Tag Archives: Dr Baldawa

Mucocolpos in a 7 Month old newborn

22 Jul

Abstract

Imperforate hymen is an extreme manifestation of hymenal variation occurring in as less as 0.0014 to 0.1% of infants girls. Female infants with imperforate hymen rarely present with urological complications. We would like to  present  an unusual case of urinary incontinence with hydroureter and hydronephrosis in a 7 month old female child due to a large mucocolpos. This infant was successfully treated surgically.

 Case  Report

A  mother presented to the Uro-Gynaecology Outpatient department with her 7 month old daughter having complaints of urinary incontinence with dribbling of urine and mass in lower abdomen since last 15-20 days. On examination there was a large cystic mass in the hypogastrium which was extending upto the umbilicus. The lower end of masscould not be reached suggesting that it was arising from the pelvis. Ultrasonography showed a large cystic mass arising from the pelvis with a distended urinary bladder, hydroureter and hydronephrosis.(Fig 1). Differential diagnosis of bladder diverticulum, mesenteric cyst, a tuboovarian mass,or an ovarian cyst was made. Cystourethrogram was performed which showed a distended urinary bladder displaced anteriorly due to some mass behind it(Fig 2,3). Detailed gynecological examination was done under anaesthesia which revealed a bulging  IMPERFORATE  HYMEN. It was incised by a cruciate incision and ~1500 ml of straw coloured fluid was drained out.

 Discussion

Ambroise Pare first described Imperforate hymen in 1633.1  Most distal form of vaginal outflow obstruction is called “imperforate hymen”. During normal embryological development, the central portion of the hymenal membrane disappears, creating the hymenal opening at the level of the vaginal vestibule.(Fig 4). Persistance of the intact hymenal membrance results in the condition of imperforate hymen. The imperforate hymen is a solid membrane interposed between the proximal uterovaginal tract and the introitus. This vertical fusion defect from other vertical fusion defects in that it is not derived from the mullerian system. This vaginal oulet obstruction leads to entrapment of vaginal and uterine secrection above it forming a cystic collection in the pelvis. Large mucocolpos can cause urethral compression anteriorly leading to bladder outflow obstruction,urinary retention with urnary tract infection, hydroureter and hydronephrosis. Most neonates with imperforate hymen are missed at birth, delaying the diagnosis sometimes upto early adolescence when they present with hematometrocolpos. Imperforate hymen may also present with back pain ,urinary retention (37%- 60% of patients ), and constipation.2 Physical examination may reveal a lower abdominal mass on plapation, or a pelvis mass on bimanual rectal examination. The diagnosis of imperforate hymen is often established during examination when a distended bluish membrane is observed at the introitus. In the absence of this finding, only imaging study by ultrasound or MRI can establish the level of obstruction. The differential diagnoses of uterovaginal obstruction include disorders of vaginal development, such as a transverse vaginal septum or complete vaginal agenesis.which may be associated with other development anomalies(e.g, Rokitansky- Kuster-Maier-Hauersysdrome).

Prenatal diagnosis of imperforate hymen has also been reported. Fetal diagnosis has occurred as early as 25 weeks` gestation. A thin bulging memebrane separating the labia in association with a distended vagina is apparent on ultrasounography,3 these finding are usually noted during an evaluation for fetal ascites and are thought to result from distal urinary tract obstruction, however they can also be related to reflux of uterine contents through the fallopian tubes. Ascites and bladder outlet obstruction are the most common associated finding in the fetal period.4  Intestinal, cardiac and anorectal defects have NOT been reported in conjuction with imperforate hymen. Sometimes polydactyly is associated with imperforate hymen as in Mckusick-Kaufmann sysdrome.5

Careful evaluation of the perineum of the newborn is essential. Female neonate has full labia majora under the influence of maternal estrogens. Inspection of the introitus reveals that hymenal membrane is pink and slightly edematous. In the newborn with an imperforate hymen, the membrane is often bulging because of retained mucoid secretions. A vaginal cyst which fills the introitus but is attatched only to one vaginal aspect should be distinguished from imperforate hymen.

