

Author Information

Parulekar SV*

(*  Professor and Head. Department of Obstetrics and Gynecology, Seth G. S. Medical College and KEM Hospital, Mumbai, India.)

Abstract

Retroperitoneal fibrosis (RPF) is a rare disorder in which there is extensive retroperitoneal inflammation and fibrosis. It usually is over the lower two lumbar vertebrae, and spreads upwards. Retroperitoneal and pelvic fibroamatosis (RPPF) is a form of intraabdominal fibromatosis which is a slowly growing mass that may be asymptomatic or may cause pressure effects on adjacent structures. A case of generalized RPPF is presented, which was mistaken for RPF due to its generalized state rather than being a localized mass.

Introduction

Albarran first described RPF in 1905. It is a rare disorder characterized by extensive chronic retroperitoneal inflammation and fibrosis, which causes obstruction of retroperitoneal structures like ureters, inferior vena cava, mesenteric vessels, and duodenal choledochal duct. It is often idiopathic, though an association with a number of conditions has been described. An immunological basis for RPPF is quite likely.[1,2] It is usually found mainly over the front of the fourth and fifth lumbar vertebrae. RPPF is a deeply situated fibroproliferative process in the retroperitoneum and pelvis, the cells being bland. Cellularity is variable. There are large areas of dense fibrous tissue. A case of RPPF mimicking RPF clinically is presented.

Case Report

A 26 year old primigravida was referred to us from a private hospital for management of an unruptured ectopic pregnancy diagnosed with ultrasonography (USG). She had 1.5 months of amenorrhea. There was no pelvic or abdominal pain or bleeding per vaginum. Her past medical and surgical history was not contributory. Her vital parameters were in normal range. General, systemic, and abdominal examination showed no abnormality. A speculum examination showed normal cervix and vagina. Bimanual pelvic examination showed tenderness in the right fornix, a 6 weeks’ size uterus, and a 2 cm x 1 cm tender, soft, smooth-surfaced mass in the right fornix. The vagina felt normal An USG done at our hospital showed 1.8 x 2.0 cm sized right adnexal ectopic pregnancy and a 3x2 cm right simple ovarian cyst. There was no free fluid in the pelvis. Her urine pregnancy test was positive. Her serum β-HCG was 5296 mIU/ml. It was repeated after 48 hours, when it was 5319 mIU/ml. She opted for methotrexate therapy. She was given methotrexate in a dose of  Methotrexate 50 mg/m² intramuscularly. Unfortunately she developed an acute abdominal pain 6 h later. An USG done at that time showed moderate hemoperitoneum. An exploratory laparotomy was done and the ruptured ectopic pregnancy was removed, after dissection from right sided tubo-ovarian mass. She made an uneventful recovery. Histopathological examination confirmed the diagnosis of tubal ectopic pregnancy. The patient was discharged on 5th postoperative day. She presented after 4 weeks with acute pain in right side of the lower abdomen. Her vital parameters were normal at that time. Abdominal examination showed no abnormality. Bimanual examination showed narrowing of the vagina from all sides, and relative fixity of the uterus. There was no pelvic tenderness or mass. A cervical swab was sent for microbiologic study and she was administered cefotaxime, gentamicin, and metronidazole parenterally, considering infection as the most likely diagnosis even in the absence of any fever or tenderness. There was no growth of any bacteria from the cervical swab. Her pain subsided after one day. A repeat examination after administration of the antibiotics for 5 days showed similar pelvic findings. A RPF was suspected and a contrast enhanced computerized tomography (CECT) was performed. It showed extensive fibrosis in the pelvis, lower retroperitoneum, and a hydronephrosis on the right side. The patient was referred to a urologist for further management. A double-J stent was passed on the right side cystoscopically, extending between the right renal pelvis and urinary bladder. A computed tomography guided biopsy was attempted, but did not yield adequate tissue. A laparotomy was done, but the entire pelvic structures were found to be involved in extensive fibrosis, and no structure could be identified clearly. A biopsy was obtained and the abdomen was closed. Histopathology of the specimen showed bland spindle-shaped cells in dense collagenous stroma. There was no evidence of any inflammation.  No mitotic figures or atypia was seen. A diagnosis of RPPF was made. Since surgical excision of such widespread tissue was not possible, the patient was offered tamoxifen therapy, which she accepted. Her hydronephrosis has resolved and she is currently asymptomatic, but longer follow-up is required to draw any meaningful conclusion about her therapy.

Figure 1. CT Scan showing extensive perivaginal fibrosis (arrows).

Figure 2. CT Scan showing right hydronephrosis (arrow).

Discussion

A patient with RPF presents with symptoms like poorly localized, dull pain in the back, flank, or lower abdomen,[3] fever, edema feet, malaise, anorexia, nausea, vomiting, and loss of weight. Some patients have deep vein thrombosis, intermittent claudications, Raynaud’s phenomenon, and ureteric colic. Association is known with ulcerative colitis, Crohn's disease, and sclerosing cholangitis. It is found to develop around an atherosclerotic aorta in response to the antigens of atherosclerotic plaques.[4] It may be secondary to infection, malignancy, retroperitoneal injury, or exposure to drugs like methyldopa, beta-blockers, and methysergide.[5,6] Genetic factors may be responsible for the development of RPF, as seen with the presence of human leukocyte antigen (HLA)–B27 cell marker in these patients.[7] Obstruction of retroperitoneal structures can be seen on excretory urography, contrast enhanced computerized tomography (CECT) of the abdomen, ultrasonography (USG) of the abdomen, and magnetic resonance imaging (MRI) of the abdomen.[8-10] A biopsy of the retroperitoneal tissue is diagnostic, the sample being obtained by laparoscopy or laparotomy. Early cases show an inflammatory infiltrate containing mainly lymphocytes, lipid-laden macrophages, and plasma cells amid numerous blood vessels. Late cases show acellular and avascular tissue with extensive fibrosis and scattered calcification.[11] Immunohistochemistry confirms the diagnosis.

RPPF occurs in women in the age group 15 to 40 years, most often between 20 and 30 years. As its origin is in the musculo-aponeurotic tissues, it can arise anywhere in the body. Lower genital tract is a rare site. Most common sites are abdominal wall, surgical scars, retroperitoneum and pelvis.[12-15] Causative factors are thought to be trauma (including surgery), Gardner’s syndrome, drugs and irradiation.[16-18] Most of the patients are asymptomatic, though some of them may present with some pain in the abdomen. Gastrointestinal bleeding or obstruction may be the presenting feature. Initial growth is often rapid, as was found in our case. Ureteric compression may lead to hydroureter and hydronephrosis, as was found in our case. Usually the ureteric involvement is unilateral. But considering the widespread fibrosis, the other ureter was considered likely to get involved some time or other in our case. Radical surgery is the most effective form of treatment known for RPPF.[19] The condition is believed to be hormone dependent, as it occurs mainly in fertile women in the reproductive age group. Presence of high affinitiy antiestrogen binding sites have been described on RRPF.[20] There are many reports of patients responding to antiestrogen tamoxifen.[21] Cytotoxic chemotherapy, NSAIDs, steroids, and interferons are also believed to be useful in the management of RPPF.[22-26] Since recurrence is likely to occur despite complete excision with disease-free margins, prolonged follow-up is required in cases of RPPF.

