a) A RARE CASE OF WAARDENBURG SYNDROME
A case of Waardenburg syndrome with poliosis, dystopia
Canthorum, vitiligo and heterochromium iridium.
AN INTERESTING CASE OF CHOROIDAL MELANOMA
45 years old lady came with complains of decreased vision in
Right Eye for the past 6months, no history of pain. On examination vision Right
Eye-NO Perception of light, Left Eye- 6/6. Pupil – relative afferent pupillary
defect in right eye, on fundus examination showed elevated mass lesion in the
choroid with exudative retinal detachment in right eye.
Figure 1 patient profile
BScan showed moderate echoic mass lesion seen in choroid close to optic nerve head projecting into vitreous with exudative retinal detachment.
Figure 3 MRIORBIT- PLAIN AND CONTRAST
MRI Orbit –plain and contrast showed hypointense mass lesion in right choroid
close to optic nerve head and extended in to vitreous cavity.
Enucleation with orbital implant.
Department of Anesthesiology, GMKMCH
Maternal cardiac arrest: Mother and baby survives following perimortem Cesarean delivery
Dr.G.Sivakumara, Dr.K.Murugesanb, Dr.A.K.Prasathc, Dr.S.Rameshd
Resuscitation of a pregnant woman in cardiac arrest (CA) is unique and has certain special recommendations in addition to the standard adult CPR.1Performing perimortem cesarean delivery is one such special recommendation. When performed within 5 minutes of resuscitative efforts, chances of successful resuscitation of the mother and potential fetal salvage are possible.
Herein, the authors report a case of a pregnant patient who presented with acute pulmonary edema and got stabilized in ICU. She had sudden cardiac arrest while being shifted onto operating table for emergency cesarean section. Upon being recognized immediately, rapid resuscitative efforts were made as per standard protocol. Perimortem cesarean delivery was performed after one minute of starting CPR and was marked by immediate return of spontaneous circulation (ROSC).
The major causes of cardiac arrest in pregnancy, American Heart Association recommendations for resuscitation of cardiac arrest in pregnancy and the scientific basis of perimortem cesarean delivery are discussed in this case report.
A 22 years old pregnant female with 34 weeks of gestation was referred from a private nursing home with breathlessness for past 3 hours. She did not have significant past medical illness. Physical examination revealed a conscious, restless, dysponeic patient with pedal edema and bilateral lung crepitations. Her vital signs were heart rate of 145/minute, blood pressure of 170/130mmHg and oxygen saturation of 70%. Clinical diagnosis of pregnancy induced hypertension (PIH) with acute pulmonary edema was made.
In the emergency department, patient was intubated and ventilated with 100% oxygen. Inj.Frusemide 100mg IV, Inj.Labetalol 20mg IV, Inj.Magnesium sulphate 4gms IV, and 4gms IM were administered. Patient got
stabilized with a heart rate of 122/minute, blood pressure of 130/90mmhg, and oxygen saturation of 100%. Obstetric surgeons decided to terminate pregnancy by emergency cesarean section since the patient had severe PIH with pulmonary edema. On getting informed consent, the patient was shifted to operating room with monitoring of heart rate, ECG, SpO2, and NIBP during transport and was ventilated through ET tube using Bain’s circuit with 100% oxygen.
Before shifting onto the operating table patient became unresponsive and a flat line tracing was noted on the monitor. The carotid artery pulsation was absent and heart sounds were not heard. On immediate recognition that the patient had sudden cardiac arrest, cardiopulmonary resuscitation was initiated with the patient on operating table which was tilted to left and the time of witnessed cardiac arrest was noted. External chest compressions, ventilation with 100% oxygen, and left uterine displacement were performed. Inj.Adrenaline 1mg was given intravenously.
Obstetric team and pediatrician were informed about the sudden cardiac arrest and were well prepared for immediate cesarean delivery. With the ongoing CPCR and after one minute of starting the resuscitative efforts, perimortem cesarean section was performed delivering a live born baby. The cardiopulmonary resuscitation was continued without any interruption. Once the uterus was emptied by perimortem cesarean delivery, the patient showed signs of revival from cardiac arrest. The successful return of spontaneous circulation (ROSC) was evidenced by sinus rhythm on monitor with heart rate of 156/minute, end tidal CO2 concentration of 18mmHg, blood pressure of 90/40mmHg, and oxygen saturation of 100%. Dopamine was infused at a rate of 5mcg/kg/minute. Anesthesia was maintained with 50% nitrous oxide in oxygen, Inj.Fentanyl 150mcg IV, Inj.Vecuronium 4mg IV, and ventilation was controlled with circle system. Patient was shifted to ICU for postoperative mechanical ventilation. Analgesia was provided with Inj.Morphine 10mg IV, Inj.Paracetamol 1gm IV 8th hourly, and sedated with Inj.Midazolam 2mg/hour infusion. Patient regained wakefulness in 2 hours. Dopamine requirement went down over 4 hours and was stopped. Patient’s vital parameters were maintained within normal limits.