Aspirating secretions beyond the obstruction should be deferred because this procedure may result in iatrogenic pyocolpos. Instead, the diagnosis should be confirmed by performing noninvasive imaging studies (Ultrasonography, MRI) to determine the extent of vaginal outflow obstruction and to diagnose other associated anomalies (i.e imperforate hymen and a transverse vaginal septum) can occur.6

References :

  1. Wall EM, Stone B, Klein BL. Imperforate hymen: a not-so-hidden diagnosis. Am J Emerg Med. May 2003;21(3):249-50.
  2. Nazir Z, Rizvi RM, Qureshi RN, Khan ZS, Khan Z. Congenital vaginal obstructions: varied presentation and outcome. Pediatr Surg Int. Sep 2006;22(9):749-53.
  3. Winderl LM, Silverman RK. Prenatal diagnosis of congenital imperforate hymen. Obstet Gynecol. May 1995;85(5 Pt 2):857-60.
  4. Ogunyemi D. Prenatal sonographic diagnosis of bladder outlet obstruction caused by a ureterocele associated with hydrocolpos and imperforate hymen. Am J Perinatol. 2001;18(1):15-21.
  5. El-Messidi A, Fleming NA. Congenital imperforate hymen and its life-threatening consequences in the neonatal period. J Pediatr Adolesc Gynecol. Apr 2006;19(2):99-103.
  6. Ahmed S, Morris LL, Atkinson E. Distal mucocolpos and proximal hematocolpos secondary to concurrent imperforate hymen and transverse vaginal septum. J Pediatr Surg. Oct 1999;34(10):1555-6.
  7. Internet website  available on http://www.embryology.ch/anglais/ugenital/genitinterne06.html#sug

(This reference has been used in legend  4a and 4b)

 

Legends :

Fig 1 :  Ultrasonography showing Hydronephrosis and Hydroureter

Fig 2 : Cystourethrogram showing bladder compressed and displaced anteriorly  due to some mass behind .(both anteroposterior and posteroanterior view)

Fig 3: Cystourethrogram showing a distended urinary bladder extending upto the ribcage of the baby.

Fig 4a: In females, the development of the SUG (urogenital sinus) begins in the 3rd month, at the same time as the formation of the vagina.7

1=Genital tubercle   2= Vestibule  2a =SUG : phallic part  2b= SUG : lower  pelvic part of definitive  urogenital sinus   3=Vaginal plate  4=Perineum  5=Rectum  6= Utero-vaginal canal   7 = Urinary bladder  8=Urethra

Fig 4b: The pelvic part of the SUG has shrunk and will be retracted into the phallic part in order to form the definitive vaginal vestibule.7

2= vestibule  3a = uterine cavity  3b =uterine cervix  6a= vagina:lower fourth out of endoderm 6b =vagina :upper 3/4th out of mesoderm   9= hymen

 

Author:

 Dr. Baldawa Pratibha S                  

M.S(Obgyn),D.N.B, D.G.O,F.C.P.S,D.F.P,   Assistant Professor .

Correspondence address :

Baldawa Hospital, Budhwar Peth, Near Kasturba Market, Solapur – 413002,

Maharashtra,India.

Phone: (+91) 217- 2324762 (home), (+91) 9745306852 [Mobile].

Email : guptapj@yahoo.com

 

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Ultrasound features of Breus` Mole — A rarity : Dr. Baldawa Pratibha S

31 Aug


Ultrasound features of Breus` Mole — A rarity

 Abstract –

Breus` mole occurs rarely, in approximately 1:1200 placentas. The author here describes a case of 38 years old diabetic and hypertensive lady who was gravida 4, parity 2, living 2, abortion 1, and presented at 26 weeks to antenatal outpatient department. On Ultrasound she was found to have a Breus` mole with Symmetrical Intrauterine Growth Retardation. She was managed conservatively with increased fetal surveillance, regular Doppler velocimetry, and medical management for her hypertension and diabetes mellitus.Her pregnancy was terminated at 33  weeks by an emergency Cesarean section in view of regular uterine contractions and oligoamnios due to Preterm Premature Rupture Of Membranes(PPROM). The 1.38 kg baby girl was discharged in good condition after 10days of Neonatal intensive care unit care. Thus a Breus` mole can lead to Intrauterine Growth Retardation and should be looked for, especially in the presence of high risk factors like hypertension, diabetes mellitus.

Case History-

A 38-year-old lady, gravida 4, parity 2, living 2, abortion 1, presented for antenatal care at 26 weeks’ gestation. She was hypertensive and diabetic since the past 2 years. She was overweight, body mass index of 30, a nonsmoker with blood group A and Rh type positive with no atypical antibodies. She had family history of hypertension , diabetes mellitus and pre-eclampsia  in her first degree relatives. She had two full term normal vaginal deliveries 10 and 6 years ago and one medical abortion 2 years ago in view of her uncontrolled diabetes mellitus. She was on regular human insulin(rHI) since then and her blood sugar levels (BSL) were well controlled with the present insulin administration.At 26 weeks she had edema feet, blood pressure(BP) of 140/90 mm Hg and  trace urine albumin.