The case presented here was unique in that the extravaginal pelvic fibromatosis developed rapidly in a period of three weeks. It was accompanied by abdominal colic, which was possibly due to ureteric obstruction. Pelvic fibromatosis was extensive, and enveloped the vagina on all sides, but did not affect the urethra or the bladder neck. It developed soon after a laparotomy and salpingectomy for an ectopic pregnancy. However the surgery was brief and did not involve more than minimum tissue handling. So it cannot be stated with certainty that RPPF was due to the surgery. Normally the condition is diagnosed on investigations of varied symptoms, but is not detected clinically because the retroperitoneum cannot be palpated. In the case presented it was detected clinically because the vagina was easily accessible for examination. Since the patients of RPPF usually present to surgeons and/or urologists, a vaginal examination is usually not performed on them. It is possible that a number of such patients have findings of RPPF. It is recommended that all patients with RPPF should undergo a bimanual pelvic examination to see if the pelvis is involved, not only because it would be of interest, but also to detect if it causes bladder neck or urethral obstruction.

References

1. Katz SM, Bates O, Yudis M, et al. Immune complex glomerulonephritis in a case of retroperitoneal fibrosis. Am J Clin Pathol. 1977 May. 67(5):436-9.
2. Carton RW, Wong R. Multifocal fibrosclerosis manifested by vena caval obstructions and associated with vasculitis. Ann Intern Med. 1969 Jan. 70(1):81-6.
3. Yachoui R, Sehgal R, Carmichael B. Idiopathic retroperitoneal fibrosis: clinicopathologic features and outcome analysis. Clin Rheumatol. 2016 Feb. 35 (2):401-7.
4. Mitchinson MJ. Chronic periaortitis and periarteritis. Histopathology. 1984 Jul. 8(4):589-600.
5. Lepor H, Walsh PC. Idiopathic retroperitoneal fibrosis. J Urol. 1979 Jul. 122(1):1-6.
6. Higgins PM, Aber GM. Idiopathic retroperitoneal fibrosis--an update. Dig Dis. 1990. 8(4):206-22.
7. Astudillo L, Alric L, Jamard B, Laroche M. Retroperitoneal fibrosis in an HLA-B27-positive patient. Rev Med Interne. 1999 Dec. 20(12):1149-50.
8. Kottra JJ, Dunnick NR. Retroperitoneal fibrosis. Radiol Clin North Am. 1996 Nov. 34(6):1259-75.
9. Arrive L, Hricak H, Tavares NJ, Miller TR. Malignant versus nonmalignant retroperitoneal fibrosis: differentiation with MR imaging. Radiology. 1989 Jul. 172(1):139-43.
10. Rubenstein WA, Gray G, Auh YH, Honig CL, Thorbjarnarson B, Williams JJ, et al. CT of fibrous tissues and tumors with sonographic correlation. AJR Am J Roentgenol.  1986; 147(5):1067-74.
11. Mitchinson MJ. The pathology of idiopathic retroperitoneal fibrosis. J Clin Pathol. 1970 Nov. 23(8):681-9.
12. Kim Y-W, Choi SJ, Jeon UB, Choo KS. Retroperitoneal fibromatosis presenting as a presacral mass. Acta Radiol Short Rep 2014; 3: 1–4.
13. Ooi BS, Lee CN, Ti TK, Chachlani N, Chua ET. Retroperitoneal fibromatosis presenting as acute duodenal obstruction. ANZ J Surg 2001; 71: 74–6. CrossRef, Medline, ISI
14. Szklaruk J, Tamm EP, Choi H, Varavithya V. MR imaging of common and uncommon large pelvic masses. Radiographics 2003; 23: 403–24. CrossRef, Medline, ISI
15. Casillas J, Sais GJ, Greve JL, Iparraguirre MC, Morillo G. Imaging of intra- and extraabdominal desmoid tumors. Radiographics 1991; 11: 959–68.
16. Li Destri G, Ferraro MJ, Calabrini M, Pennisi M, Magro G, et al. Desmoid-type fibromatosis of the mesentery: report of a sporadic case with emphasis on differential diagnostic problems. Case Rep Med 2014; 2014: 1–7.
17. Kikkawa A, Kido A, Kumai T, Hoshida T. Extraabdominal fibromatosis in retroperitoneal space. World J Surg Oncol 2004; 2: 33.
18. Rodriguez-Bigas MA, Mahoney MC, Karakousis CP, Petrelli NJ. Desmoid tumors in patients with familial adenomatous polyposis. Cancer 1994; 74: 1270–4.
19. Cormio G1, Cormio L, Marzullo A, Pansini F, Traficante A, Selvaggi FP, Selvaggi L. Fibromatosis of the female pelvis. Ann Chir Gynaecol. 1997;86(1):84-6.
20. Lim CL, Walker MJ, Mehta RR et al. Estrogen and antiestrogen binding sites in desmoid tumors. Eur J Cancer Clin Oncol1986; 22: 583–587.
21. Kinzbrunner B, Ritter S, Domingo J et al. Remission of rapidly growing desmoid tumors after tamoxifen therapy. Cancer1983; 52: 2201–2204.
22. Mukherjee A, Malcolm A, De la Hunt M et al. Pelvic fibromatosis (desmoid)—treatment with steroids and tamoxifen. Br J Urol1995; 75: 559–560.
23. Izes JK, Zinman LN, Larsen CR. Regression of large pelvic desmoid tumor by tamoxifen and sulindac. Urology1996; 47: 756–759.
24. Dominguez-Malagon HR, Alfeiran-Ruiz A, Chavaria-Xicotencatl P et al. Clinical and cellular effects of colchicine in fibromatosis. Cancer1992; 69: 2478–2483.
25. Weiss AJ, Lackman RD. Low-dose chemotherapy of desmoid tumors. Cancer1989; 64: 1192–1194.
26. Geurs F, Kok TC. Regression of a great abdominal desmoid tumor by interferon-α. J Clin Gastrenterol1993; 16: 264–265.

Citation

Parulekar SV. Retroperitoneal And Pelvic Fibromatosis. JPGO 2016. Volume 3 No. 10. Available from: http://www.jpgo.org/2016/10/retroperitoneal-and-pelvic-fibromatosis.html

Unicornuate Uterus With Functional Rudimentary Non-Communicating Right Horn

Author Information

Santoso BI, Hidayah GN, Surya R

(Obstetrics and Gynaecology Department, Faculty of Medicine Universitas Indonesia, Cipto Mangunkusumo Hospital, Jakarta – Indonesia)

Abstract

Mullerian tract anomaly is found in 2-3 % of women.  An 18 year old woman with recurrent dysmenorrhea was referred to us. Two years before referral, she presented to another consultant gynecologist with the same symptoms and at that time a laparotomy was performed because of suspected endometriosis. During surgery, unicornuate uterus with right rudimentary horn, with right hematometra was found and the hematometra was evacuated. Recently, dysmenorrhea symptoms recurred and the patient presented to us. On physical examination we found a uterine enlargement with no vaginal bulge. On ultrasound a unicornuate uterus with right rudimentary horn with a normal left uterine cavity and cervical canal was seen next to a hematometra and hematosalpinx on the right side. Intravenous pyelography revealed absence of the right kidney. We performed a right salpingectomy and excised the right uterine horn on laparotomy. On six months follow up, no recurrence of symptoms or pathology was found. The initial diagnosis was challenging due to rareness of Mullerian tract anomaly. In such a case, with history of conservative surgical treatment, we suggest a horn removal and salpingectomy.

Introduction

The prevalence of Müllerian tract anomaly is 2-3 %.[1] Disorder in the lateral and vertical fusion of the Müllerian duct could lead to Müllerian anomaly syndrome. The rareness and the wide variety of the Müllerian anomaly make the diagnosis difficult and challenging, thus making the management suboptimal. Our case was one such example.