Postoperative renal function tests, electrolytes, hemoglobin, and coagulation profiles were unremarkable. ECG showed sinus rhythm with heart rate 80/minute. Chest X-ray showed features of pulmonary edema, normal cardiac shadow and correctly placed right subclavian venous catheter. Weaning off mechanical ventilatory support was possible after 12 hours and subsequently patient could be extubated. Mother got discharged from ICU to ward on sixth postoperative day and the baby got discharged from NICU on eighth day with intact neurological functions.
Cardiac arrest during late pregnancy occurs in approximately 1:30,000 pregnancies.2The major causes are hemorrhagic shock, eclampsia, amniotic fluid embolism, sepsis, and thromboembolic events. The minor causes are acquired or congenital heart diseases, anaphylaxis, and trauma.3
Cardiovascular and pulmonary reserves are poor during pregnancy due to the anatomic and physiologic changes. This complicates the resuscitation of a pregnant woman. Apart from the standard adult resuscitation guidelines, attention to certain pregnancy-specific interventions is crucial to the outcome. Some of these pregnancy-specific concerns are,
1. Potentially difficult airway
2. Propensity for rapid hypoxemia
3. Risk of pulmonary aspiration
4. Aorta-caval compression
5. Treatment of reversible causes, and
6. Timing perimortem cesarean section
An obviously gravid uterus is one that is sufficiently large enough to cause aortacaval compression. Obstruction of venous return by gravid uterus can produce hypotension and may precipitate arrest in the critically ill pregnant patients.1 Aortacaval compression can occur for singleton pregnancy by ≥20 weeks of gestation that corresponds to fundal height up to umbilicus level. Left-lateral tilt results in improved maternal hemodynamics of blood pressure, cardiac output, stroke volume and improved fetal parameters of oxygenation.
Perimortem cesarean section:
Perimortem caesarean delivery was first recommended by Katz and colleagues in 1986. He described ‘4 minute rule’ from the time of arrest with baby delivered within 5 minutes. The 5 minute time limit was based on theoretical considerations such as oxygen consumption, neurological injury and a single case report.4The benefits of perimortem caesarean delivery are, improvement in venous return, effective chest compressions, reduced metabolic demand by fetus, and improved respiratory mechanics.
The timing of restoration of adequate cardiac output is critical for both the mother and the baby. The mother is likely to experience hypoxia earlier in the course of an arrest due to the increased oxygen demands of pregnancy and decreased oxygen storage, while the fetus relies on the maternal circulation for oxygen supply.
The neonatal outcomes are better when the gestational age is advanced and the cardiac arrest occurs in delivery room or in the operating room.5 Ultrasound examination helps in determining the gestational age. If the gestational age is more than 24weeks, delivery of the fetus either alive or dead will help in restoring the venous return and improves the chance of successful maternal resuscitation.
In this case report, timely recognition, standard resuscitation efforts and perimortem caesarean delivery within the time limit were the reasons for successful outcome of mother and baby with intact neurological functions. It is important to have maternal cardiac arrest team which comprises of anesthesiologist, obstetrician, neonatologist, and cardiologist. All the essential equipments should be kept ready in the delivery suite.
1. Part 12: Cardiac Arrest in Special situations. 2010 American Heart Association Guidelines for Cardiopulmonary. Resuscitation and Emergency Cardiovascular Care.
2. Obstetric Anesthesia, principles and practice, David H.Chestnut, 3rd edition, Elsevier Mosby publication.
3. Successful Cardiopulmonary Resuscitation in Pregnancy: A Case Report. Ozgur Soguta e, Atilla Kamazb, Mehmet Ozgur Erdoganc, Yusuf Sezend. J Clin Med Res 2010;2(1):50-52
4. Cardiopulmonary arrest in pregnancy: two case reports of successful outcomes in association with perimortem Caesarean delivery, N. J. McDonnell, British Journal of Anaesthesia 103 (3): 406–9 (2009)
5. Maternal deaths from anaesthesia: An extract from Why Mothers Die 2000–2002, the Confidential Enquiries into Maternal Deaths in the United Kingdom, Chapter 9: Anaesthesia, British Journal of Anaesthesia 94 (4): 417–23 (2005)
Interesting Case from Department of Surgical Gastroenterology
Right hepatectomy done for a case of HCC
Tumour in Right Lobe
Parenchymal transection done using Harmonic
Interesting Case By Department of Anesthesia on 24.4.15
FOREIGN BODY IN THE UPPER OESOPHAGUS – MULTIDISCIPLINARY APPROACH
Foreign bodies in the esophagus are considered to be a serious clinical condition, both in
adults & children due to possible complications (esophageal perforation, mediastenitis, fistulization,
airway obstruction) with a high mortality & morbidity. Therefore rapid & accurate diagnosis together
with subsequent treatment is necessary and in 1% of cases, surgical intervention is required.
