Review Gynecol Obstet Invest 2014;78:213–223 DOI: 10.1159/000358828
Received: May 7, 2013 Accepted after revision: January 18, 2014 Published online: March 26, 2014
Developmental and Clinical Overview of Lithopaidion Giuseppe Santoro a Antonio Simone Laganà b Emanuele Sturlese b Valentina Giacobbe b Annalisa Retto b Vittorio Palmara b Departments of a Biomedical Sciences and Morpho-Functional Images and b Pediatric, Gynaecological, Microbiological and Biomedical Sciences, University of Messina, Messina, Italy
Key Words Lithopaidion · Lithopedion · Lithopaedion · Lithokelyphos · Lithokelyphopedion · Lithokelyphopaedion · Abdominal pregnancy · Fallopian ectopic pregnancy
Abstract Lithopaidion, or stone child, is generally a single rare asymptomatic formation that evolves from an undiagnosed and untreated advanced abdominal pregnancy. The dead fetus is retained in the maternal abdominal cavity, which causes calcification. In this paper, we review the literature on the epidemiology, etiopathogenesis and clinical features of lithopaidion and report a unique case of lithokelyphos in a patient with an ectopic fallopian pregnancy. We propose a model to unify the data. The new word ‘lithopaidion’ can be utilized instead of lithopedion. © 2014 S. Karger AG, Basel
Introduction
Lithopaidion (stone child) is a rare formation that evolves from an undiagnosed and untreated advanced abdominal pregnancy in which the dead fetus is retained in © 2014 S. Karger AG, Basel 0378–7346/14/0784–0213$39.50/0 E-Mail [email protected] www.karger.com/goi
the maternal abdominal cavity with consequent calcification [1, 2]. In this review, we performed a MEDLINE search using ‘lithopedion’, ‘lithopaedion’, ‘lithopaidion’, ‘lithokelyphos’, ‘lithokelyphopedion’, ‘lithokelyphopaedion’ and ‘calcified abdominal pregnancy’ as keywords. A total of 162 articles were identified through this search, with publication dates from 1893 to 2013. Overall, 21 articles were cited twice, 1 article was cited 3 times, and 86 papers were excluded because the abstract was not available (n = 40), the study was not published in English (n = 30) or the subject was not related to the topic of our review (n = 17). Thus, 52 references were selected [1, 3–53]. The full paper was available for 30 articles, and 22 abstracts provided sufficient information. All of these articles had been published in international peer-reviewed journals. In addition, a total of 20 other references were considered: 11 about lithopaidion (9 papers quoted in MEDLINE articles, 1 book chapter and 1 open access article) and 9 articles related to the physiopathology of the female genital tracts; of these, 8 were published in international peerreviewed journals, and 1 was a book chapter [54–73]. The essential data of the papers are shown in table 1.
Giuseppe Santoro Department of Biomedical Sciences and Morpho-Functional Images AOU Policlinic ‘G. Martino’, Via Consolare Valeria IT–98125 Messina (Italy) E-Mail giuseppe.santoro @ unime.it
Table 1. Essential data of included papers First author, year
Category
Essential data
Chang, 2001
case report
1
Pescetto, 2001 Frayer, 1999
book chapter case report
Rothschild, 1993
case report
7 × 4 × 4 cm lithopaidion retained for 50 years, corresponding to 20th week of fetal death, was found in a 76-year-old woman affected by cervical neoplasm site of ectopic pregnancy lithopaidion retained for 37 years, extending from pelvis to lower costal margins, was found in a 67-year-old woman identification of a 3,100-year-old lithopaidion in the Archaic Southwest antedates its first clinical notation by 2,100 years lithopaidion corresponding to 15th week of fetal death was diagnosed lithopaidion retained for 4 years was found in the abdominal cavity, during laparotomy for a complete rupture of the uterus, in a 41-year-old woman lithokelyphopedion, retained for at least 20 years, was found in a 69-year-old woman lithokelyphopedion was diagnosed by magnetic resonance rare case