Case Report

An 18 year old woman was referred to our center due to recurrent, progressing dysmenorrhea. Her menarche was at 14 years of age. Two years prior to referral to our center she had presented to a gynecologist with dysmenorrhea especially in the right lower quadrant. She was diagnosed as having an endometriotic cyst and underwent a laparotomy. During surgery the gynecologist found a right sided hematometra in an unicornuate uterus with right rudimentary horn. The hematometra was evacuated. After surgery her menstrual cycle was suppressed with medical treatment. She received a gonadotropin releasing hormone (GnRH) analogue for the first 6 months and then she was continued on an oral combined contraceptive for a further period of six months.  With resumption of her menstrual cycle after finishing her treatment, dysmenorrhea symptoms recurred and the patient was referred to our center.  

At physical examination an abdominal mass arising from the pelvis, 2 fingers below the umbilicus was detected. Vaginal examination was not done since the patient declined a history of intercourse. Rectal examination showed no vaginal bulge but an enlarged unicornuate uterus of 18 weeks of gestational age size was felt. On ultrasound examination an unicornuate uterus with right rudimentary horn was seen. The left horn of the uterus showed a normal uterine cavity and cervical canal, while the right horn was found to have a hematometra and a hematosalpinx on the right side.  A cervical canal was not visible in the right rudimentary horn and there was no apparent connection between the right and left horns. Our analysis was that the right hematometra and the right hematosalpinx correlated with an outflow tract obstruction from the right horn (figure 1). Right kidney was not visualized on ultrasound examination. Absence of the right kidney was confirmed by intravenous pyelography. 

Figure 1. Uterus Didelphys. Right sided hematometra in right rudimentary horn with agenesis of the right cervix. (Yellow arrow). Normal left uterine horn (pink arrow)

A laparotomy was performed. A hematometra of  20 x 12 x 10 cm was seen in the right uterine horn and a right hematosalpinx measuring 15 x 8 x 6 cm was found. The uterine corpus of the left horn, left tube and both ovaries were normal. The right uterine horn had no cervical part and it had no connection to the left uterine horn. The right horn was completely separated from the left unicornuate uterus by a well-established myometrium (figure 2). We performed a right salpingectomy and right horn was excised.

The histopathology result of the excised right rudimentary horn was chronic endometritis and adenomyosis. The right salpinx showed chronic salpingitis and hydrosalpinx with external endometriosis. The histopathological results also confirmed that macroscopically and microscopically, there was no cervical or vaginal part of the right horn.

The patient had an uncomplicated post-operative recovery and was discharged in good condition two days after surgery. On six month post-operative evaluation, there was no recurrent symptom, and no pathology was found in clinical or ultrasound examination. 

Discussion

We found right sided hematosalpinx, ipsilateral hematometra in a case of unicornuate uterus with right rudimentary horn, and ipsilateral renal agenesis. There was no cervical part on the right rudimentary horn. The right horn was completely separated from the left unicornuate uterus by a well-established myometrium. The diagnostic method used in our case was ultrasound imaging and intravenous pyelography. Others recommend magnetic resonance imaging and laparoscopy for establishing the diagnosis of Müllerian tract anomaly.[2,3,4] 

Figure 2. Schematic illustration of obstructed right unicornuate horn with hematometra, right ovary, right hematosalpinx and normal left uterine horn and ovary

The primary management target in our case was to relive symptoms, repair the anatomical malformation, and preserve fertility function. Since we were preoperatively informed through physical and ultrasound examination about the absence of a connection between the right horn and left unicornuate uterus, we knew that metroplasty was not an appropriate option as there was no connection with the left uterus. Considering the recurrence of symptoms after her first surgery and the pathologic findings, we decided to perform a right salpingectomy and removal of the right horn. This was also done in several other reports in which unicornuate uterus with hematometra in the rudimentary horn was found besides the ipsilateral hematosalpinx. The horn removal was also done in our case, along with salpingectomy.[2,3,4]

Conclusion

Our case was a right rudimentary horn hematometra and right hematosalpinx and normal left unicornuate uterus with right sided renal agenesis. As we found no complaint and no pathology on six month follow up, we suggest to perform a horn removal and salpingectomy for such case with recurrence of symptoms despite  previous conservative surgical treatment.

Acknowledgment

We thank  Mr. Jan-Paul W.R. Roovers, M.D., PhD, uro-gynecologist, who has reviewed this manuscript; and Dr. Aria Wibawa, OBGYN, fetomaternal consultant  for the excellent ultrasound imaging. 

References

1. Han B, Herndon CN, Rosen MP, Wang ZJ, Daldrup-Link H. Uterine didelphys associated with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. Radiol Case Rep. 2010;5(1):327.
2. Zapardiel I, Alvarez P, Perez-Medina T, Bajo-Arenas JM. Laparoscopic management of a cavitated noncommunicating rudimentary uterine horn of a unicornuate uterus: a case report. Journal of Medical Case Reports.2010;4:215.
3. Agarwal M, Das A, Singh AS. Dysmenorrhea due to a rare müllerian anomaly. Nigerian Journal of Clinical Practice.2011;14(3):377-9
4. Liatsikos SA, Tsikouras P, Souftas V, Ammari A, Prassopoulos P, Maroulis G, etal. Diagnosis and laparoscopic management of a rudimentary uterine horn in a teenage girl, presenting with haematometra and severe endometriosis: our experience and review of literature. Minim Invasive Ther Allied Technol. 2010;19(4):241-7.

Citation

Santoso BI, Hidayah GN, Surya R. Unicornuate Uterus with Functional Rudimentary non-Communicating Right Horn. JPGO 2016. Volume 3 No. 10. Available from: http://www.jpgo.org/2016/10/unicornuate-uterus-with-functional.html

Spontaneous Postpartum Paravaginal Hematoma

Author Information

Singhal N*, Madhva Prasad S**, Gupta AS***.

(* Third Year Resident, ** Assistant Professor, *** Professor, Department of Obstetrics and Gynecology, Seth G.S Medical College and K.E.M. Hospital, Mumbai, India.)

Abstract

Paravaginal hematoma is a rare complication of vaginal delivery that can occur during delivery, just after delivery or late in puerperium. Various predisposing factors such as precipitate labor, instrumental delivery and trauma have been suggested. We report an interesting case of spontaneous asymptomatic paravaginal hematoma that developed in a woman who had a full term normal delivery.

Introduction

Paravaginal hematoma is a rare complication of vaginal delivery. It can be life threatening if not recognized and managed quickly as the patient can lose a large amount of blood in a short time. We report a patient who was incidentally diagnosed with this complication after undergoing a full term normal delivery.

Case Report

A 25 year old para 3 living 3 presented to the outpatient department (OPD) for postnatal follow-up on day 22 of full term normal delivery. She gave non-specific complaints of difficulty in micturition but no other specific complaints. She did not give history of excessive bleeding per vaginum, foul smelling discharge or any bowel disturbances. There was no history of sexual intercourse after delivery.

On general examination her pulse was 70 beats per minute, blood pressure was120/70 mm of Hg and there was no pallor. Systemic examination was unremarkable. Abdomen was soft and no masses were palpable.  On local examination, external genitalia appeared normal and no swelling or redness was seen. On speculum examination a mass arising from the right vaginal wall, reaching and almost encircling the introitus was seen. It was bluish in color.  The cervical os could not be visualized. There was no bleeding or lochia. On vaginal examination, a mass was felt arising from right lateral wall of the vagina about 7 x 7 cm, which was non-tender, firm to cystic in consistency, with restricted mobility, and occupying the pouch of Douglas, but not extending anteriorly.  It was not possible to reach the upper edge of the swelling. The uterus was not felt separately, was about 8-10 weeks in size and was slightly deviated to the left side. The left fornix was free.. 

