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Smolarczyk R.Premature ovarian insufciency—aetiopathology, epidemiology, and diagnostic evaluation. Prz Menopauzalny. 2018;17(3):105–8. https://doi.org/10.5114/pm.2018.78550.
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15. ScienceDirect.Com. Current evaluation of amenorrhea—ScienceDirect. Science, Health and
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https://doi.org/10.1093/humrep/deg005.
P. Bhatt and R. Myneni
Chapter 34
Forgotten Copper T: AnIntrauterine
Contraceptive Device Misdiagnosed
asSecondary Sterility
PushpaBhatt andRevathiMyneni
Learning Objectives
By the end of this presentation, the clinician will be able to:
1. Create a diagnosis based on history and clinical presentation conrmed by X-ray.
2. Discuss and summarize a case with forgotten copper IUD which can be misdiagnosed as secondary sterility.
3. Discuss the clinical case presentation of the patient and what were the features
that resulted in the misdiagnosis.
4. Enumerate the differential diagnosis of secondary sterility.
5. Emphasize the need for consent and proper communication with the patient.
6. Apply the knowledge gained from the case in a clinical setting.
Introduction
The abbreviation “IUD” stands for intrauterine device. It offers long-term contraception. It is a “T”-shaped device inserted into the uterine cavity. The T-shaped
plastic frame with copper wire around stops sperm from fertilizing with the egg,
thus preventing pregnancy. This device is also called copper T. An intrauterine
device (IUD) is a highly effective and reversible long-term method of family planning used in Asia; 27% of women use reversible IUD globally; the IUD is the most
widely used reversible contraceptive. In Scandinavia, 20–40% of contraceptive
users have IUD, while about 60 million Chinese women use this type of contraceptive [1]. IUD use varies by nation, reecting differences in culture, contraceptive
P. Bhatt (*) · R. Myneni
St. Martinus University Faculty of Medicine, Willemstad, Curacao
e-mail: puhshpa.bhatt@martinus.edu
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023
H. Tohid et al. (eds.), The Misdiagnosis Casebook in Clinical Medicine,
https://doi.org/10.1007/978-3-031-28296-6_34
241
242
P. Bhatt and R. Myneni
availability and choice, provider views and training, and women’s fertility objectives. IUD for women wishing to practice long-term contraception is highly effective birth control without having to resort to hormonal methods [2]. Copper T can
be inserted anytime during a normal menstrual cycle. For breastfeeding mothers
they are recommended 8 weeks after delivery before inserting copper T. Before
inserting, healthcare providers evaluate overall health and do a pelvic exam to
exclude pregnancy and evaluate the status of the uterus. The patient is also screened
for sexually transmitted infections (STI) . Common complications after insertion
include vaginal discharge, failed insertion, pain, infection, menstrual disorders, and
perforation [3, 4]. Sometimes it remains inside the uterus for more than 10 years.
Many times patients are unaware of its existence inside. We are reporting such a
case where we found copper T placement as an accidental nding during secondary
sterility management. In this case copper T was inserted at one of the hospitals of
India. She was seeking treatment for secondary sterility. The case was managed
with different modality.
Clinical Case Presentation
A 35-year-old married woman, gravida 1 and para 1, was referred to Baitadi hospital of Far Western Region of Nepal with the complaints of vaginal discharge off and
on and being unable to conceive for the past 14 years. Her past history revealed that
5 years after the birth of her rst baby, she was unsuccessful to conceive further. She
went to several health facilities where they offered different treatments such as dilation and curettage (D&C), oral contraceptive for menstrual regulation for few
months, treatment for sexually transmitted diseases, etc. This continued off and on
for 12 years in India and in Nepal as well. She was under treatment from the primary
health care of Patan village development committee, where she was referred to
Baitadi hospital. In Baitadi hospital, her thorough history revealed that, for the last
few months, her vaginal discharge has increased. She was given vaginal tablets for
7 days and both husband and wife were treated for chlamydia and referred to our
hospital for secondary sterility. Her history revealed that she lived in Guwahati in
India with her husband. There she had delivered a female baby. Patient had a followup visit for immunization of the baby after 6 weeks of delivery. She was offered
some counseling on family planning and asked to visit after 6 months.