A 67 years old female patient referred to our emergency department in April 2015,
complaining of dysphagia, odynophagia, diffuse chest pain for 4 hours with alleged history of
accidental ingestion of artificial dentures at around 1pm in her house.
Antero-posterior & lateral chest X-Rays revealed radio opaque ingested foreign body of size
5cm* 3cm located in the upper cervical esophagus. An immediate gastroenterological evaluation,
performed with flexible endoscopy under sedation, confirmed an artificial removable partial denture
with wire in upper cervical esophagus. Unfortunately due to shape & dimensions of this particular
foreign body endoscopic removal failed. So it was planned to remove by surgical intervention. So
patient was taken up for surgery with risk factors of old age, full stomach, emergency surgery & site
of surgery. With all emergency drugs & difficult airway cart ready, patient was premedicated with H2
Blocker, prokinetics, Inj. Midazolam 2mg IV & Inj. Fentanyl 80 mics. Then after preoxygenated with
100% Oxygen, patient was induced with Inj. Propofol 80mg IV & Inj. Succinylcholine 50mg IV. Under
Modified RSI, patient was intubated with 7.5mm ETT & ventilation controlled with closed circuit.
Maintained with halothane 0.5%, O2:N2O 1:2, Inj. Vecuronium 4mg IV. Open cervical
oesophagostomy was done. Foreign body was removed in toto without any complications & primary
closure was done. At the end of the procedure, residual neuromuscular blockade was reversed &
extubated. Patient was shifted to PACU for observation. In PACU, patient remained
hemodynamically stable with adequate analgesia & IV antibiotics & adequate hydration. Then
patient was discharged home after 7 days of observation with suture removal.
A Multidisciplinary approach with radiologist, gastroenterologists, ENT surgeon &
Anaesthetist helped in accurate diagnosis & management.
2) RTA WITH HEAD INJURY WITH LEFT PNEUMOTHORAX: A CASE REPORT
Pulmonary complications are prevalent in the critically ill neurological population. Direct
brain injury, depressed level of consciousness, inability to protect airway, disruption of natural
defense barriers, decreased mobility & secondary neurological insults inherent to severe brain injury
are the main of pulmonary complications in critically ill neurological patients. Along with brain injury,
our patient had direct chest trauma. So earlier identification, prevention & management of
pulmonary complications are the cornerstone of earlier & complete recovery.
A 4 year old male child was admitted with alleged history of hit by a two wheeler while playing
near home. Patient was initially crying, taken to a nearby hospital. CT scan brain showed minimal
interhemispherical bleed & right femur fracture in X-RAY. There was a drop in GCS. Hence patient
was shifted to a tertiary care hospital for further management. His GCS was found to be 6/15, thus
intubated & ventilated. Chest X-RAY showed left pneumothorax & an ICD was placed. Patient
underwent closed reduction for left femur fracture & TENS nailing. For right mandible fracture ORIF
& IMF wiring was done. Surgical tracheostomy was also done. Due to cost constraints patient
attenders decided to shift the patient to our hospital for further management. At the time of
admission, patient GCS was E2 VT M3, unconscious with tracheostomy tube insitu connected to
pressure control mode ventilation, febrile, CVS S1S2, RS B/L Extensive crepitations with ICD tube in
situ, HR 125/min, SPO2 96%, temperature 39deg Celsius. CT chest showed hemorrhage / contusion
in anterior segment of right upper lobe & in apicoposterior segment of left upper lobe & linear
minimally displaced fracture of left first rib. USG abdomen & pelvis showed mild left hemothorax
with no hemoperitoneum / obvious solid organ injury. Repeat CT Brain showed resolving
interhemispherical bleed, no mass effect. Patient was treated with IV Antibiotics, IVF, pressure
control mode of ventilation, RT feeds. ICD Tube was removed after 4 days of admission. Then patient
slowly weaned from ventilator support & exposed to room air for 5days. Then after IMF Wiring
removal, tracheostomy tube was removed & stoma closed. Femur fracture stabilized well. 0n 24th
day, patient was discharged home with no neurological deficit / sequelae. Advised to review after
Respiratory failure, pneumonia, acute lung injury, ARDS, pulmonary edema, pulmonary
contusions, hemo/pneumothorax, pulmonary embolism are frequently encountered in setting of
direct chest trauma. In the absence of feasible pharmcological agents to target these goals, bed side
techniques such as thoracocentesis, closed thoracostomies, lung protective ventilator strategies,
bundles for prevention of VAP, DVT prophylaxis are the cornerstone in the prevention &
management of pulmonary complications in severe brain injured patients & in direct chest trauma
Department of Anesthesia
Newer horizon in Airway management for thyroid surgeries:
A case report
Dr.Nagarajan MD(a), Dr.MohanHariraj MD(b) ,Dr.Vijayakumar(c)
a- Professor, b-Assistant professor, c-Junior Resident
Airway management is a fundamental goal of anesthesiologists. Failed intubation is associated with serious complications. Thyroid surgery is usually considered a risk factor for difficult intubation, but this has not been widely studied(1).When thyroid enlargement is associated with deformed airway there is difficulty in airway management.(2)
Video laryngoscopes are new intubation devices which contain miniature video camera, enabling the operator to visualize the glottis indirectly, which are so designed enabling the clinician to use it successfully without special training.(3)In this report we bring forth the use of KING vision video laryngoscope in securing the airway for a huge multi nodular goiter coming for de bulking surgery.