She was admitted and her case records were reviewed. She had a normal antenatal course. Her previous 2 term pregnancies were uneventful. Registration hemoglobin (Hb) was 13.1 gram % and pre-labor Hb value was 12.4 gram%. Labor onset was spontaneous at 38 weeks of gestation. First stage of labor lasted for 5 hours and second stage lasted for 30 minutes. No intervention was required. There was no evidence of precipitate labor. No episiotomy was given. There was no fundal pressure applied and a male child of 2.75 kg was born with apgar score of 9/10. Placenta delivered spontaneously and completely. Uterus was well contracted and there was no evidence of abnormal blood loss. There was no vaginal or perineal tear. She had no difficulty in micturition and defecation. She was discharged on day 4 of delivery.

Currently, she was admitted for evaluation of the hematoma. Bed side coagulation tests were normal and laboratory coagulation profile was within normal limits. Hemoglobin was 10.1 gram%. These tests were monitored serially and remained normal. Urine and stool examination were normal. Vaginal swab culture also did not show any growth. Ultrasonography (USG) was suggestive of organized hematoma in the right adnexal region extending upto the perineal region, with a volume of around 120 cc. Magnetic resonance imaging (MRI) of the pelvis was done. It was suggestive of approximately 10 x 8 x 8 cm sized organized hematoma postero-inferior to the uterus and inferiorly it was extending to the pelvic floor. No uterine injury was seen (figure 1). 

Figure 1. MRI of the pelvis. U is the uterus, Cx is the cervix and H is the hematoma.

She was started on parenteral antibiotics (piperacillin-tazobactam, metronidazole and gentamicin). In view of her stable condition and no specific symptomatology, she was managed conservatively. She followed up in the OPD fortnightly. She remained symptom free. USG was repeated. At 2 months post-delivery, the volume of the hematoma had reduced to around 75 cc (figure 2) and at 4 months post-delivery, the volume had reduced to 30cc. She was advised to follow up in future if required. She did not report any problems with sexual intercourse, which she resumed around 3 months after delivery.

Figure 2. USG pelvis. H is Hematoma. U is uterus.

Discussion

The incidence of puerperal hematomas has been reported to range from 1 in 300 to 1 in 1000 deliveries.[1] In a retrospective observational study conducted by Genre et al, the estimated incidence of pelvi-abdominal hematomas was 1/4200 deliveries.[2] Primigravid status, episiotomy, instrumental delivery, hypertensive disorders , multifetal gestation, large fetal size, obstructed labor, use of uterine fundal pressure in second stage of labor and vulvo-vaginal varices have all been described as high risk for puerperal hematomas.[3,4,5]

The presentation of an episiotomy hematoma can be catastrophic, wherein the patient may present within few hours of delivery with features of shock and collapse. Local examination would generally reveal a massive tender swelling in the vulvo-vaginal area. Inability to achieve hemostasis in the episiotomy suturing is the most important cause. Classically, there is vaginal bleeding in this condition. In anatomical parlance, this qualifies as an “infra-levator” hematoma. When a paravaginal “supra-levator” hematoma occurs, a broad ligament hematoma usually forms and massive collection of blood in the retroperitoneum results. This occurs in the absence of any vaginal bleeding. Such a hematoma occurs due to avulsion of paragenital connective tissue. The condition is also characterized by deviation of the uterus to the contralateral side. The condition may be slow in evolution, depending on the vessels involved. The blood loss can be extensive; and the extensive vascularity of the uterus contributes to this. Rupture of various vessels supplying the genital organs has been implicated in various individual cases in medical literature. Rupture of the uterine artery after a spontaneous delivery necessitating laparotomy has been described by Becker et al.[6] Extensive Retzius space hematoma following spontaneous delivery has been reported by Fieni et al.[7] Spontaneous rupture of an ovarian artery aneurysm causing massive retroperitoneal hemorrhage has been reported by Enakpene et al recently and Guillem et al previously.[8,9] Psoas muscle hematoma following an uncomplicated vaginal delivery has also been described.[10] Puerperal hematoma arising from rupture of internal iliac artery has been described by Egan et al.[11] It is clear that any of the extensive vasculature present in the paravaginal connective tissue can lead to the formation of such hematomas, and the presentation could be dramatic and in the immediate postpartum period. However, in our case the hematoma was detected only on day 22 of delivery when she came for a regular visit. It was detected during a routine follow up, on pelvic examination.  Such a late presentation has not been reported widely. This suggests a slowly developing venous bleed in the paragenital tissues. After admission she had no tachycardia, hypotension or anemia. Hemoglobin did not drop further.  This indicated that the hematoma had stabilized and was not expanding. A major vessel rupture was unlikely in our case, and the venous bleeding which took place was probably self-limited by its own pressure on the surrounding fixed structures and other natural hemostatic mechanisms. Her follow up as an outpatient showed the gradual resolution of the hematoma.

Management for puerperal hematomas include minimally invasive vascular interventional procedures. Temporizing measures such as tamponade using Bakri balloon have also been described.[12] Angiography followed by selective arterial embolization of the bleeding segment have been done.[13,14 ] Hemodynamically stable patients may be considered for ultrasonographic guided aspiration of the collection, as described by Mukhopadhay et al.[15] Inability to specifically identify the bleeding vessel and need for repeated procedures are drawbacks in this technique. 

However when such methods are employed, patient needs to be continuously monitored. In the event of further deterioration, the patient may require operative measures. The conservative operative measures include surgical evacuation of the hematoma, identification of any bleeding vessel, followed by ligation, step-wise devascularisation of the uterus. None of these procedures were required in our case. 

To conclude, this case is being presented to highlight that puerperal hematomas may occur even in the absence of any specific risk factors. These may present late, remain asymptomatic and may be amenable to conservative management. 