of lithopaidion with coexistent cervical pregnancy historical case of lithopaidion in a 50-year-old woman in 1582 a lithopaidion was found during a necroscopy performed on a Madame Chatri who died at the age of 68 comments to Dr. Bondeson’s assumptions about the D’Ailleboust family rare case of lithopaidion, found in the omentum during laparotomy, with coexistent tubal pregnancy lithopaidion retained for 1 year was found among coils of small intestine, with the head covered by great omentum, in a 31-year-old woman lithopaidion retained for 15 years, corresponding to the 3rd month of pregnancy, was removed from the right uterine appendages during laparotomy 6 × 4.5 × 3.2 cm lithopaidion retained for 12 years was found in a 33-year-old patient 6 months pregnant, with a 3-week history of acute pain localized in the right groin, a lump in the right groin painful to touch and swelling and pain in the right leg lithopaidion retained for 45 years was found at the brim of the pelvis in a 67-year-old woman; a fibromyoma was also present lithopaidion retained for 29 years was found in the abdominal cavity of a 54-year-old woman suffering from acute dyspnea lithokelyphopedion retained for more than 45 years was found above the pubis of an obese woman, aged 83, who died of cerebral hemorrhage 15 × 25 cm infra-umbilical lithopaidion retained for 18 years, corresponding to 21st week of fetal death, was found in a 40-year-old woman 8 × 4 × 3 cm lithopaidion retained for 9 years was found in the abdomen of a 54-year-old woman
10 11
Shah-hosseini, 1987 case report Kobuch, 1984 case report Korényi-Both, 1978 Chako, 1996 Bustamante Sarabia, 1989 Bjerke, 2007 Noble, 2003
case report case report case report
Claoué, 1996 Venter, 1980 Griffith, 1930
letters to editor case report proceedings
Whitehouse, 1922 Luker, 1914
case report
Fraser, 1913
case report
Dean, 1893
case report
Cave, 1937
note with case report case report
Passini, 2000 Chase, 1968 Bondeson, 1996 Odom, 2006
Schwarz, 1952 Ede, 2011 Fagan, 1980
Speiser, 1995 Burger, 2007
Lammes, 2000 Jain, 2000
214
case report letters to editor
case report
Reference
case report and review historical review historical case of ‘stone-child of Sens’ and review of the literature case report and an unrecognized and long-standing lithopaidion, corresponding to 20th week of fetal death, with review erosion into the bowel with fecal fistula formation in a 33-year-old woman; a literature review was also shown case report 17 × 11 × 8.5 cm lithokelyphopaedion, result of a tubal pregnancy, corresponding to 32nd week of fetal death, was found in a dead woman of 40 – 50 years of age case report lithopaidion, corresponding to 30th week of fetal death, was found in a 69-year-old woman who underwent a left formal above-knee amputation for a dry gangrene case report 2 cases of lithopaidion were encountered; in 1 case, computed tomographic findings were illustrated; moreover, a calcified fetus and investing membranes were readily identified on a plain film of the abdomen case report lithopaidion retained for 60 years, corresponding to 31st week of fetal death, was found in a 92-year-old woman who died 1 week after admission to hospital for marasmus senilis and pneumonia images in lithopaidion was diagnosed and removed by laparoscopy in a 33-year-old woman reproductive medicine case report lithopaidion mimicking an ovarian tumor was diagnosed in a 62-year-old woman with aspecific symptoms in the lower abdomen case report lithopaidion, presenting as a pelvic abscess and corresponding to 16th week of fetal death, with an abdominal pregnancy was diagnosed in a 38-year-old woman
Gynecol Obstet Invest 2014;78:213–223 DOI: 10.1159/000358828
2 3 4 5 6 7 8 9
12 13 14 15 16
17 18 19 20 21 22 23
24 25 26
27 28
29 30
Santoro /Laganà /Sturlese /Giacobbe / Retto /Palmara
Table 1 (continued) First author, year
Category
Essential data
Reference
Lachman, 2001
case report and review
31
Kim, 2002
case report
El Hag, 2002