75 yr old female, a known bronchial asthmatic with history of thyroid swelling for the past 5 yrs with obstructive symptoms came for de bulking surgery. On examination she was conscious and oriented, moderately built and nourished, with huge thyroid swelling of size 10 x 12 cm with retro sternal extension. Cardiovascular examination was normal. Respiratory system revealed bilateral wheeze. Her vitals were-PR- 142/min, BP-152/100mm Hg, Spo2-93% in room air. Airway examination revealed mouth opening < 3cm, MPC- class III, neck movements< 80 degree and thyromental distance <6 cm. Spine was normal. All her blood investigations were within normal limits except for mild increase in T4.Echo revealed mild MR and AR with normal LV function. Mobility of vocal cords was confirmed by Ent surgeon. CT chest and PFT could not be taken due to poor patient compliance. She was assessed under ASA IV E for Near total Thyroidectomy.
The plan for surgery was endotracheal intubation general anaethesia with controlled ventilation. Informed risk consent was obtained. Anaesthesia machine was checked and all emergency gadgets were kept ready. Preoperative monitors included HR,Spo2,ECG,IBP and ETCO2.She was given Salbutamol nebulisation, Premedicated with iv glycopyrollate 0.4 mg, im morphine 10 mg, iv midazolam 1 mg. Before starting the case awake laryngoscopy using King vision video laryngoscope was attempted to view the glottis after locally anaesthetising the airway with 10 % lignocaine spray.The Cormack lehene was grade I..She was Preoxygenated with 100 % oxygen for 5 min, induced with Iv propofol 50 mg and Iv Sch 50 mg, intubated with 7.5 mm cuffed flexometallic ETT using KING vision video laryngoscope . Anaesthesia was maintained with 02 and N20 66%:33% , FGF 5- 6 lts/min +halothane 0-1% + Vecuronium 4 mg LD and titrated doses+ IPPV. Duration of surgery – 7 hrs.3000 ml crystalloids, 3 whole blood, 3 FFP given. Estimated blood loss – 2-2.5 lts .Total urine output- 300 ml. Dopamine infusion started towards the end of surgery with adrenaline bolus in view of unforeseen hypotension. Post procedure Tracheostomy was done and FM tube was removed. Pt was awake, obeying commands. In view of hemodynamic instability, prolonged surgery, neck edema patient shifted to PACU for Elective post operative ventilation .Shifting vitals- PR-126/min,BP-90/60 mm Hg with dopamine support, Spo2-93 % .she was weaned from ventilation and inotropes and was discharged from PACU on 10 th POD.She was discharged from hospital 10 days later to Oncology dept of MMC for further management.
Video laryngoscopes form the new era of laryngoscopy and tracheal intubation. They offer superior visualization of the glottic structure. They not only aid in tracheal intubation in patients with difficult airway but also enhance teaching to beginners(5) .The King Vision Video laryngoscope is an affordable, durable and portable video laryngoscope. It is designed for indirect laryngoscopy, difficult endotracheal intubations as well as routine intubations.
The mechanisms of airway obstruction in thyroid swelling include extrinsic tracheal compression ,tracheal invasion , tracheomalacia , vocal cord paralysis or a combination of the above. The most frequent cause of thyroid-induced airway obstruction is the presence of a substernal goiter compressing the trachea, with or without associated tracheomalacia. Huge goitres with decrease neck mobility and malignant infiltration of airway may cause anatomical derangement on laryngeal inlet, which may make difficult visualisation of laryngeal opening during laryngoscopy in sniffing position. The presence of a cancerous goitre is a major factor for predicting difficult endotracheal intubation(4)
Our case presented with all the classical predictive criteria for difficult intubation. The process of intubation in such cases involves three steps namely visualization of glottis, entry of endotracheal tube into glottis and passage
Of tube into larynx and trachea. In most cases with huge goiter visualization of glottis with conventional laryngoscope is not going to be difficult. In this case there was inspiratory stridor hinting at extrathoraxic obstruction. So conventional laryngoscopy is going to be difficult. Hence we used King vision laryngoscope for intubation.