References

1. Cunningham FG, Obstetrical hemorrhage. In Cunningham FG, Leveno KJ, Bloom SL, Spong CY, Dashe JS, Hoffman BS, editors. Williams’ Obstetrics. 24th ed. New York: Mc Graw hill. 2014; pp. 783.  
2. Genre L, Tanchoux F, Parant O. Pelvi-abdominal hematoma: a five years study. Eur J Obst Gyn Rep Biol. 2012 May; 41(3):290–7. 
3. Riethmuller D, Pequegnot-Jeannin C, Rabenja CA, Koeberle P, Schaal JP, Maillet R. A rare cause of postpartum hemorrhage: a genital thrombus. Eur J Obst Gyn Rep Biol. 1997; 26(2):154–8. 
4. Lowenstein L, Haddad L, Itskovitz-Eldor J, Sabo E, Jakobi P. [Episiotomy as a risk factor for early perineal trauma]. [Article in Hebrew]. Harefuah. 2005 Jun;144(6):389–93,456. Available from: http://www.ncbi.nlm.nih.gov/pubmed/15999555
5. Wei S-C, Chen C-P. Uterine rupture due to traumatic assisted fundal pressure. Taiwan J Obstet Gynecol. 2006 Jun; 45(2):170–2. 
6. Becker R, Kowalsky BL, Hatzmann W. Rupture of the uterine artery after spontaneous delivery with unusual course in childbed. J Obst and neonatol. 2002 May-Jun; 206(3):107–13. 
7. Fieni S, Berretta R, Merisio C, Melpignano M, Gramellini D. Retzius’ space haematoma after spontaneous delivery: a case report. Acta Biomed. 2005 Dec; 76(3):175–7. 
8. Enakpene CA, Stern T, Salazar MJB, Mukherjee P. Spontaneous Rupture of an Ovarian Artery Aneurysm: A Rare Postpartum Complication. Case Rep Obstet Gynecol [Internet]. 2016;2016:1029561. Available from: http://www.ncbi.nlm.nih.gov/pubmed/27034862 
9. Guillem P, Bondue X, Chambon JP, Lemaitre L, Bounoua F. Spontaneous retroperitoneal hematoma from rupture of an aneurysm of the ovarian artery following delivery. Ann Vasc Surg. 1999 Jul; 13(4):445–8. 
10. Rafi J, Muppala H. Conservative management of massive puerperal spontaneous onset retroperitoneal psoas muscle haematoma following normal vaginal delivery. J Obstet Gynaecol. 2008 Jan; 28(1):105–6. 
11. Egan E, Dundee P, Lawrentschuk N. Vulvar hematoma secondary to spontaneous rupture of the internal iliac artery: clinical review. Am J Obstet Gynecol. 2009 Jan;200(1):e17-8. 
12. Gizzo S, Saccardi C, Patrelli TS, Di Gangi S, D’Antona D, Battista Nardelli G. Bakri balloon in vaginal-perineal hematomas complicating vaginal delivery: a new therapeutic approach. J Low Genit Tract Dis. 2013 Apr; 17(2):125–8. 
13. Distefano M, Casarella L, Amoroso S, Di Stasi C, Scambia G, Tropeano G. Selective arterial embolization as a first-line treatment for postpartum hematomas. Obstet Gynecol. 2013 Feb; 121(2 Pt 2 Suppl 1):443–7. 
14. Lee SL, Kim YH, Lee HJ. Selective angiographic embolisation of an infralevator vulvovaginal haematoma after birth: case report. J Obstet Gynaecol. 2015; 35(6):639–40.
15. Mukhopadhyay D, Jennings PE, Banerjee M, Gada R. Ultrasound-Guided Drainage of Supralevator Hematoma in a Hemodynamically Stable Patient. Obstet Gynecol . 2015 Dec; 126(6):1188–90.

Citation

Singhal N, Madhva Prasad S, Gupta AS. Spontaneous Postpartum Paravaginal Hematoma. JPGO 2016. Volume 3 No. 10. Available from: http://www.jpgo.org/2016/10/spontaneous-postpartum-paravaginal.html

Intrauterine Foreign Body Or Osseous Metaplasia Of The Endometrium

Author Information

Singhal N*, Madhva Prasad S**, Gupta AS***.

(* Third Year Resident, ** Assistant professor, *** Professor, Department of Obstetrics and Gynecology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai, India.)

Abstract

Intrauterine foreign body is a rare complication and can occur after late first or second trimester abortion. The calcified fragment may work as an intrauterine contraceptive device causing infertility or may cause menstrual disturbances or pelvic pain. Hysteroscopic removal of the foreign body can be done or it may be removed by curettage. A case of secondary infertility was diagnosed to have a foreign body in the uterine cavity that may have been left behind after previous second trimester termination of pregnancy.

Introduction

Many causes of secondary infertility exist. Uterine factors that may be associated with infertility are submucous fibroids, fibroid polyps, and uterine malformations. Rarely, retained foreign bodies may prevent endometrial implantation. In patients with previous history of abortion, retained bony products can get impinged into the endometrium. 

Case Report

A 27 year old woman cohabiting since 5 years with one previous abortion presented in the gynecology out-patient department (OPD) with infertility.  Her only pregnancy was a spontaneous conception 4 years prior. At 16 weeks of gestation in view of malformation incompatible with life, the pregnancy was terminated by an aspirotomy procedure, followed by uterine curettage. She had regular menstrual cycles and no major complaints apart from inability to conceive. 

Detailed physical examination was done and there were no contributory findings. Abdominal examination was unremarkable. Speculum examination showed normal cervix and vagina. Uterus was normal in size, shape and mobility. Previous notes were assessed and uterine curettage procedure appeared to have been uneventful. Hormonal profile was normal, semen analysis parameters were normal and there were no coital problems. 

Trans-vaginal ultrasound showed normal size and shape of the uterus with a well-defined focal hyperechogenicity of 1.5 x 1.5 cm and shadowing suggestive of calcification/ foreign body. Computed tomography (CT) of the pelvis was done. It was suggestive of 1 x 0.9 x 0.94 cm size calcified density in the uterine endometrial cavity suggestive of foreign body or an old calcified clot (figure 1 and figure 2). 

Pre operative evaluation was done and all parameters were normal. A diagnostic hysteroscopy and laparoscopy was performed. On hysteroscopy, uterine cavity was normal, bilateral cornuae were visualized and endometrium appeared normal. A 1.5 x 1.5 cm sized calcified foreign body was seen in right anterolateral wall of the uterus close to the uterine fundus (figure 3). This was removed by curettage and sent for histopathological examination (figure 4). Endometrial curettage was also sent for histopathological examination. On laparoscopy, uterus, bilateral fallopian tubes and ovaries appeared normal. On chromopertubation, incidentally bluish discoloration of the myometrium was noted near the left cornual area probably suggestive of previous endometrial breach. However, bilateral free spill was seen. Patient was discharged on day 2 with advice to take oral ethinyl estradiol 0.05 mg twice a day for one cycle and then to undergo follicular monitoring with planned relation.

Histopathology of the curettage showed endometrial glands with compact fibrosing stroma with evidence of extensive calcification suggestive of osseous metaplasia in the endometrium. However, surprisingly, the other fragment of foreign body which was sent for histopathological examination (figure 4) could not be proven as human tissue and was reported as such and was discarded.

Figure 1. Sagittal section of CT image with arrow showing area of intrauterine calcification.

Figure 2. Coronal section of CT image with arrow showing area of intrauterine calcification.

Figure 3. Hysteroscopic image with arrow showing intrauterine foreign body.

Figure 4. Foreign body after removal. 

Discussion

A varied range of conditions can cause secondary infertility. Ultrasonography (USG) is one of the primarily employed radiological modalities in the evaluation of infertility. Initially, our case was evaluated as per the standard protocol for infertility evaluation, wherein the unusual finding of intrauterine calcification was noted on USG. Such a finding could be suggestive of a foreign body, intrauterine contraceptive device, Asherman syndrome, or a calcified fibroid.[1] Rare disorders like heterotopic bone/ osseous metaplasia can also result in this finding.[2] Less than 100 cases of uterine cavity calcification due to various causes have been reported in current medical literature. A history of antecedent abortion was present in around 75% of the patients studied and almost 90% of the patients were identified with USG.[3] The symptomatology of such calcification depends on the underlying pathology. The order of frequency of presentation is infertility, menstrual abnormalities and pelvic pain. [4] Our patient had only symptoms of infertility. She did not report dysmenorrhea, menstrual problems or pelvic pain. Though 76% of patients with intrauterine fetal bones have infertility, the contribution of intrauterine fetal bones to infertility is very meager. In a 6 year study period in West Africa only 11 women had intrauterine retention of fetal bones.[5] The contraceptive effect of such retained foreign bodies is similar to other intrauterine contraceptive devices resulting out of poor endometrial implantation and local release of prostaglandins.[6] Occasionally patient may have forgotten about a previous insertion of an intrauterine contraceptive device in the remote past.  If the threads get detached, the condition can result in a diagnostic dilemma, requiring hysteroscopic evaluation.[7] Hysteroscopy is the standard modality for management of this entity, both for confirmation and for operative removal.[8] Makris et al reported a series of 2000 patients who underwent hysteroscopy for evaluation of abnormal uterine bleeding. Of these, only 3 patients had intrauterine foreign body. This exemplifies the rare nature of this condition.[9]