case report
Mishra, 2007
case report
Massinde, 2009
case report
lithopaidion retained for at least 40 years, corresponding to 34th week of intrauterine fetal death, was found in an 80-year-old woman affected by severe abdominal pain; she died 2 days later as a result of renal failure 11 × 11 × 8 cm lithopaidion presented as ovarian tumor without a symptom and retained for 40 years, corresponding to 20th week of fetal death, was found embedded within the omentum in a 63-year-old woman by laparotomy lithopaidion 10 cm in diameter, with coexistent extrauterine placental site trophoblastic tumor, retained for 5 years, corresponding to 5th month of fetal death, was found in the pouch of Douglas of a 35-year-old woman, who presented with acute abdomen and peritonitis following rectal perforation by laparotomy twin lithopaidions retained for 8 years, corresponding to 5th month of fetal death, were found behind the left ovary and in the right iliac fossa of a 40-year-old woman, suffering from features of acute intestinal obstruction lithopaidion with coexistent live abdominal ectopic pregnancy were removed by laparotomy in a multigravida with complaints of abdominal swelling that had been occurring for 2 years lithopaidion retained for 35 years was diagnosed by computed tomographic scan for an asymptomatic palpable mass in a 74-year-old woman
Sun G, 2010
images in reproductive medicine Sun J, 2010 brief calcified fetal skull was located in the uterine cavity of a 34-year-old patient, whereas the other body communications parts, including the spine and limbs, were situated in the abdominal cavity Gonçalves, 2011 case report lithokelyphopaedion retained for 50 years was found adherent to the deep plans around the umbilical region and the hypogastrium in a 77-year-old woman suffering from dehydration, urinary tract infection and infected limb pressure ulcers Yeh, 2013 case report and selective reduction of a heterotopic abdominal pregnancy during the 2nd trimester using fetal review intracardiac injection with potassium chloride enabled subsequent vaginal delivery of the intrauterine pregnancy at term and the abdominal fetus remained as a stable lithopaidion Sunday-Adeoye, review retrospective analysis of all 20 cases of abdominal pregnancies between 1976 and 2006 at the Mater 2011 Misericordiae Hospital, Afikpo, southeastern Nigeria; there were 4 live births, 2 early neonatal deaths and 4 cases of lithopaidion (1 coexisting with an ongoing intrauterine pregnancy) Roberts, 1952 medical unusual case of 1 fetus, in a twin pregnancy of a 29-year-old woman; death was due to an infarction memoranda or to a small hemorrhage in the neighborhood of the cord, and becoming almost completely calcified Bustamante Sarabia, case report lithopaidion in a true cervical pregnancy manifested with intermittent vaginal bleeding, fetid 1989 leukorrhea and slight pelvic pain Luwuliza-Kirunda, case report case of primary hepatic pregnancy presenting as a lithopaidion in the right hypochondrium 1978 Huerta Bahena, case report lithopaidion incidentally diagnosed during diagnostic workup in a woman with invasive carcinoma 1994 of the cervix Spirtos, 1987 case report and lithokelyphos and its instrumental diagnosis review Newman, 1983 case report and lithokelyphos was presented as an undiagnosed abdominal mass in a woman with cervical cancer review N’Gbesso, 1998 case report abdominal plain film alone is sufficient for diagnosis of lithopaidion and undertaking surgery Glass, 1953 case report retrocolic lithopaidion adhesed to the ascending colon caused a volvulus of the cecum; the case was further complicated by atresia of the right main stem bronchus with either total atelectasis or congenital absence of the right lung, with a resultant marked shift of the mediastinum Zaheer, 1971 case report lithopaidion was found in the pelvis to the right of the midline of a 36-year-old woman suffering from right-sided colicky abdominal pain accompanied by nausea