Airtraq and king vision laryngoscope has curvature of 90 degree, identical with normal anatomical oropharyngeal curvature. Line of visualisation and insertion of endotracheal tube is same in Airtraq and King Vision laryngoscope. So, it is advisable to use newer scopes for intubating a patient with huge goitre causing restriction on neck mobility. Even with altered neck movement and position, view of glottis inlet is satisfactory by using Airtraq and king vision laryngoscope.(4)
3.Video laryngoscopes in adult airway management
4.Airway management in patients with thyroid swelling, Apeksh Patwa1*, Apeksha Patwa2, Amit Shah3
5.Airway management by Rashid Khan
ANAESTHETIC MANAGEMENT OF A PAEDIATRIC CASE OF PYOPERICARDIUM FOR EMERGENCY
Paediatric cardiac emergencies are always
associated with major degrees of hemodynamic instability and cardiovascular collapse.Immeadiate surgical intervention needed especially in paediatric patients presents with cardiac tamponade.Anaesthetic management is a major challenge in these patients,with induction of anaesthesia being the most important stage as sudden cardiovascular collapse can occur immeadiately
after induction of anaesthesia.
A 4 year male child ,weighing 16 kg presented with history of fever and cough 4 days duration,and breathlessness -3 days duration.
Prior to that he had joint pain and inability to move left lower limb diagnosed to have septic
Arthritis and arthrotomy done outside hospital 15 days back.o/e the child was irritable with fever .Tachypnoeic and tachycardia(+).Auscultation revealed normal
Respiratory sounds and heart sounds were barely audible.peripheral pulses were feeble.Investigations showed raised leukocyte
Counts,with normal Hb and cardiomegaly on
CXR.ECG showed low voltage complexes and ECHO showed large pericardial effusion.A decision of emergency pericardiectomy was taken.Drugs for induction of anesthesia and emergency drugs kept ready inview of unexpected complication.
After obtainining high risk consent,child shifted to ot and monitors connected.Premedicated with inj midazolam and fentanyl in titrated doses.Induced with Inj ketamine and succinylcholine,the child was intubated with 5 mm ETT,and bilateral air entry confirmed and tube secured.Surgery was proceeded,incision over the 5 th intercostal space.Ribs retracted aside pericardium visualized.after careful
Dissection of pericardium found to have pus
Inside pericardial sac.Pus drained out and send
For culture and sensitivity.Surgery was uneventful child extubated on table after adequate recovery.shifted to ICU for observation.
Pericardial tamponade can be associated with
Significant hemodynamic instability especially in paediatric patients.such patients need immeadiate intervention either in form of pericardiocenthesis or surgical decompression.Goal of anaesthetic management is to maintain adequate cardiac output.Prompt recognition and initiation of
Appropriate therapy in paediatric cardiac emergencies are essential for favouable outcome.The time between induction of anesthesia and surgical pericardiectomy is
Important.Ketamine is the induction agent of
Choice because of ability to increase systemic vascular resistance,and less fall in blood pressure as compared to sedatives .Hence ketamine induction is safe compared to other modes of induction in a hemodynamically compromised paediatric cardiac patient.
1) Case By OBG dept
PUBIC DIASTASIS presenting as traumatic PPH –a rare case presentation
Parturition-induced pelvic instability is rare. Incidence rates of symphysial rupture after vaginal delivery ranging from one in 600 to one in 30,000 deliveries. Peripartum ligamentous relaxation with moderate widening of symphysis pubis and sacroiliac joints is physiologic and occurs regularly resulting in widening of the birth canal facilitating delivery. This occurs secondary to increased elasticity of the pelvic joints induced by an elevation in circulating progesterone and relaxin.
Mrs.kavitha, 32yrs, para3, live3, abortion3 has been referred from jedarpalayam phc, as a case of traumatic PPH .She was booked and immunized at jedarpalayam phc. She got admitted in phc with labour pain on 20-5-15 ,5am delivered an alive boy wt2.75kg by LN on 20-5-15 at 9.35am,excessive bleeding after delivery managed with iv fluid and uterotonics and referred to GMKMCH .
O/E patient pallor++, afebrile, peripheries cold & clammy, no pedal edema, dyspnoeic, tachypnoeic, hypotension ,spo2 95%, clotting time 8 minutes. P/A uterus well contracted, shifted to left. P/V excessive bleeding ++. Under GA VAGINAL EXPOLARATION done, external urethral meatus identified with difficulty ,urethra found to be avulsed from its attachment, exposing the suburethral tissue and part of bladder ,torrential bleeding from torn vessels present.Pubic diastasis measuring 8cm found managed by pelvic external fixation. Laparotomy done to R/O uterine rupture ,uterus,adnexa found to be normal, suprapubic catheterization done, multiple vaginal lacerations- sutured. Blood and blood products given. Post op period uneventful .