Ultrasound revealed symmetrically growth retarded fetus equivalent to 21 weeks at 26 weeks’ gestation with estimated fetal weight(EFW) of 402 gms + 42 gms. It also revealed the presence of hypoechoic protuberaces on fetal surface of the placenta, with no vascularity, suggestive of old subchorionic hematomas (SH).(Fig 1a,b,c,d). The placenta was thick (more than 5cm) and located posteriorly with no retroplacental hematoma or collection. These SH were spread mainly along the upper and lower fetal surface of the placenta and also reached upto umbilical cord insertion at the lower end of placenta. The fetus showed no gross congenital anomalies. At 26 weeks, on Doppler ultrasound, she had bilateral uterine artery notching, and a fortnightly ultrasound scan for fetal growth assessment was recommended. At 28 weeks, there was persistence of the notching of both uterine arteries.

She was admitted to hospital and strict BP and BSL surveillance was done. She was monitored for premonitory symptoms of eclampsia like headache, vomiting, blurring of vision and for symptoms of hyperglycemia or hypoglycaemia like sudden palpitations, excessive sweating, giddiness. Despite administration of antihypertensives like alphamethyldopa 250 mg four times daily, at 30 weeks, the diastolic BP increased to 150/100 mmHg with mild proteinuria but biochemical profile was normal.

However, her BSL was well controlled by titration with rHI. Nifedipine (5mg ) four times daily was added  to alphamethyldopa and weekly Doppler studies conducted. 2 doses of intramuscular betamethasone were administered 24 hrs apart to accelerate fetal pulmonary maturity.At 32 weeks, she had persistently raised diastolic BP of above 100 mmHg and proteinuria 2+. At 33 weeks, she developed PPROM followed by regular uterine contractions. Doppler study reported absent diastolic flow in Umbilical artery (Fig 2) and  severe oligoamnios (AFI = 4 cm ), EFW of 1400 + 317 gms. At 33 weeks, the diastolic BP remained above 110 mmHg with premonitory symptoms of vomiting and headache, increasing uric acid levels, proteinuria. In view of the fetoplacental insufficiency and severe oligoamnios, a decision of emergency caesarean section under spinal anaesthesia was taken.

rHI was withheld, senior paediatricians on call were summoned and vacancy in Neonatal Intensive Care Unit (NICU) confirmed. A live female baby was born with Apgar scores of 7 at 1 min and  9 at 5 min, weighing 1,380 g.The placenta weighed 420 gms. Macroscopically, the placenta showed scattered areas of old hematomas on the  fetal surface,some measuring up to 5 x 4 cm, and containing yellowish fluid., more towards the lower and upper end of placenta.(Fig 3). There were few areas of calcification on maternal surface of placenta. Histopathological examination of the placenta revealed extensive fibrin deposition in subchorionic space with old hemorrhage.

Thus, macroscopically and microscopically diagnosis of Breus` mole was confirmed.It had compressed the umbilical cord causing fetoplacental insufficiency. Baby was transferred to the NICU where she received expert care and showed rapid improvement.The mother was transferred to the postoperative ward and antihypertensives were continued.Her diastolic blood pressure decreased to 90 mm Hg on day 2 and then became normal  by day 5. Her BSL was checked regularly and decreasing doses of rHI were required to maintain satisfactory BSL.The postoperative period was uneventful and she was discharged on the postoperative day 10 along with her baby.

Discussion-

Since the first description of a subchorionic tuberous hematoma by Karl Breus` in 1892 in five cases of missed abortion, its etiology and pathogenesis remained a dilemma in the 18th  and early 19th century. (1,2) .The term Breus` “Mole” does not actually mean molar degeneration as seen in hydatiform moles,but is related to “mass” or coagulated blood.(1) Breus` mole is a condition in which maternal blood collects and separates the chorionic plate from the villous chorion .

(2) Breus`postulated that after embryonic death , membranous growth continues which later gets filled up with hemorrhage forming the hematoma, thus concluding fetal death to be the primary factor and hematoma development as secondary. (1,3) But in the later years, after further studies, many authors contradicted Breus` postulation, most prominent among which was a  report by Shanklin and Scott who studied ten placenta of 25 weeks and beyond and had seven liveborn infants, thus disapproving of the fact that fetal death is a primary event in formation of Breus` mole. Their incidence was 1:1200 placentas.(1,4) .

 

Despite various studies no unified etiology has emerged for development of Breus mole, however it may occur in mothers with various medical problems including disorders of circulation, complex heart disease, monosomyX (45XO -Turner syndrome),hypertension, diabetes and anticoagulation therapy, maternal thrombophilia.(1,4,5,6) Baxi and Pearlstone  suggested that presence of autoantibodies increases the tendency of platelet aggregation leading to thrombosis and/ or vasculitis causing Subchorionic thrombohematoma.(2,7).