and vomiting; it determined acute intestinal obstruction after accidental uterine perforation Leke, 1983 case report and case of obstructed labor in a term pregnancy caused by cephalopelvic disproportion as a result of a review lithopaidion retained in a sacculus of the lower uterine segment Binns, 1966 correspondence lithopaidion, after silent uterine rupture, retained for 3 years was found in the right lower quadrant of the abdomen of a 25-year-old woman Reiss, 1996 letters to editor the lithopaidion found in the abdominal cavity by Bondeson was determined by a ruptured uterus Pragay, 1979 case report biochemical analysis of a lithopaidion found in the abdominal cavity of a 69-year-old woman who had died of unrelated causes; quantitative inorganic analysis of tissues showed significant decrease of K and Cl, significant increase in Mg, P, Na and enormous increase in Ca Küchenmeister, 1881 review review of ancient records of lithopaidion
Overview of Lithopaidion
Gynecol Obstet Invest 2014;78:213–223 DOI: 10.1159/000358828
32
33
34
35 36
37 38
39
40
41
42 43 44 45 46 47 48
49
50 51 52 53
54
215
Table 1 (continued) First author, year
Category
Essential data
Reference
Bainbridge, 1912
lithopaidion as a result of extrauterine pregnancy
55
9 lithopaidions in 44 cases of extrauterine pregnancy lithopaidion in which a carcinoma developed review of 236 case of lithopaidions favorable conditions for the deposition of calcium in lithopaidion all changes which an abdominal fetus may undergo if it is not removed lithopaidion as a result of extrauterine pregnancy
56 57 58 59 60 61
Poole, 2012 Croxatto, 2002 Moore, 1988 Talo, 1991 Dixon, 2009 Brundin, 1969
review original article original article original article original article book chapter
coexistent lithopaidion and contralateral tubal pregnancy history of lithopaidion lithokelyphopaedion retained for 2 years, corresponding to 5th month of fetal death, was found in the pelvis of a 32-year-old woman affected by intestinal acute obstruction incidence of ectopic pregnancies in Italy ectopic pregnancy: incidence, risk factors, clinical data and treatment the authors studied the distribution of ectopic pregnancy sites, immediate complications, determining factors, and subsequent fertility abdominal ectopic pregnancy: location and treatment physiology of gamete and embryo transport through the fallopian tube effects of estrogen on contractions of the smooth muscle of rat oviduct role of myosalpinx in gamete and embryo transport oviduct interstitial cells of Cajal were demonstrated for pacemaker activity the mammalian oviduct
62 63 64
Parazzini, 2013 Tay, 2000 Bouyer, 2002
case report and review review case report review case report review case report and review case report book chapter open access case report original article review original article
Masson, 1928 Auvray, 1924 Reeves, 1941 Emmanuel, 1966 D’Aunoy, 1922 Tien, 1949 Temple, 1959 Kelly, 1863 Allodé, 2012
Classification
68 69 70 71 72 73
Epidemiology
The classification proposed by Küchenmeister in 1881 [54] and reviewed by Dean and Marnoch in 1893 [18] and Cave in 1937 [19] describes three types of ‘lithopedion’: (1) the first is called ‘lithokelyphos’ (stone sheath or egg shell) and is when the membranes are calcified but not the fetus; (2) the second is called ‘lithokelyphopaedion’ (stone sheath child) and is when the membranes and the fetus are calcified and are adherent to one another; (3) the last category is ‘true lithopaedion’ or ‘lithoteknon’ (stone child) and is when only the child’s body is calcified; the escape of the fetus into the abdominal cavity, leaving its membranes behind, is said to account for this type. Lately, the suffix ‘pedion’ has been preferred over ‘peadion’. The latter term was a Latin transformation (ae: diphthong) of the Greek term ‘pedion’, which in turn originated from the Greek term ‘παιδιον’ (paidion). For this reason it is possible to use the term ‘lithopaidion’ instead of lithopedion.