Incidence of causes of PPH, atonic uterus 80%, trauma to the genital tract - lacerations, haematomas, inversion, rupture uterus -10-15%,retained tissue -3-5% ,coagulation 1-2%.Diagnosis of etiology of PPH - P/A uterus firm and contracted-atonicity ruled out ,bedside test- DIC ruled out , retained tissue ruled out by USG ,suspecting for traumatic PPH detailed speculum examination under good light is needed.
Peripartum ligamentous relaxation with moderate widening of symphysis pubis and sacroiliac joint is physiological and occur regularly resulting in widening of the birth canal & facilitating delivery.Pubic diastasis may cause anterior widening and loss of stiffness within the pubic symphysis causing instability in the pubic joint .Treatment of postpartum pubic diastasis bed rest ,analgesics and application of a pelvic binder when diastasis <3cm ,surgical intervention -external &internal fixation. Complications are soft tissue infection or osteomyelitis.
PREVENTION OF PELVIC FLOOR TRAUMA
Identifying women at risk and avoidance of obstetric interventions
Selective episiotomy on case-to-case basis
Warm compressions to the perineum
No touch technique until crowning of the head
Avoidance of Valsalva pushing
Maintaining the women in left lateral position till active pushing
Controlled head delivery .
Traumatic PPH is prevented by proper assessment of CPD &conduct of labour.
2) OBG dept rare case
A VERY RARE CASE OF HYPERREACTIOLUTEINALIS ASSOCIATED WITH HYPOTHYROIDISM IN NORMAL PREGNANCY -AN OFTEN MISTAKEN DIAGNOSIS.
Hyperreactioluteinalis refers to moderate to cystic enlargement of ovaries due to multiple benign theca lutein cysts due to abnormal response of ovaries to Beta HCG. It is most commonly associated with vesicular mole and Choriocarcinoma. It is even more uncommonly reported in pregnancy unassociated with trophoblastic disease .Only 51 cases of Hyperreactioluteinalis unassociated with trophoblastic disease have been reported in English literature. 11 cases were fetal hydrops (8 immunologic and 3 non-immunologic), 8 cases of multiple pregnancy and 30 cases of normal pregnancy. Presenting here is a case of HL associated with Hypothyroidism in a spontaneously conceived singleton pregnancy managed non-surgically resulting in successful vaginal delivery.
25yr old G2P1L1 with 5 months amennorhoea was referred as a case of bilateral ovarian cyst complicating pregnancy discovered on a routine antenatal ultrasound.The patient had no comorbidity and this was a spontaneous conception. Patient was asymptomatic; abdomen was distended and was not tense or tender. Pelvic examination-firm and closed cervix and uterus was of 20 weeks gestation. Laboratory value of Hb-11.2g/dl, white cells, platelets, urea and creatinine was normal, Beta HCG-24,244mIU/ml, CA 125-10.8 IU/ml, FSH, LH and Testosterone were within normal range. BP was stable throughout the admission days ranging from 100/70-120/80mmHg.Ultrasound showed a Single gestation of 16-17 weeks, placenta was normal, Right ovarian cyst of 20*10cm and left ovarian cyst of 11*4.5cm with thin septations,No solid components,no ascites,liver,Gall bladder ,spleen,pancreas and kidneys were normal and there was no peritoneal or omental deposits or any other evidence of metastasis.Vascularity in both adnexae was preserved.MRI showed T1 Hypointense and T2 Hyperintense well defined multiloculated cyst with thin septations <3mm ,with no obvious soft tissue component, with no diffusion restriction.After the history ,examination and investigation report pt was clinically diagnosed as a case of Benign ovarian cyst complicating Pregnancy .But surprisingly TSH was >100mIU/ml .As the pt had elevated TSH which would have sensitized the ovarian follicle to the potentiated follicle stimulating hormone response leading to bilateral large ovarian cysts,pt was provisionally diagnosed as a case of Hyperreactioleuteinalis with 20weeks of gestation.Pt was fully evaluated and history reviewed. Case reports of Hyperreactioleutalis were the background of the diagnosis and decision not to end up with unnecessary laparotomy for these large ovarian cysts as this is an often mistaken diagnosis resulting in loss of ovaries. Hence pt was under observation with careful vitals monitoring to rule out torsion or rupture, and pt had no complaint of abdominal pain or vomiting . Patient was started on T. Eltroxin 100ug OD for Hypothyroidism. . This patient had not received exogenous ovarian stimulation and endogenous gonadotropin levels were within normal limits, except that patient had profound hypothyroidism..Pt was discharged after 2 weeks of admission and evaluation as the pt was asymptomatic and was advised to report immediately if any abdominal pain or vomiting. The complications expected during the expectant management of such a large cyst were Torsion, Rupture, Entrapment of enlarged ovaries. At 28 weeks of