In our patient, the location of the retained fragment was in the antero-lateral wall, close to the fundus. This location is similar to that reported in the initial descriptions of such cases, wherein Dajani et al had reported that fundus was the most common location where retention took place.[10]

Our patient underwent only hysteroscopy and curettage. However, intraoperative USG to ensure completeness of curettage has been suggested.[11] In our case there was no cause found for infertility apart from the presence of the foreign body, which was removed. However, we do not know whether this was responsible for her infertility. Restoration of fertility has been reported to be a common sequel after complete removal of the foreign body.  In their review article, Pereira et al have reported that 55% of the patients conceived after the procedure.[3]

Histopathology of the curettage showed endometrial glands with compact fibrosing stroma with evidence of extensive calcification suggestive of osseous metaplasia in the endometrium. We presume that the calcified piece that was removed was a foreign body (figure 4) as the pathologist who processed it reported it as not of human origin though retained fetal bone should have been reported as human tissue. Unfortunately they did not process it further and discarded it. It is possible that this foreign body may have stimulated osseous metaplasia of the endometrium or maybe this piece is not a foreign body but has broken from the osseous metaplasia that is seen in the endometrium. Endometrial osseous metaplasia is a known but rare cause of infertility.[12] Dystrophic calcification, heterotopia, ossification of post-abortion endometritis, metastatic calcification, and prolonged estrogen supplement after abortion have all been cited as explanations for the endometrial osseous metaplasia The most accepted theory regarding the source of the osseous tissue is metaplastic change of endometrial stromal cells into osteoblastic cells which produce bone.[13]

Many advances have taken place since publication of possibly the first similar article in modern literature.[14]

This case report is presented to highlight that the treating gynecologist should consider osseous metaplasia and retained foreign body as an important differential diagnosis when endometrial calcification is detected on USG, especially those with history of second trimester abortion. With the only existing pathology causing infertility being treated, it is assumed that the patient would conceive in the near future, and is being followed as an outpatient.  

References 

1. Topçu HO, Şimşek BŞ, Taşdemir U, Güzel Aİ, Doğanay M. Retention of fetal bones 8 years following termination of pregnancy. J Exp Ther Oncol. 2014; 10(4):267–9. 
2. Madaan M, Suman S, Sharma R, Kapoor N, Garg P, Raj SS. Osseous metaplasia of the endometrium and successful hysteroscopic resection: a report of two cases and a review of the literature. Asian J Endosc Surg. 2015 Feb; 8(1):63–6. 
3. Pereira MC, Vaz MM, Miranda SP, Araújo SR, Menezes DB, das Chagas Medeiros F. Uterine cavity calcifications: a report of 7 cases and a systematic literature review. J Minim Invasive Gynecol. 2014; 21(3):346–52. 
4. Samraj S, Crawford S, Singh N, Patel R, Rowen D. An unusual case of pelvic pain: retention of fetal bone after abortion. Int J STD AIDS. 2008 May;19(5):353–4. 
5. Graham O, Cheng LC, Parsons JH. The ultrasound diagnosis of retained fetal bones in West African patients complaining of infertility. BJOG. 2000 Jan; 107(1):122–4. 
6. Lanzarone VF, Pardey JM. Retained intrauterine fetal bone as a rare cause of secondary infertility. Aust N Z J Obstet Gynaecol. 2009 Dec;49(6):700–1. 
7. Samuel M, Kumar U, Parsons J, Ross J, Taylor C. Retained fetal bones following termination of pregnancy mistaken for an intrauterine contraceptive device. J Fam Plann Reprod Health Care. 2009 Jul; 35(3):195–6.
8. Goldberg JM, Roberts S. Restoration of fertility after hysteroscopic removal of intrauterine bone fragments. Obstet Gynecol. 2008 Aug; 112(2 Pt 2):470–2. 
9. Makris N, Stefanidis K, Loutradis D, Anastasiadou K, Hatjipappas G, Antsaklis A. The incidence of retained fetal bone revealed in 2000 diagnostic hysteroscopies. JSLS 2006;10(1):76–7. 
10. Dajani YF, Khalaf SM. Intrauterine bone contraceptive device: an accident of nature. Fertil Steril. 1985 Jan;43(1):149–50. 
11. Elford K, Claman P. Novel treatment of a patient with secondary infertility due to retained fetal bone. Fertil Steril. 2003 Apr;79(4):1028–30.
12. Garg D, Bekker G, Akselrod F, Narasimhulu DM. Endometrial osseous metaplasia: an unusual cause of infertility. BMJ Case Rep. 2015 Apr 1;2015. Available from: http://www.ncbi.nlm.nih.gov/pubmed/25837325.
13. Fernandes G, Patil A, Samant PY, Parulekar SV Endometrial Osseous Metaplasia. JPGO 2014 Volume 1 Number 8. Available from:  http://www.jpgo.org/2014/08/endometrial-osseous-metaplasia.html
14. Chervenak FA, Amin HK, Neuwirth RS. Symptomatic intrauterine retention of fetal bones. Obstet Gynecol . 1982 Jun;59(6 Suppl):58S – 61S.

Citation

Singhal N, Madhva Prasad S, Gupta AS. Intrauterine Foreign Body Or Osseous Metaplasia Of The Endometrium. JPGO 2016. Volume 3 No. 10. Available from: http://www.jpgo.org/2016/10/intrauterine-foreign-body-or-osseous.html

Fraser Syndrome, A Typical Case Detected Antenatally

Author Information

Chhatrapati A*, Nandu KR**, Nadkarni T***, Jassawalla MJ****

(* Assistant Professor, ** Fetal medicine consultant, *** Additional Professor, **** Prof, Medical Director. Department of Obstetrics and Gynecology, Nowrosjee Wadia Maternity Hospital, Mumbai, India.)

Abstract

Fraser syndrome is an autosomal recessive syndrome with multiple malformations. Cryptopthalmos, syndactyly (cutaneous), genito-urinary and laryngeal malformations are the major manifestations. Herewith presenting a case of antenatally diagnosed Fraser syndrome. The infants which are not severely affected can survive upto early childhood but majority die in utero or in early neonatal period. 

Introduction

Fraser syndrome was named as such by George Fraser in 1962 in 2 sisters affected by it at birth. It is a rare syndrome with an incidence of 0.043 per 10,000 live births and 1.1 in 10,000 still births.[1] It is an autosomal recessive syndrome characterized by variable phenotypic expression. It is caused due to mutation in the FRAS1 gene (607830) on chromosome 4q21, the FREM2 gene (608945) on chromosome 13q13 or the GRIP1 gene (604597) on chromosome 12q14.[2] It has a recurrence risk of 25% among siblings; therefore prenatal diagnosis is an important task for counseling of the affected families.[3] Prenatal diagnosis has been reported in less than 20 cases antenatally, to the best of our knowledge.[1,4]

Case Report

A 27 year old gravida 2 abortion 1 was referred to our institute in view of abnormal ultrasound findings at 19 weeks of gestation. She was in a consanguineous marriage (married to her cousin brother) and her husband had fathered both the pregnancies. A single prior second trimester pregnancy in 2005 was terminated at the same institute, in view of multiple malformations detected on ultrasound (USG) examination. There was scoliosis, crowding of the fetal parts and oligohydramnios on USG. The detailed USG report was not available. She had received anti D after the termination of pregnancy due to her Rh negative blood group status.

