hyperparathyroidism (PHPT) is rare condition during pregnancy; however, it can carry significant risks to both mother and foetus during and after the pregnancy. The definitive treatment is parathyroidectomy undertaken preferably in the second or early third trimester. A case of asymptomatic PHPT diagnosed and treated by parathyroidectomy in the third trimester is described

Khan, Mohammed Ataullah

Khan, Rozilla Sadia

Naru, Tahira

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eCommons@AKUDepartment of Obstetrics & Gynaecology Division of Woman and Child HealthApril 2011Primary hyperparathyrodism in pregnancy andreview of literatureTahira NaruAga Khan UniversityRozilla Sadia KhanAga Khan UniversityMohammed Ataullah KhanAga Khan UniversityFollow this and additional works at: http://ecommons.aku.edu/pakistan_fhs_mc_women_childhealth_obstet_gynaecolPart of the Obstetrics and Gynecology CommonsRecommended CitationNaru, T., Khan, R., Khan, M. (2011). Primary hyperparathyrodism in pregnancy and review of literature. Journal of the PakistanMedical Association, 61(4), 401-3.Available at: http://ecommons.aku.edu/pakistan_fhs_mc_women_childhealth_obstet_gynaecol/7401 J Pak Med AssocCase ReportPrimary hyperparathyrodism in pregnancy and review of literatureTahira Naru,1 Rozilla Sadia Khan,2 Mohammed Ataullah Khan3Department of Obstetrics and Gynaecology,1,2 Department of Medicine,3 Aga Khan University Hospital, Karachi, Pakistan.AbstractPrimary hyperparathyroidism (PHPT) is rarecondition during pregnancy; however, it can carry significantrisks to both mother and foetus during and after thepregnancy. The definitive treatment is parathyroidectomyundertaken preferably in the second or early third trimester. Acase of asymptomatic PHPT diagnosed and treated byparathyroidectomy in the third trimester is described.Keywords: Hyperparathyroidism, Parathyroid adenoma,hypercalcaemia, pregnancy.IntroductionPHPT is an uncommon condition, usually diagnosedin people in their fifth or sixth decade of life. Its prevalencein the general population is 0.15%. Although the exactincidence during pregnancy is not known, the incidence ofPHPT in women of child bearing age is estimated to be 8cases per 100,000 per year.1 Physiological changes associatedwith pregnancy make this condition difficult to diagnose, and80% of affected pregnant women are found to beasymptomatic. The pregnant female is at a risk of significantpregnancy problems and complications ranging from anuncomplicated delivery to miscarriages, stillbirth or evenneonatal death.1,2 It is therefore important for the obstetrician,surgeon, paediatrician and endocrinologist to be aware of thiscondition, and its potential complications to define a clearapproach for its management.We present a case of asymptomatic primaryhyperparathyroidism treated by parathyroidectomy in thethird trimester, in a mother after the development of tetany ina new born infant.Case ReportA 23-year-old woman, gravida 2, Para 1, was bookedat 19 weeks gestation. Her previous pregnancy wasuneventful, but at 3 months of age her daughter had hadseizures and was found to have low calcium and vitamin Dlevels with normal Parathyroid hormone (PTH) levels. Shewas given calcium and vitamin D therapy. The mother wasalso evaluated and found to have calcium levels of 10.6mg/dl(normal range 8.6-10.5) borderline high and very lowVitamin D levels <1.1ng/ml (Normal range => 30ng/ml).PTH was not done. She was given intramuscular injection of600,000 IU vitamin D once a month for six months. Aftertreatment her vitamin D level was 23.6 ng/ml.On the basis of the previous history of seizures in theinfant, she was investigated in the index pregnancy whenvitamin D level was normal (28.92 ng/ml) and calcium levelswere raised (12.7 mg/dl). She was referred to theendocrinologist. Further investigation revealed raised PTH 224 pg/mg(normal 18-67) with decreased serum phosphorus andmagnesium (1.6 mg/dl) with normal urinary calcium (238mg/24hour) and renal functions. An Ultrasound scan of thethyroid and parathyroid revealed normal texture of both lobesof thyroid and a hypoechoic mass lesion measuring 8.4 X 6.4mm seen on the posterior-inferior to lower pole of left thyroidlobe. Colour Doppler showed normal flow. The diagnosis ofprimary hyperparathyroidism was made on the basis ofbiochemical and radiological investigations at 22 weeks ofgestation.Patient consented for Parathyroidectomy after 5weeks due to fear of complications. A left inferiorparathyroidectomy was performed at 27 weeks gestation withcyclogest given postoperatively as a tocolytic