216
65 66 67
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According to the literature, the incidence of abdominal pregnancy is approximately 1 in 11,000 pregnancies, and lithopaidions represent 1.5–1.8% of these cases [20]. Küchenmeister [54], to whom we owe so much knowledge of this condition prior to 1881, informs us that an Arabic doctor called Albucasis found a lithopaidion approximately 1,000 years ago. Other cases were described later. In 1540, Christopher Bain removed a lithopaidion from an Italian woman [21]. In 1557, Spach, as quoted by Bainbridge [55] and Chase [21], showed the figure of a lithopaidion drawn in situ with the mother’s belly laid open in surgery. In 1582, the so-called ‘lithopedion Senonensis’ (lithopedion of Sens), which was removed from a dead woman in the town of Sens in France, was reported and became one of the foremost curiosities of France [22]; it has since become immortal among English-speaking people through its misuse in Tristram Shandy [63]. At Leinzell in 1720, a lithopaidion was observed during the autopsy of a 74-year-old woman who had carried it within her for 48 years, during which time she had several children [63]. Similarly, in 1721, Anna Mullern, 46 years before her death, declared herself pregnant; the swelling Santoro /Laganà /Sturlese /Giacobbe / Retto /Palmara
remained until she died at the age of 94. She desired that her body be examined after death; the surgeon broke open the mass with a hatchet and determined it to be a lithopaidion [21]. In 1881, Küchenmeister [54] reviewed 49 cases of lithopaidion published between 1582 and 1881 (table 2), which were quoted by Dean and Marnoch in 1893 [18]. Around 1880/1885, 46 cases were reported [24], although until 1900 there had been only 38 recorded cases, and in the next 12 years only 12 more cases were reported [21]. Masson and Simon [56] referred to 9 cases of lithopaidion at the Mayo Clinic from 1903 to 1926, and 9 more cases were seen there subsequently [21]. In 1924, Auvray [57] reported the first case of a lithopaidion in which a carcinoma developed [21]. In 1937, Cave [19] reported 196 cases up to 1933 and an insignificant number after 1933. In 1941, Reeves and Lipman [58] reported 236 cases, and an additional 15 cases were reported between 1947 and 1951 [23]. In 1952, Schwarz [24] referred to 196 cases recorded by 1937. In 1968, Chase [21] reported 270 cases. In 2000, Passini et al. [20] found fewer than 300 reported cases in 400 years of world medical literature; however, from 1968 to 1999 at least 47 new cases of lithopaidion were published. Moreover, it has been confirmed that in the last 10 years, as prenatal care and surveillance have increased, the presentation of lithopaidions is rare [28], even though at least 17 cases of lithopaidions were demonstrated in the last century [1, 20, 23, 25, 28–39, 64]; only in this case, we have not considered the 4 cases of lithopaidions reported by Sunday-Adeoye et al. [40] because it was not possible to determine the exact century of their occurrence. However, a complete analysis of the English literature has revealed more than 330 case of lithopaidion so far. See table 2 for a list of studies showing the number of lithopaidion cases reviewed. Based on a literature search in this field, the patient’s age at the time of diagnosis varied from 23 [61] or 30 years [25] to 100 years [25, 61], with a mean age of 55 years. The mean tolerated duration of lithopaidion was 22 years; 9 of 128 women selected by Tien [61] had carried them for more than 50 years, and more than 60% of women were over 40 years old, as recorded for women of the last century [20]. The period over which the fetus was retained in the abdominal cavity varied from 4 to 60 years [23, 25–27], and fetal death occurred between the third and sixth month of gestational age in 1/5 of cases, between the seventh and eighth month in approximately 1/4 of cases and at full term in approximately 2/5 of cases [20]. A lithopaidion can originate from a primary abdominal pregnancy, an aborted tubal pregnancy or an intrauterine Overview of Lithopaidion
Table 2. List of studies showing the number of lithopaidion cases
reviewed First author, year
Number of lithopaidion cases reviewed
Kuchenmeister, between 1582 and 1881, 49 cases 1881 were reviewed Schwarz, 1952 during 1880 – 1885 approximately, 46 cases were reported Chase, 1968 until 1900, only 38 cases had been reported, and in the next 12 years only 12 more case were added Masson, 1928; there were 9 cases of lithopaidion at Chase, 1968 the Mayo Clinic from 1903 to 1926, and later 9 more cases were seen there Cave, 1937 up to 1933, there were 196 cases Reeves, 1941 up to 1941, 236 cases were reported Odom, 2006 an additional 15 cases were reported between 1947 and 1951 Schwarz, 1952 196 cases were recorded by 1937 Chase, 1968 270 cases were reported Passini, 2000 fewer than 300 cases Currently more than 330 cases
Reference 54 24 21
56; 21
19 58 23 24 21 20
The first case of lithopaidion was discovered by Albucasis in approximately 880. The subsequent reviews show dissimilarities. A complete analysis of the English literature has revealed more than 330 cases of lithopaidion so far.