gestation, pt came to antenatal OP, pt had no complaints, clinically there was no evidence of torsion or rupture and repeat USG done showed right ovarian cyst of 13.3*8cm multiloculated cyst, TSH was -28.25 mIU/ml and hence T. Eltroxin dose was increased to 125ug OD.As the patient was asymptomatic and there was marked decrease in the size of the ovarian cyst which was well in correspondence with the decrease in TSH value,confirmed the diagnosis of Hyperreactioluteinalis and lead to the decision that the pt can be followed up and managed conservatively. At 31 weeks of gestation Right ovarian cyst was 7.1*5.1cm, TSH-5.3mIU/ml. At 38 weeks pt was electively admitted for safe confinement.TSH was normal and Ultrasound repeated showed right ovarian cyst of 6*4cm with single live gestation of 38 weeks in Breech presentation. Pt spontaneously went in for labour,pt was carefully monitored for vitals and progress during labour and delivered an alive girl baby of 2.5kg by Assisted Breech delivery. Placenta was normal. Intrapartum and postpartum period was uneventful. Ultrasound done on 2nd postnatal day showed Rt ovarian cyst of 6*4cm. Pt did not have any complaints and was clinically stable, hence discharged on 4th postnatal day with advice to continue T.Eltroxin and to have follow up .Pt came after 6 weeks for follow up, USG done showed- Right ovarian cyst was 4.2*3.4 cm. A waiting time of 6 weeks was given for all the pregnancy related hormones like oestrogen, progesterone, Beta HCG to come to normalcy and then to accurately assess the size of the ovarian cyst in relation to the TSH value. Ovaries were of normal size 24 weeks postpartum.TSH value was within normal limit in the postpartum period.
Through out the follow up period pt did not have complaint of abdominal pain ,vomitting ,tachycardia,abdominal tenderness on examination suggestive of torsion ,or free fluid abdomen and hypotension suggestive of rupture ovarian cyst. Pt was asymptomatic through out. The ovarian cyst was multiloculated with thin septations and the cyst wall was neither thickened nor enhanced,without solid components and no areas of calcification, suggesitive only of a benign ovarian cyst. Ultrasound showed no ascites,no metastasis in liver or peritoneal or omental deposits.MRI done showed features of benign ovarian cyst and CA-125 was normal with which diagnosis of benign ovarian cyst was confirmed. During follow up-pt clinical condition was stable with no clinical or radiological evidence of torsion or rupture.Most important observation was the marked decrease in size of the ovarian cyst with decrease in TSH value. The reduction in ovarian volume with decrease in TSH along with asymptomatic presentation, Ultrasound and MRI not suggestive of malignancy ,normal values of CA 125 ,Beta HCG,FSH,LH and Testosterone, uneventful antenatal ,intrapartum and postpartum period which strongly contributed to the decision of close observational and conservative management of this pt even after visualizing such a large ovarian cyst on ultrasound.Pt did not have any abdominal emergency situation that required emergency laparotomy and unnecessary oophorectomy.
1. Ovarian neoplasms: Differentiated by using MRI and CA-125 values.
Malignant ovarian neoplasm was not a differential diagnosis as CA-125 was within normal limits and ultrasound and MRI did not show any solid component in the ovarian cyst or ascites or evidence of metastasis.Pt was of an younger age with low risk for malignancy.
2. Ovarian Hyperstimulation syndrome(OHSS)-Pathophysiology of cysts is similar to OHSS. It rarely occurs in spontaneous ovulatory cycle. Typically iatrogenic OHSS is diagnosed immediately following conception. OHSS is associated with ovarian enlargement of >10cm,ascites,pleural effusion which helps in differentiation.our pt was a case of spontaneous conception and there was no ascites or pleural effusion which rules out OHSS.
Recognition of Hyperreactioluteinalis is important because misinterpretation at laparotomy or erroneous histologic diagnosis have resulted in unnecessary surgery resulting in sterilisation. Its Natural course is postpartum regression in course ,most often does not require any intervention or medication..This condition being self limiting Burger reported the first case that was not associated with trophoblastic disease in 1938.There have been fewer case reports in last 10 years due to new types of presentations different from those previously reported or cases that lead to a better understanding of aetiology or treatment. Still only sporadic cases of Hyperreactioluteinalis, especially managed conservatively have been reported.
This condition is also seen in patients with PCOD due to an increased sensitivity of ovarian stroma to Beta HCG. It also has been seen in association with diabetes mellitus complicating pregnancy, ovulation induction and Clomiphene intake.