This patient revealed, 6 months after the birth of her baby after the rst menses,
she visited a family planning clinic to seek advice in Gohati hospital. She was
examined and asked to lie down on a table. The patient was told to watch for the
thread that should hang down into the vagina. She never felt it. The patient did not
realize that it was placed. However, she was given a bunch of papers to keep them
safely for her record. She was unaware of having any consent. There was confusion
because the patient was unable to remember if at all the IUD insertion took place or
not. And she did not notice any thread as well. A thorough history of her menstrual
cycle was taken, and cross-questioning for Depo injection (people’s choice in Nepal
34 Forgotten Copper T: An Intrauterine Contraceptive Device Misdiagnosed…
243
for long-term family planning) was asked and IUD insertion (once it was a very
popular family planning program in India) as well. Among the migrant and mobile
couples, copper T is the choice for family planning in India. On speculum examination she had moderate vaginal discharge and on per vaginal examination showed an
anteverted normal size and non-tender uterus. The semen analysis result of her husband was normal. Her blood test was all in a normal range. Venereal disease research
laboratory (VDRL) tests for both were negative. We wanted to exclude all possibilities for sec sterility. Pelvic X-ray was done, and it showed IUD is in the pelvic cavity based on her history of presence of copper T, so it was easy to anticipate that due
to the copper T, she was unable to conceive. The patient’s history was taken into
consideration and she was referred to a referral hospital in Pithoragarh district,
located at the border between India and Nepal, for surgical removal of copperT. After
7 days the patient came back to Baitadi hospital with the copper T in her hand that
was removed under general anesthesia. Her report shows that “through cervical
dilatation copper T was removed, lying in the cavity of a normal size uterus, and the
size of the thread was too small.” She was given antibiotics for 5 days and asked to
plan further to conceive. In 1 year she conceived and visited Baitadi hospital for
antenatal care.
Discussion
Globally, it is estimated that more than 60 million women use IUD, and the forgotten IUD are not documented [5]. There are several reasons for this phenomenon:
the IUD may fragment at removal, the absence of threads may be misinterpreted, or
the patient may simply have forgotten that she had an IUD inserted. Presently various types of IUD are available. Long-acting reversible contraceptive (LARC)
methods, including IUD, the levonorgestrel-releasing intrauterine system (LNGIUS), depot- medroxyprogesterone acetate (DMPA) injections, and implants, are
highly cost- effective than the oral contraceptive pill. Furthermore, IUD, intrauterine suppositories, and implants are more cost-effective than injectable contraceptives [6]. IUD are good choice for breastfeeding women and those who had abortion,
have some diseases such as diabetes, or have chronic migraines with or without
aura. The effectiveness, safety, long duration of action, and reversibility of IUD
made it a popular choice among women in the global population. Intrauterine contraceptive devices are used by over 50% of the women in some Asian countries and
between 6 and 27% in Europe [7]. Woman who aspire to use an IUD device, whatever the type should get detailed information in the form of oral as well as written
format that will alert them on what family planning method to choose [6]. Most
importantly they should get routine follow-up after the insertion of the IUD.This
follow-up is required to conrm the presence of IUD in its place and also to exclude
if any unwanted symptoms appear after the insertion such as bleeding, pain, and
discharge [8]. Women should be advised that menstrual bleeding and cramping
may initially increase with the use of the copper T. Some investigators have already
244
P. Bhatt and R. Myneni
reported on gynecologic symptoms caused by such forgotten IUD, such as actinomycotic pyometra [9] and dysfunctional uterine bleeding and perforations [10].