pregnancy followed by uterine rupture [1, 2]. Tien [61] collected 114 cases of lithopaidions, of which 74 were the result of the gestational sac rupture of a tubal pregnancy, 13 originated from an ovarian pregnancy, 8 were the result of a primary abdominal pregnancy, and in 5 patients the lithopaidion originated in the horn of a bicornuate uterus. Lithopaidion is generally a single obstetric phenomenon; the literature includes 1 report of twins with lithopaidion [34] and a case of lithopaidion in twins [41], 1 case of lithopaidion with a coexistent live abdominal ectopic pregnancy [35], a case of lithopaidion formation after a cervical pregnancy [42], a case of lithopaidion evolving from hepatic pregnancy [43], 1 case with a coexistent placental site trophoblastic tumor that was discovered upon the removal of the lithopaidion (after a colostomy for rectal perforation was performed) [33], and 2 cases with coexistent cervical neoplasm [1, 44] (table 3). Moreover, in this report, we document the first case of lithopaidion in a patient with an ectopic fallopian pregGynecol Obstet Invest 2014;78:213–223 DOI: 10.1159/000358828
217
Fig. 1. Ultrasound observation: right fallopian tube with an apparent gestational chamber (arrow).
Fig. 2. Ultrasound observation: left adnexa with corpus luteum (section A).
Table 3. Lithopaidion is generally a single obstetric phenomenon, but coexistent pathologies are sometimes discovered
diuretics and angiotensin II receptor antagonists. An examination revealed external genitals and a nulliparous vagina, a uterus with increased volume and a posterior well-closed cervix. The left adnexa were not palpable, and the posterior fornix was painful. Upon hospitalization, her body temperature, blood pressure and heart rate were normal. The hematic values were normal, and her β-HCG value was 4,141 mIU/ml. Mycoplasma urealyticum vaginal infection was discovered. The patient underwent a transvaginal ultrasound, which revealed an enlarged uterus showing a myoma. The right fallopian tube was enlarged with an apparent gestational chamber (fig. 1). The left adnexa had increased volume and showed a 37 × 36-mm corpus luteum (fig. 2). There was 20 mm of hematic fluid in the pouch of Douglas. After being briefed on the possible surgical procedures, she underwent a laparoscopy with general anesthesia. In hemodynamically stable patients, laparoscopic treatment of ectopic pregnancy offers major economic and health benefits compared with laparotomy. After the pneumoperitoneum was performed with 3.5 liters of preheated CO2, the principal intra-umbilical opening was prepared for the introduction of the laparoscopic optics. We performed two accessory openings: left and right. We observed hemoperitoneum, a uterus with increased volume and a right fallopian tube with increased volume. The fallopian tube was expanded because of an ectopic pregnancy in the ampulla. The right adnexa were regular, and the left adnexa had increased volume from a bleeding corpus luteum. Coagulation of bleeding from the left adnexa and ovarian
First author, year
Coexistent pathologies
Auvray, 1924
case of a lithopaidion in which a carcinoma developed Roberts, 1952 lithopaidion in twins Luwuliza-Kirunda, lithopaidion evolving from 1978 hepatic pregnancy Bustamante Sarabia, lithopaidion formation after a 1989 cervical pregnancy Huerta Bahena, 1994; coexistent cervical neoplasm Chang, 2001 El Hag, 2002 coexistent placental site trophoblastic tumor Mishra, 2007 twins with lithopaidion Massinde, 2009 coexistent live abdominal ectopic pregnancy