drug. Transienthypocalcaemia was observed in the postoperative period andcalcium carbonate 2gm daily was started. Calcidol 0.25 mcgwas also given orally to prevent hypocalcaemia.Histopathology confirmed a benign adenoma weighing 0.3gm. The foetus was monitored by serial growth scans andremained normal. Patient opted for an elective caesareansection at 38 gestational weeks and delivered a healthy babyboy weighing 3 kilogram with good Apgar scores. Herpostoperative period was uneventful. She is currentlymaintaining a calcium level of 9.0 dl/ml on one gram calciumintake per day. The baby was also monitored with calciumlevel and maintained his calcium level without developmentof any seizure yet at the age of ten months.DiscussionPrimary hyperparathyroidism in pregnancy needs tobe recognized and treated due to the risks it confers to bothmother and foetus. In the mother, the symptoms ofhypercalcaemia secondary to hyperparathyroidism aresimilar to those in the non-pregnant patient.3 Maternal andfoetal complication rates have been quoted to be as high as67% and 80% respectively.4,5 Nephrolithiasis is the mostcommon symptomatic presentation with an increasedincidence of 24-36% during pregnancy.6Approximately 40% of patients with PHPT arehypercalciuric, and most of the remaining patients havenormal values.7 The calcium excreted in a 24-h urinecollection varies with age, sex, and race, and one 24-hcollection does not capture the large variability in themeasurement, therefore most studies on PHPT use serumcalcium and PTH for assessment.8Other complications include bone disease (osteitisfibrosa cystica), pancreatitis and hyperemesis gravidarum.Potentially, the most serious maternal complication ishypercalcaemic crisis, which classically presents withnausea, vomiting, weakness, dehydration and altered mentalstate changes. There can also be a rapid progression touraemia, coma and death.2,4 Other manifestations of PPHTinclude spontaneous miscarriage, intrauterine growthretardation, low birth weight, premature delivery andintrauterine foetal death. In the postpartum period, maternalhypercalcaemia can worsen due to the removal of the foetaldemand for calcium. Untreated hyperparathyroidism has 80%foetal complication rate.6 The incidence of a neonatalcomplication has been reported as high as 53% of which 27 -31% is neonatal death.3,5Symptomatic PPHT is rarely detected in pregnancydue to the physiological changes that mask the symptoms,this includes maternal blood volume expansion,hypoalbuminaemia, increased foetal calcium requirements,and increased calcium clearance.Calcium metabolism in pregnancy is a dynamicprocess. Maternal serum calcium falls by about 10% inpregnancy; however as the serum albumin falls by 20%, theionized calcium remains unchanged. Foetal 1, 25-dihydroxyvitamin D, synthesized in foetal kidney andplacenta, acts as the major stimulus and regulator of calciumtransfer across the placenta.It increases maternal gastrointestinal absorption ofcalcium by 150mg -400mg daily, additionally maternalurinary excretion is also increased from 90 to 300 mg daily.Major foetal calcium demands of approximately 25-30g, arerequired in the 3rd trimester for skeletal tissue mineralization.This requires an active transport of calcium across theplacenta and the foetal serum calcium remains higher thanmaternal blood. Primary hyperparathyroidism results in highconcentrations of foetal serum calcium that acts to suppressthe parathyroid glands. Foetal calcitonin concentrations arehigh to encourage bone mineralization. At birth however, theneonate is suddenly deprived of this source of calcium. It isincapable of mobilizing calcium from bone owing to the lowconcentrations of parathyroid hormone and highVol. 61, No. 4, April 2011 402concentrations of calcitonin. Acute neonatal hypocalcaemiaresults in tetany and convulsions, usually at 5 to 14 days ofage. If the infant is breast fed, tetany can be delayed by onemonth or more.2,9Diagnosis of primary hyperparathyroidism should beconfirmed by elevated serum calcium and PTH levels.Preoperative imaging by ultrasound of the parathyroid glandsis recommended. Parathyroid ultrasound has a sensitivity of69% and specificity of 94% and is safe in pregnancy. Thegold standard is Technetium-99m sestamibi and ultrasound,with a combined sensitivity of 94.5%.10 However,Technetium-99m sestamibi is contraindicated in pregnancyand mostly an ultrasound will suffice. Other causes of hypercalcaemia like excessiveingestion of calcium and vitamin D, thiazide diuretics,lithium therapy, sarcoidosis and haemoconcentration must beruled