In the present pregnancy she was registered with a local practitioner at 6 weeks of gestation. The current pregnancy was a spontaneous conception. All her blood investigations were within normal range. She had missed her first trimester screening. Anomaly USG scan performed at 18 weeks of gestation was suggestive of congenital high airway obstruction (CHAO), bilateral congenital talipes equinovarus (CTEV) and unilateral renal agenesis. The fetal growth was consistent with her dates. Anatomic evaluation showed lungs that were enlarged and hyperechogenic with fluid bronchogram. The diaphragm was everted, trachea and main bronchus were grossly dilated. Fetal cardia was compressed in between the hyperinflated lungs. Heart was centrally located and structurally normal on real time imaging and color Doppler scanning. In addition to above there was unilateral renal agenesis, single umbilical artery and both legs exhibited bilateral severe CTEV.

Figure 1.  Image at the level of the 4 chamber heart showing bilateral hyperechoic lungs and centrally compressed placed heart.

Figure 2. Image showing unilateral renal agenesis (left side of the image) with bilaterally echogenic lungs and dilated, fluid filled trachea and bronchial tree (fluid bronchogram ) with inverted diaphragm.

Figure 3. USG image showing single umbilical artery

  

The genitalia revealed a phallus like structure and hence it was thought to be a male fetus. However abiding by the PCPNDT Act of the country the same was not disclosed to the couple. The examination of the intracranial structures and digits were unremarkable. In the presence of multiple malformation, consistently lethal CHAOS, consanguinity and previously affected sibling the parents were counseled about the possibility of a genetic abnormality and unfavorable prognosis in the index pregnancy. Parents decided to terminate the pregnancy. Autopsy was also offered. 

Macroscopic examination of the abortus revealed eyelids that were fused (partial cryptopthalmos), broad nasal bridge and hair line receding up to the corner of the brows. In addition to confirming the ultrasound findings the abortus also had imperforate anus and ambiguous genitalia. The external genitalia revealed phallus with fused labia. Scrotal sac was absent.

Figure 4.  Abortus showing fused eyelids.

Figure 5.  Abortus showing imperforate anus and fused labia

Figure 6.  Bilateral severe CTEV

An autopsy was done by the pathologist after the parents consented for the same. Autopsy showed hyperinflated, edematous lungs with rib markings. They were occupying most of the mediastinal space. Heart was compressed, trachea was dilated and the site of obstruction was at the level of the vocal cords in the larynx. In addition spleen was in form of accessory splenules interspersed in the mesentry and there was hepatomegaly.

Figure 7. Postmortem specimen showing hyperinflated, edematous lungs with prominent rib marking and compressed small centrally placed cardia. 

Discussion

Fraser syndrome was first reported by Zehender in 1872 as a syndrome with complex malformations and multi-system involvement including bilateral cryptophthalmos, syndactyly, hypertelorism, umbilical hernia, abnormal genitalia, anal stenosis and hoarseness of voice.[4] Similar cryptophthalmos syndromes were reported in several other cases.[5] In 1962 Fraser published a case of two siblings who exhibited cryptophthalmos, syndactyly, stenosis of the larynx, defects of the nose and the ear, laryngeal and genito-urinary malformations.[4] Since then the term Fraser syndrome has been used for similar malformation sequences involving multiple organ system, including cases with or without cryptophthalmos (cryptophthalmos spectrum). 

Thomas et al reviewed 124 cases of cryptophthalmos. He established the least required diagnostic criteria to differentiate Fraser syndrome from isolated cryptophthalmos.[5] The major criteria were cryptophthalmos, syndactyly, abnormal genitalia and positive family history and the minor criteria included congenital malformations of the nose, ears or larynx, cleft lip and / or cleft palate, skeletal defects, anterior abdominal wall defects, agenesis of either or both kidneys and mental retardation). Diagnosis is based on presence of at least 2 major and 1 minor criterion or 1 major and 4 minor criteria.[5] 

Van Haelst (2007) revised the diagnostic criteria described by Thomas due to the variable phenotypic expression. He added airway tract and urinary tract abnormality to major criteria and removed mental retardation and cleft lip and palate from the minor criteria.

Revised major criteria are syndactyly. (cutaneous), cryptophthalmos spectrum (hidden eye), urinary tract abnormalities, ambiguous genitalia, laryngeal and tracheal abnormalities, positive family history of affected sibling and the revised minor criteria are anorectal defects, dysplastic ears, skull ossification defects, umbilical abnormalities, nasal anomalies. The  uncommon deformities are cleft lip/ palate, cardiac malformations, musculoskeletal abnormalities, and mental retardation

For confirmation of diagnosis, 3 major, or 2 major and 2 minor, or 1 major and 3 minor criteria should be present.[6]

In our case as there was fulfillment of criteria the diagnosis was clear. However the variability of phenotype expression can make diagnosis challenging. Most of the literature search suggested the contrasting association between oligohydramnios which is seen with renal agenesis (which would otherwise be associated with hypoplastic lungs) and bilateral hyperechogenic lungs is highly suggestive sonographic marker of Fraser syndrome.[7] However as in our cases the presence of a single functioning kidney accounted for normal liquor levels. To our best knowledge less than 20 cases have been diagnosed prenatally.

The parental genetic testing for the carrier status could not be offered as tests are not available in India. Parents were counseled regarding the 25% risk of recurrence in the following pregnancies and the diagnosis of which could be suspected from 13 weeks of gestation by ultrasound. Although there is single reported case of affected individual alive upto 96 yrs;[8] there are no reported cases of affected individuals with reproductive capacity, to the best of our knowledge.

Conclusion

In conclusion, prenatal diagnosis of Fraser syndrome is possible with an expert sonologist, but due to the great variety of possible malformations the diagnosis will remain doubtful in most cases in which there is no positive family history of affected sibling. High index of suspicion needs to be exercised when multiple malformations are detected.

 Acknowledgment

Dr Umesh Athavale (radiologist, Nowrosjee Wadia maternity hospital), & Dr Jaya Deshpande (pathologist, Nowrosjee Wadia maternity hospital)

References 

1. Narang M, Kumar M, Shah D. Fraser-Crypto- phthalmos syndrome with colonic atresia. Indian Journal of Paediatrics.2008;75(2):89-91
2. Mc Gregor L, Makela V, Darling SM, Vrontou S, Chalepakis G, Roberts C, et al. Fraser syndrome and mouse blebbed phenotype caused by mutations in FRAS1/FrasI encoding a putative extracellular matrix protein. Nature Genet.2003;34:203-8
3. Ramsing M, Rehder H, Holzgreve W, Meinecke P, Lenz W. Fraser syndrome (cryptophthalmos with syndactyly) in the fetus and newborn. Clin Genet. 1990;37(2):84-96
4. Berg C, Geipel A, Germer U, Pertersen-Hansen A, Koch-Dörfler M, Gembruch U. Prenatal detection of Fraser syndrome without cryptophthalmos: case report and review of the literature. Ultrasound Obstet Gynecol. 2001;18(1):76-80.
5. Thomas IT, Frias JL, Felix V, Sanchez de Leon L, Hernandez RA, Jones MC. Isolated and syndromic cryptophthalmos. Am J Med Genet. 1986;25(1):85-98. 
6. Van Haelst MM, Scambler PJ, Fraser Syndrome Collaboration group, Hennekam R CM, Fraser syndrome: A clinical study of 59 cases and evaluation of diagnostic criteria. Am. J. Med. Genet. 2007; Part A; 143A; 3194-3203
7. Fryns JP, van Schoubroeck D, Vandenberghe K, Nagels H, Klerckx P. Diagnostic echographic findings in cryptophthalmos syndrome (Fraser syndrome). Prenat Diagn. 1997;17(6):582-4.
8. Impallomeni M, Subramanian D, Mahmood N, Illes J. Fraser syndrome in a 96-year-old female. Age Ageing.2006;35(6):642-3.