Reference 57 41 43 42 44; 1 33 34 35
nancy. After 6 weeks of amenorrhea, a 32-year-old woman with parity 0-0-1-0 was admitted to the hospital for pelvic pain and metrorrhagia. Her history included the following parameters: menarche occurred at the age of 14, and menses had been regular since. She had undergone a cystectomy of the right ovary by laparoscopy 13 years previously. At the age of 30, she had amenorrhea with positive β-HCG; her β-HCG values decreased after 10 days. She had high blood pressure and was treated with thiazide 218
Gynecol Obstet Invest 2014;78:213–223 DOI: 10.1159/000358828
Santoro /Laganà /Sturlese /Giacobbe / Retto /Palmara
Color version available online
drilling were performed. A right salpingectomy was performed through electrical resection. In the pouch of Douglas, we found a new 1 × 2 × 2.5-cm formation (fig. 3) that was removed and sent for a histological examination. The postsurgical course was regular, and the day after surgery the woman was discharged normally. The notes from the histological examination referred to ovular tissue with extended coagulative necrosis and a confluent area of calcification, which was defined as a lithokelyphos. The principal features of this case are summarized in table 4. Approval by the ethics committee and informed consent from the patient were obtained.
Etiopathogenesis
Lithopaidion is a rare formation that evolves from an undiagnosed and untreated advanced abdominal pregnancy. Ectopic pregnancy is a public health problem that has increased in the last years [65]; it represents the major cause of maternal mortality, but its etiology [66] and pathogenesis are not clearly understood. Several risk factors have been identified such as pelvic inflammatory disease (especially for infections involving Chlamydia trachomatis), previous ectopic pregnancy, tubal damage from infection or surgery, a history of infertility treatment, treatment for in vitro fertilization, increased age, smoking, and past or current use of an intrauterine device [66, 67]. Abdominal pregnancies should be considered in all patients until an intrauterine location can be confirmed [68]. Until now, ectopic pregnancy and lithopaidion have been observed as single, separate obstetric phenomena. Our observed case documents an occurrence of lithopaidion in a patient with an ectopic fallopian pregnancy. Only 3 similar cases have been reported previously [13, 62]. Temple and Hester [62] reported a lithopaidion and a contralateral tubal pregnancy, while Venter and Campher [13] described a tubal pregnancy and a lithopaidion in the omentum; it has not been possible to read these reports for an appropriate evaluation of the data. The third case was referred to as a coexistent lithopaidion of advanced gestation with a live abdominal ectopic pregnancy at 15 weeks of gestation [35]. Ectopic pregnancy and lithopaidion imply a change in embryo transport. When they are observed in the same patient, we can hypothesize a common pathogenesis that is caused by altered fallopian contractions. The embryo is passively transported to the uterus by highly regulated and coordinated mechanical events in which the stochasOverview of Lithopaidion
Fig. 3. Macroscopic find of right fallopian ectopic pregnancy (im-
age A) and lithopaidion (image B).