out. Studies suggest that the foetal mortality rate can bereduced by a factor of four if operative cure is achieved.5,6Management:Treatment of hyperparathyroidism during pregnancyshould be individualized, based on the severity of disease,symptoms, and gestational age of the foetus. A significantdecline in maternal and peri natal complications may beachieved with a high index of suspicion, appropriatediagnosis, and timely management of mothers. In certainsituations intervention may be required, but excision ofparathyroid adenoma remains the only definitive treatment.In most reported cases, the adenoma accounts for 90%of primary hyperparathyroidism, carcinoma for 2% andhyperplasia (8-9%) for the remaining number of cases.3Role of medical therapy in pregnancy: Medical therapy in pregnancy for symptomaticprimary hyperparathyroidism has been discouraged, due tosafety issues of drug therapy, and the suboptimal control ofserum calcium which leads to a high foetal loss rate.6However in symptom-free patients or those with noradiologically identifiable parathyroid adenoma or those withmild hypercalcaemia diagnosed in 3rd trimester, may bemanaged medically, postponing operation until after delivery.If conservative management is considered intensive maternaland foetal surveillance should be initiated.Medical therapy primarily involves stabilizing thepatient with hydration, limiting calcium intake, correctingelectrolyte imbalance, administration of magnesium sulfate,oral phosphate and calciuretic diuretics.2,3Role of surgery: Symptomatic and poorly controlled hypercalcaemicpatients should be offered surgical resection which can becarried out in the second or early third trimester. Earlier reports have suggested low complication rates,most common being laryngeal nerve injury orhypoparathyroidism, with a quoted mortality rate of 0.1%.6However a recent review of surgically managed patients whounderwent parathyroidectomy even in the third trimester,found it to be effective and safe with reduced maternal andfoetal risk for complications. Post operative hypocalcaemiacan be easily managed with calcium and vitamin Dsupplementation.6,11Conclusion Primary hyperparathyroidism during pregnancythough rare, is associated with high maternal, foetal andneonatal mortality. Increased awareness, earlier detection,and better management are the key to decreasing itscomplications. A surgical option should be considered inview of a poor control of the hypercalcaemic state inpregnancy by medical or conservative management.Hyperparathyroidism during pregnancy may be safely treatedwith parathyroidectomy when performed during the secondtrimester or early third trimester.Reference1. Heath H 3rd, Hodgson SF, Kennedy MA. Primary hyperparathroidsim.Incidence, morbidity, and potential economic impact in a community. N Eng JMed 1980; 302: 189-93.2. Kohlmeier L, Marcus R. Calcium disorders of pregnancy. Endocrinol MetabClin North Am 1995; 24: 15-39.3. Kelly TR. Primary hyperparathyroidism during pregnancy. Surgery 1991; 110:1028-34.4. Kort KC, Schiller HJ, Numaan PJ, Hyperparathyroidism and pregnancy. Am JSurg 1999; 177: 66-8. 5. Delmonico FL, Neer RM, Cosimi AB, Barnes AB, Russell PS.Hyperparathyroidism during pregnancy. Am J Surg 1976; 131: 328-37.6. Schnatz PF, Curry SL. Primary hyperparathyroidism in pregnancy: evidence-based management.Obstst Gynecol Surv 2002; 57: 365-76.7. Silverberg, SJ, Shane, E, Jacobs, TP, Siris ES, Gartenberg F, Seldin D, et al.Nephrolithiasis and bone involvement in primary hyperparathyroidism. Am JMed 1990; 89: 327-34.8. Silverberg SJ, Lewiecki EM, Mosekilde L, Peacock M, Rubin MR. Presentationof Asymptomatic Primary Hyperparathyroidism: Proceedings of the ThirdInternational Workshop. J Clin Endocrinol Metab 2009; 94: 351-65.9. Cushard WG Jr, Creditor MA, Canterbury JM, Reiss E. Physiologichyperparathyroidism in pregnancy. J Clin Endocrinol Metab 1972; 34: 767-71.10. Lumachi F , Zucchetta P, Marzola MC, Boccagni P, Angelini F, Bui F, et al.Advantages of combined technetium-99m sestamibi scintigraphy and highresolution ultrasonography in parathyroid localization: comparative study in 91pateints with primary hyperparathyroidism. Eur J Endocrinol 2000; 143: 755-60.11. Schnatz PF, Thaxton S. Parathyroidectomy in the third trimester of pregnancy.Obstet Gynecol Surv 2005; 60: 672-82.403 J Pak Med Assoc

Primary hyperparathyrodism in pregnancy and review of literature

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Primary hyperparathyrodism in pregnancy and review of literature

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