The patient was not given relevant information on follow-up. So she lost the followup contacts. When she aspired to have a second baby, she was not investigated for
IUD.She unnecessarily had to go to different institutions where she received irrelevant investigation and treatment. Studies indicate that the copper IUD exerts its
contraceptive effects primarily by preventing fertilization through inhibition of
sperm motility and viability [11]. The copper ions cause an intrauterine inammatory response that is cytotoxic to sperm and phagocytizes them; 18h after natural
insemination, no viable spermatozoa remain in the endometrial cavity. Copper has
a direct negative impact on sperm motility and the capacity of sperm to enter cervical mucus. Copper ions also cause inammatory changes around the egg during
ovulation, which is similar to what women with endometriosis experience. The US
Food and Drug Administration (FDA) has approved the use of the copper IUD for
up to 10 continuous years, during which it remains highly effective. It has a reported
failure rate at 1 year of 0.8 per 100 women [12]. The most common side effects
reported are heavy menstrual bleeding and pain [13]. If our patient had received
X-ray, the IUD would have been visualized early enough to avoid all timeconsuming follow-up for secondary sterility. The patient was not a case of secondary sterility but misdiagnosed as such because of the copper T, making her unable
to conceive, that was forgotten by a patient and not identied by healthcare providers [13].
Conclusion
There is not much data on forgotten IUDs, but we believe issues and problems
should be reported more. Counseling is the central issue to be addressed effectively. To avoid unintended pregnancy, healthcare providers at all levels should
incorporate effective counseling for using IUCD during the postpartum period.
The most effective long-term, safe, and reversible method is IUCD. Counseling
should be clear and be offered with informed consent. Follow-up is required with
IUCD to see if copper T strings are in the right place. Our patient needed to be
counseled more, and she could not locate the strings; thus, she was lost for followup. The healthcare providers may have offered to counsel, she was not able to
understand well enough to report at the healthcare facility, or she ignored the consequences. Healthcare providers are therefore required to be sure that all information is well understood. The family planning services should aim to improve
quality through proper counseling and follow-up services which help to support
women if they choose to use IUCD in the postpartum period effectively. It is
essential to consider unexplained secondary sterility resulting from a forgotten IUD.
34 Forgotten Copper T: An Intrauterine Contraceptive Device Misdiagnosed…
245
References
1. Gorvett Z.Why isn’t this birth control used more? BBC Future. BBC—Homepage. https://
www.bbc.com/future/article/20180830- what- to- know- about- the- coil- or- iud- contraceptivemethod/. Accessed 15 Oct 2022.
2. Iyengar K, Iyengar SD. The Copper-T 380A IUD: a ten-year alternative to female sterilisation in India. Reproductive Health Matters. 2000;8(16):125–33. https://doi.org/10.1016/
S0968- 8080(00)90194- 0.
3. The Swaddle. IUDs in India: the most effective, long lasting birth control no one uses. The
Swaddle. https://www.facebook.com/theswaddleindia. Accessed 9 Aug 2018. https://theswad-
dle.com/iuds- in- india- most- effective- long- lasting- birth- control- method- no- one- uses/
4. Thapa S, Dangal G, Karki A, Bajracharya N.Missing intrauterine device copper-T: case series.
J Nepal Health Res Counc. 2018;16(40):354–6. https://doi.org/10.3126/jnhrc.v16i3.21438.
5. Knudsen HJ, Rasmussen K. The forgotten intrauterine device: a cause of infertility. Arch
Gynecol Obstet. 1993;253(3):143–4. https://doi.org/10.1007/BF02767331.
6. NHS National Institute for Health and Clinical Experience (NICE). Long-acting reversible
contraception: quick reference guide. Clinical guideline No 30. Oct 2005. Available at: http://
www.nice.org.uk/CG030quickrefguide. Accessed 1 July 2010.
7. d’Arcangues C. Worldwide use of intrauterine devices for contraception. Contraception.
2007;75(6 Suppl):S2–7. https://doi.org/10.1016/j.contraception.2006.12.024.
8. Ron-El R, Weinraub Z, Langer R, Bukovsky I, Caspi E.The importance of ultrasonography
in infertile women with “forgotten” intrauterine contraceptive devices. Am J Obstet Gynecol.
1989;161(1):211–2. https://doi.org/10.1016/0002- 9378(89)90268- 8.