Citation

Chhatrapati A, Nandu KR, Nadkarni T, Jassawalla MJ. Fraser Syndrome, a Typical Case Detected Antenatally. JPGO 2016. Volume 3 No. 10. Available from: http://www.jpgo.org/2016/10/fraser-syndrome-typical-case-detected.html

Obstructed Labor In A Case Of Prune Belly Syndrome : A Rare Case Report

Author Information

Pardeshi S*, Swaminathan G**, Warke HS***, Mayadeo NM****.

(*Assistant Professor, **Third year resident, *** Associate Professor, **** Professor. Department of Obstetrics and Gynecology, Seth GS Medical College & KEM Hospital, Mumbai, India.)

Abstract

Obstructed labor carries a high risk of maternal morbidity and mortality and is prevalent in the developing world. Common causes include congenital fetal abnormalities like polycystic kidneys, hydrocephalus, hydronephrosis, locked twins, uterine abnormalities, contracted pelvis & maternal pelvic tumors. A case of Prune belly syndrome diagnosed immediately after delivery is presented here. It had led to obstructed labor. She was successfully managed by trans abdominal tapping of the cystic masses following which she delivered vaginally.

Introduction

Obstructed labor is uncommon in developed countries. Obstructed labor with a dead fetus is a reality and is known to cause maternal morbidity and mortality in developing countries. Various maternal and fetal causes have been identified.[1,2] Fetal urinary tract abnormalities can cause obstructed labor and in developing countries, many patients present in active labor with no previous antenatal visit. The anomalies are then detected only when they cause obstructed labor. 

Prune belly syndrome is a rare congenital disease which is characterized by a triad of urinary tract and abdominal wall muscle abnormalities, and bilateral cryptorchidism. It is also associated with genital, cardiovascular, respiratory and other musculoskeletal malformations.[3,4] 

Case Report

A 22 year old primigravida was referred from a rural hospital to our tertiary care hospital in view of obstructed labor with fetal head and upper limbs delivered out of the introitus. She, a migrant laborer, was completely unregistered & uninvestigated. She went to the nearby rural hospital with complaints of pain in abdomen one day prior to referral. First stage of labor was uneventful. However progress of labor in the second stage was arrested after the delivery of the fetal head and both upper limbs for which she was transferred to our tertiary care center for further management. She reached our center about 7 hours after obstruction.

On examination patient was dehydrated, pulse rate was 106 beats per minute and blood pressure was 110/70 mm of Hg. Patient’s heart sounds were normal and the chest was clear. Patient was in distress due to pain. Mild pallor was present. Per abdominally uterus was full term with fundal height of 38 cms. She was getting good uterine contractions. Fetal heart sounds were not heard with stethoscope. On local examination, fetal head and both the upper limbs were lying outside the introitus. 

Figure 1.  Fetal head and both the upper limbs lying outside the introitus

On vaginal examination, fetal head and hands were visible at the perineum. The cervix was fully dilated with the shoulders at station 0 to +1. In view of the diagnostic dilemma an ultrasound scan to ascertain the cause of obstructed labor was done. 

Ultrasound (USG) showed massive distension of fetal abdomen caused by multiseptate cystic masses. Probable diagnosis was fetal hydronephrosis and distended urinary bladder. Fetal heart beats were absent. Written consent was taken and ultrasound guided trans abdominal tapping of the cystic masses was done using wide bore spinal needle and IV drip set under all aseptic precautions. About 1.2 liters of fluid was drained. After decompression of the fetal abdomen, the fetal trunk delivered. Congenitally anomalous, male fetus with grossly distended and decompressed abdomen was still born.

Figure 2. Ultrasound showing multiseptate cystic masses

On detailed examination, multiple abnormalities consistent with Prune belly syndrome were noted. The abdomen was distended with prune-like appearance (wrinkled, loose skin) and absent anterior abdominal wall musculature. The male genitalia were abnormal with a small scrotum with cryptorchidism. The chest was considerably small, consistent with lung hypoplasia secondary to anhydramnios and talipes equinovarus was present. Parents refused an autopsy examination of the fetus. Postpartum period was uneventful. Broad spectrum antibiotics were given and the patient was discharged on 5th post-delivery day. 

Figure 3. Fetus showing features of Prune belly Syndrome

Discussion

Prune belly syndrome can be diagnosed antenatally by ultrasound. Ultrasonographic findings include oligohydramnios or anhydramnios, hydronephrosis, hydroureter and distended urinary bladder with a thin and attenuated abdominal wall.[5] In the new born, the prune like abdomen usually leads to the diagnosis. The prognosis of this condition is generally poor as many infants are either stillborn or die within the first few weeks of life. The routine use of ultrasound screening for fetal anomalies has resulted in more affected pregnancies being antenatally terminated.[5] If diagnosis of urinary obstruction is done antenatally, it may be possible to perform intrauterine surgery and place a vesicoamniotic shunt to prevent the development of Prune belly syndrome.[6] In developing countries the situation is different where significant number of antenatal women especially from rural areas do not get basic antenatal care. Routine ultrasonography is not done in majority of the cases and patients visit the health care facility only when active labor sets in. In such cases obstructed labor results as in our case. Proper care given antenatally would have led to early detection of the congenital anomaly on a routine ultrasound scan.

Conclusion

Our aim of presenting this case was to create an awareness regarding the need for antenatal registration, provision of basic health care facilities, and the importance of routine antenatal  ultrasound screening for detection of congenital anomalies to enable their appropriate management. In case the congenital anomaly leads to obstructed labor appropriate management such as transabdominal tapping can be offered rather than performing cesarean section. Availability of ultrasound in rural areas would prevent unnecessary operative intervention in such cases of obstructed labor thereby reducing maternal morbidity and mortality.

References

1. Nwogu-Ikojo EE, Nweze SO, Ezegwui HU. Obstructed labor in Enugu, Nigeria. J Obstet Gynaecol.2008;28(6):596–9. 
2. Ozumba BC, Uchegbu H. Incidence and management of obstructed labor in eastern Nigeria. Aust N Z J Obstet Gynaecol 1991;31(3):213–6. 
3. Routh JC, Huang L, Retik AB, Nelson CP. Contemporary epidemiology and characterization of newborn males with prune belly syndrome. Urology 2010;76(1):44–8. 
4. Nunn IN, Stephens FD. The triad syndrome: a composite anomaly of the abdominal wall, urinary system and testes. J Urol 1961;86:782–94. 
5. Ellison L, Cendron M, Ornvold K, Baker E. Early diagnosis of fetal bladder outlet obstruction. J Pediatr Surg 2000;35(3):513–4. 
6. Biard JM, Johnson MP, Carr MC, Wilson RD, Hedrick HL, Pavlock C, et al. Long-term outcomes in children treated by prenatal vesicoamniotic shunting for lower urinary tract obstruction. Obstet Gynecol 2005;106(3):503-8.

Citation

Pardeshi S, Swaminathan G, Warke HS, Mayadeo NM. Obstructed labor in a case of Prune belly Syndrome: A rare case report. JPGO 2016. Volume 3 No. 10. Available from: http://www.jpgo.org/2016/10/obstructed-labor-in-case-of-prune-belly.html