This case is rare in the aspect of the fact that it is one of few reports of Hyperreactioluteinalis occurring in a spontaneously conceived pregnancy associated with Hypothyroidism and more over was managed expectantly throughout followed by successful vaginal delivery. Hyperreactioluteinalis is mostly bilateral and found incidentally at the time of LSCS or on routine ultrasound. Hyperreactioluteinalis may present in any trimester as an abnormal mass or acute abdomen. Majority of Hyperreactioluteinalis -70% of cases occur in third trimester or immediate postpartum. Usually there are no symptoms .Depending on the size of masses either patients are asymptomatic or they present with pain from haemorrhage, intra-abdominal pressure, torsion ,rarely ascites, virilization in 15% of mothers due to hyperandrogenism, hyperemesis gravidarum or hyperthyroidism have been reported and are not related to Hyperreactioluteinalis but are proved by the underlying problem that is causing high Beta HCG level. One study suggested that FSH may play a role in aetiology of HL.Main aetiologic factor in the development of HL is some intrinsic sensitivity to gonadotrophins causing marked hypertrophy followed by luteinization of theca interna layer. Conservative approach is indicated with wedge biopsy and frozen section for diagnosis.Oophorectomy is necessary only to remove infracted tissue or to control haemorrhage. Prognosis is good and the Cysts typically involutes within 6 months .It only rarely recurs.With their large number of locules ,hyperreactioluteinalis can even mimic a malignancy in particular mucinous borderline tumour of intestinal type however they are differentiated by the fact that they have smaller thin walled cysts and not as much solid component as seen in HL.They can be differentiated based on Beta HCG levels ,ovarian tumour markers and imaging modality correlation like USG and MRI. On ultrasound examination Hyperreactiluteinalis is characterized by large adnexal masses that consists of many thin walled small theca lutein cysts giving it appearance of a ‘spoke Wheel’ . Microscopically they are follicular cysts with luteinization of theca interna or granulosa cells, edema of theca layer and stroma. The causal association between the hypothyroidism and development of hyperreactioluteinalis is supported by data from animal research. Scommegna et al reported that the development of ovarian follicular cysts in rats can be stimulated by drug induced hypothyroidism. It has been demonstrated in multiple species that perinatal exposure to thyroid stimulating hormone in vivo and in vitro causes a hormonal imprinting effect with a durable amplification of the response to subsequent follicle stimulating hormone receptor binding. However, these findings in human are speculative and remains to be demonstrated but it is conceivable that an unrecognized profound hypothyroidism in our patient sensitized the ovarian follicle to potentiated follicle stimulating hormone response. An increased capillary permeability to protein in patients with hypothyroidism has been demonstrated which may account for high concentration of proteins in effusion and could have contributed to an increased fluid accumulation in the ovarian follicles.
The importance of knowing this entity is that Hyperreailuteinalis is benign and usually innocuous and does not need any specific treatment except in cases of surgical emergencies like torsion.However many times it is mistaken for ovarian malignancy and inadverdently operated upon.The self limiting course of this condition advocates a conservative management and necessitates differentiation from other malignant mimics.
29 years patient reported with proptosis left eye for 15 days referred from Dharmapuri govt. hospital. Known
Diabetic on Inj.insulin. On examination vision RE-6/6, LE- no PL, pupil- dilated, not reacting to light, exposure
Keratitis, with external ophthalmoplegia. MRA showed cavernous sinus thrombosis. It’s an interesting case of
cavernous sinus thrombosis.
4) INTERSTING CASE REPORT FOR THE MONTH OF FEBRUARY, 2016
35 Years old male came to our department with complains of defective vision in right eye since ten days. On examination patient visual acuity in right eye was 6/36, left eye was 6/6 and with defective field of vision. Fundus examination revealed ring reflex in macula.
Fundus fluorescein angiography showed ink blot appearance. Focal laser given and the patient regained his vision.
Central serous retinopathy is a fluid detachment of macula layers from their supporting tissue. This allows choroidal fluid to leak in to the sub-retinal space due to small breaks in the retinal pigment epithelium. Cause is unknown, stress played an important role.CSR has also been associated with cortisol and corticosteroids. Person with CSR have high levels of cortisol. The prognosis is generally excellent and over 90% will regain vision.
Diagnosis starts with Fundus examination, followed with confirmation by Fundus fluorescein angiography(FFA) and optical coherence tomography. FFA Showed one or more fluorescent spots with fluid leakage . 10-15% of this case presents with “classic smoke stack” appearance and “ink blot” appearance. Amsler grid useful in documenting visual field.
Treatment should be considered if it does not disappear within 3-4 months. Focal laser photocoagulation will be helpful in improving visual prognosis.