9. Agarwal N, Kriplani A.Secondary infertility and dysfunctional uterine bleeding from a Lippes
loop placed 32 years earlier. Int J Gynaecol Obstet. 2000;69(2):167–8. https://doi.org/10.1016/
s0020- 7292(99)00205- 2.
10. Kriplani A, Buckshee K, Relan S, Kapila K. ‘Forgotten’ intrauterine device leading to
actinomycotic pyometra—13 years after menopause. Eur J Obstet Gynecol Reprod Biol.
1994;53(3):215–6. https://doi.org/10.1016/0028- 2243(94)90122- 8.
11. Rivera R, Yacobson I, Grimes D.The mechanism of action of hormonal contraceptives and
intrauterine contraceptive devices. Am J Obstet Gynecol. 1999;181(5 Pt 1):1263–9. https://doi.
org/10.1016/s0002- 9378(99)70120- 1.
12. Trussell J. Contraceptive failure in the United States. Contraception. 2011;83(5):397–404.
https://doi.org/10.1016/j.contraception.2011.01.021.
13. Godfrey EM, Folger SG, Jeng G, Jamieson DJ, Curtis KM.Treatment of bleeding irregularities in women with copper-containing IUDs: a systematic review. Contraception.
2013;87(5):549–66. https://doi.org/10.1016/j.contraception.2012.09.006.
Part VII
Hematology
Chapter 35
Congenital Methemoglobinemia
Misdiagnosed asPolycythemia Vera
MohammedMohammed
Learning Objectives
By the end of this presentation, the clinician will be able to:
1. Dene methemoglobinemia.
2. Consider the diagnosis of congenital methemoglobinemia in patients presenting
with cyanosis and dyspnea when cardiopulmonary causes are excluded.
3. Evaluate a patient suspected to have methemoglobinemia.
4. Avoid unnecessary procedures and treatments in patients suspected to have congenital methemoglobinemia.
5. Formulate an appropriate management plan for patients with congenital
methemoglobinemia.
Introduction
Methemoglobinemia is a rare hematologic disorder that causes cyanosis and dyspnea unrelated to cardiac and pulmonary causes. Methemoglobinemia is usually
asymptomatic, even when methemoglobin levels are as elevated as 40% of the total
Hb levels [1]. Hereditary congenital methemoglobinemia due to deciency of nicotinamide adenine dinucleotide (NADH) cytochrome b5 reductase enzyme is a
remarkably rare recessive inherited disorder which is not well reported in medical
literature. In this clinical case of congenital methemoglobinemia, the patient presented with persistent cyanosis and polycythemia in the absence of cardiopulmonary causes which led to a misdiagnosis of polycythemia vera for which the patient
unnecessarily underwent bone marrow biopsy and was treated with imatinib.
M. Mohammed (*)
St. Martinus University Faculty of Medicine, Willemstad, Curacao
e-mail: mohammed.mohammed@martinus.edu
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023
H. Tohid et al. (eds.), The Misdiagnosis Casebook in Clinical Medicine,
https://doi.org/10.1007/978-3-031-28296-6_35
249
250
M. Mohammed
Clinical Case Presentation
A 29-year-old man from India had generalized fatigue and discoloration of hands
for 6 months. His CBC revealed high red blood cell (RBC) count (6.7×106/uL),
increased Hb (20g/dL) with normal leukocyte and platelet counts, and unremarkable peripheral blood smear. The patient was evaluated in another medical center
based on his persistent polycythemia, and he was diagnosed with polycythemia
vera. Bone marrow examination showed erythroid hyperplasia but without panmyelosis. Despite the fact that molecular studies were negative for JAK2 mutation, the
patient was treated with imatinib based on the assumed diagnosis of polycythemia vera.