Breus` mole has been pathologically described as cystic degeneration of subchorionic intervillous thrombi.(2) There is regular formation of laminated thrombi in small quantities in the subchorionic space causing backward flow of intervillous (maternal ) blood.This eddying of intervillous blood causes small amounts of fibrin deposition in subchorionic space which is seen as white patches or as bosellations on the fetal surface of placenta.(2,8) When such large patches of subchorionic coagulation contract, fluid may be extruded from them thus forming cysts which contain old clotted blood.(2,8)A Breus mole (coagulated  mass) may be large enough to cause protuberance from fetal surface of placenta and be detected by prenatal ultrasound.(8,9) .

Their significance depends not on their size, but on their site of appearance. For eg: if near cord insertion they may lead to cord compression and decreased fetal perfusion , fetoplacental insufficiency  as discussed in one of the cases by Kirkinen & Jouppila where hematoma dissected into base of the umbilical cord causing umbilical venous obstruction and furcate cord insertion. (8,9) Also a massive subchorionic thrombohematoma may lead to Oligohydarmnios and Intrauterine Growth retardation as described in their case report by Naoko Nishida.(2) Thus the indepth knowledge on the pathogenesis of Breus mole concluded to its histopathological fibrinoid nature and its clinical significance of probability of causing Intrauterine growth retardation and oligoamnios.

Fig 1

Subchorionic hematoma

Fig 2Absent diastolic flow in umbilical artery

 

References-

1. Benirschke K, Kaufmann P. Pathology of the human placenta.4th ed.New York:

Springer; 2000 .

2.Naoko Nishida, Shunji Suzuki,Yukie Hamamura,Kenji Higarashi ,Zuisei

Hayashi,Rintaro Sawa, Yoshio Yoneyama,Hirobumi Asakura,Ken Kawabata,Yoshi

Shima,Sumio Shin,Tsutomu Araki􀀒:Massive Subchorionic Hematoma(Breus`mole)

complicated by Intrauterine Growth Retardation. JNipponMed Sch 2001: 68.

3. James Blake Thomas:  Breus` Mole.Obstetrics & Gynecology 1964;24:794-97

4. Shanklin DR, Scott JS:Massive subchorial thrombohaematoma (Breus' mole). . Br J
    Obstet Gynaecol. 1975 Jun;82(6):476-87.

5. A.E.Madu: Breus’ mole in pregnancy.Journal of Obstetrics and Gynecology

2006;26:815-16.

6. Usta, Ihab M., Abdallah, Mazen , El-Hajj,  Nassar, Anwar H: Massive

Subchorionic Hematomas Following Thrombolytic Therapy in Pregnancy.

Obstetrics & Gynecology 2004; 5:1079-82

7. Baxi LV, Pearlstone MM:Subchorionic hematomas and the presence of

autoantibodies.Am J Obstet Gynecol 1991 ;165 :1423-24.

8. Ona Marie Faye-Petersen, Debra S. Heller,Vijay V. Joshi. Handbook Of Placental

Pathology.2nd ed.United Kingdom: Taylor and Francis Group; 2006

9. P. Jouppila, P. Kirkinen, R. Puukka : Correlation between umbilical vein blood

flow and umbilical blood viscosity in normal and complicated pregnancies.

Archives of Gynecology and Obstetrics 1986 ;237

Legends –

Fig 1a- Subchorionic hematoma at the upper end of placenta appearing as

protuberances on fetal surface of placenta.

Fig 1b- Large Subchorionic hematoma (4.7 x 3.1 cm) at the lower end of placenta .

Fig 1c – Colour doppler showing absent vascularity in the hypoechoic collections

on fetal surface of placenta thus excluding placental lakes .

Fig 1d- Thick posteriorly located placenta.

Fig 2 – Absent diastolic flow in umbilical artery seen on Doppler ultrasound at 33

weeks.

Fig 3- Gross appearance of placenta showing  areas of old hematomas on the  fetal

surface,some measuring up to 5 x 4 cm, and containing yellowish fluid., more

towards the lower and upper end of placenta

Author:

 Dr. Baldawa Pratibha S                  

M.S(Obgyn),D.N.B, D.G.O,F.C.P.S,D.F.P,   Assistant Professor .

Correspondence address :

Baldawa Hospital, Budhwar Peth, Near Kasturba Market, Solapur – 413002,

Maharashtra,India.

Phone: (+91) 217- 2324762 (home), (+91) 9745306852 [Mobile].

Email : guptapj@yahoo.com

 

Department(s) and institution(s) –

Department of Obstetrics & Gynecology.

ShreeUthrodhamThirunalMedicalCollege,

Vattapara,Trivandrum,Kerala,INDIA.

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