In a different institute, the patient was reevaluated, and he was found to have
cyanosis of the face and both upper limbs since the age of 13 years old, but it
became more evident in the last 1 year following treatment with imatinib after the
assumed diagnosis of polycythemia vera. There was no history of chest pain, syncope, or palpitations. There was no history of medication intake or exposure to
oxidants. He had no history of growth or developmental retardation or neurologic
manifestations and no hepatosplenomegaly. Pulse oximetry revealed oxygen saturation of 92%, however the patient didn’t show any evidence of respiratory distress,
and his clinical examination was unremarkable. The diagnosis of polycythemia vera
was challenged and ruled out because of negative JAK2 gene mutation, normal
erythropoietin level, and absence of panmyelosis features in the bone marrow examination, which was repeated 4 months after stopping treatment with imatinib. A
different diagnosis of hemoglobin M disease is considered because of the cyanosis
and no evidence of cardiopulmonary disease; however, hemoglobinopathies screening by high-performance liquid chromatography and manual hemoglobin electrophoresis showed normal hemoglobin pattern. Arterial blood gas showed the
following results: pH, 7.380 (normal range: 7.35–7.45); PCO2, 38 mmHg (normal
range: 35–45mmHg); PO2, 96–101mmHg (normal range: 83–108mmHg); arterial
O2 saturation, 100% (normal range: 95–99.0%); low HCO3 at 21.8mmol/L (normal
range: 23–29mmol/L); and low oxygen saturation (O2 Hb) at 62.4% (normal range:
94–98%).
The nding of cyanosis and low oxygen saturation despite the normal arterial
oxygen tension was strongly suggestive of methemoglobinemia.
Arterial blood gas showed a methemoglobin level of 38% (normal range:
0–1.5%). Cytochrome b5 reductase (methemoglobin reductase B) was found to be
decient at level of <2.6U/g Hb (normal range: 6.6–13.3), and therefore the diagnosis of congenital methemoglobinemia was conrmed. Karyotyping was done and
showed no chromosomal abnormalities. Other family members had no family history of hemoglobin disorders or cyanosis, and they were asymptomatic. He was
managed with vitamin C 500mg once daily because of the lack of methylene blue
tablets. After 1 month of treatment, the cyanosis improved signicantly and methemoglobin levels were reduced remarkably from 38 to 19.2%.
35 Congenital Methemoglobinemia Misdiagnosed asPolycythemia Vera
251
Differential Diagnosis
1. Polycythemia vera
2. Hemoglobin M disease
3. Acquired methemoglobinemia
What WasMisdiagnosed inThis Case andWhy?
Congenital methemoglobinemia was misdiagnosed because of the following
reasons:
1. Cyanosis was overlooked.
2. Not taking into account the bone marrow ndings (absence of features of pan-
myelosis) and the negative JAK2 mutation.
3. Lack of systematic epidemiological studies about congenital
methemoglobinemia.
Discussion
Methemoglobin results when ferrous iron is oxidized to ferric iron within the heme
component of hemoglobin [1]. Normally, less than 1% of hemoglobin is present in
the oxidized form (methemoglobin), which has decreased the ability to carry oxygen, but with increased oxygen afnity at the remaining binding sites [2]. This leads
to decrease of oxygen delivery to tissue causing hypoxemia and lactic acidosis.
Acquired methemoglobinemia due to oxidizing agents is not uncommon; on the
other hand, congenital deciency of the methemoglobin reductase B enzyme is
extremely rare, and only very few cases are reported in medical literature worldwide; therefore the clinical characteristics and the incidence of congenital methemoglobinemia are almost unknown because of the underreporting of these cases.
The patient had a saturation gap which occurs when there is a difference between
the sulfur dioxide (SO2) measured by pulse oximetry (the lower value) and the SO2
on arterial blood-gas analysis. This was the hint for the diagnosis of methemoglobinemia. Patients with methemoglobinemia typically have a saturation gap of
greater than 5% [3]. The failure of very high oxygen saturation to correct cyanosis
is highly suggestive of methemoglobinemia. Patients with congenital methemoglobinemia develop physiological compensatory mechanisms and can remain asymptomatic even with high levels of methemoglobin (up to 40%). These compensatory
mechanisms include changes in the concentration of 2,3-diphosphoglycerate and
pH, synthesis of globin chains, and secondary polycythemia [4, 5].
If levels of methemoglobin exceed 70%, that is usually fatal [1].