Table 4. Principal features of a patient with lithopaidion and an ectopic fallopian pregnancy
Age of woman at time of diagnosis Retain time of lithopaidion Dimension of lithopaidion Time of fetal death Types of lithopaidion Place of lithopaidion Concomitant pathologies Symptoms Instrumental diagnosis Differential diagnosis Complications Type of intervention
32 years 2 years 1 × 2 × 2.5 cm 6 weeks lithokelyphos pouch of Douglas fallopian ectopic pregnancy, myoma pelvic pains, metrorrhagia during laparoscopy none none laparoscopy
tic contractile activity of the myosalpinx plays a dominant role [69]. Moreover, the embryonic transit is characterized by rapid back-and-forth movements, as the simultaneous and successive contractions and relaxations of the myosalpinx along the isthmus causes changes in the gradients of intraluminal pressure [70]. These movements are generated by several pacemakers that are constantly changing locations [71]. The interstitial cells of Cajal, Gynecol Obstet Invest 2014;78:213–223 DOI: 10.1159/000358828
219
Table 5. Percentage of frequent symptoms in women with litho-
Table 6. Percentage of symptomatic and asymptomatic women
paidion Symptoms
Percentage
Pain Heaviness Compression syndromes
39.4% 24.2% 36.4%
which populate the human oviduct, have recently been identified as specialized pacemaker cells [72]. The propagation of the myoelectric activity that is initiated by the pacemakers occurs in both directions over short stretches at a pace of 1–2 mm/s. The permanent relocation of the pacemakers along the oviduct causes the luminal content to move at random. The tonic contractions that occur at the ampullary-isthmic junction and the uterotubal junction are responsible for the transient arrest of transport that occurs at these points [73]. Alteration of the contractile function of the myosalpinx can determine the fallopian implantation or expulsion of the embryo. In the present paper, we report a lithopaidion and a right fallopian pregnancy with a bleeding corpus luteum in the left adnexa. This condition presumes an internal (from the left fallopian tube to the right fallopian tube through the uterus) or external (from the left fallopian tube to the right fallopian tube through the peritoneal cavity) expulsion of the fertilized egg. The report of a lithopaidion together with a tubal pregnancy highlights the fundamental importance of evaluating the clinical history of the patient to avoid further episodes and to target therapy and repeated controls. Even if the presence of a lithopaidion did not alter the surgical strategy for the ectopic pregnancy, the presence of anatomical and functional abnormalities of the female genitalia suggest the possibility that the ectopic pregnancy could be repeated, which would compromise the procreative capacity of the patient.
Clinical Features
Most lithopaidions are found incidentally [1]. No laboratory tests or typical symptoms are currently able to diagnose a lithopaidion [1]. The patient is often unaware of her condition. The usual history is of a false pregnancy followed by gradual decrease in the size of the abdomen and the return of menstruation. Frequent symptoms are abdominal/pelvic pain (even if its character is variable), abdominal/pelvic heaviness (that is not specific) and 220
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Patients
Percentage
Asymptomatic Symptomatic
90% 10%
compression syndromes particularly affecting the urinary tract and bowel (table 5). The differential diagnosis on the presentation of lithopaidion may include ovarian neoplasm [32], myomas, inflammatory masses, epiploon calcifications, and urinary tract or bladder tumors [45]. The majority of cases are asymptomatic (table 6) and are discovered incidentally in the course of clinical examination, on a straight abdominal radiograph for other pathologies [20, 21, 25, 35], or even postmortem [19, 22, 24]. This last detection method in particular led us to believe that there are more than 330 reported lithopaidions because many cases remain unrecognized. The X-ray differential diagnosis may include other masses that contain large amounts of calcium, including ovarian tumors, epiploic appendage and aortic aneurism. Computed tomography could better define the cause of the pelvic mass [26, 36, 46, 47]. Moreover, the lithopaidion is more common in situations linked to low socioeconomic status and lack of prenatal care [23, 25]. Particularly in the last millennium, 80% of patients with lithopaidion presented from areas lacking accurate diagnostic instruments. Lithopaidion can cause bowel obstruction in rare cases; in 1953, Glass and Abramson [48] reported the occurrence of volvulus of the cecum due to a lithopaidion, and Zaheer [49] (in 1971) and recently Allodé et al. [64] showed a small bowel obstruction due to the same cause. Mishra et al. [34] reported a case with features of intestinal obstruction. Jain and Eckert [30] reported a lithopaidion-like pelvic abscess, while Leke et al. [50] reported a case of obstructed labor in a term pregnancy caused by cephalopelvic disproportion as a result of a lithopaidion retained in a sacculus of the lower uterine segment. Odom et al. [23] managed an event of erosion into the bowel with fecal fistula formation caused by a lithopaidion (table 7). To form a lithopaidion, the following four conditions are required [2, 19, 59]: (1) survival of the fetus for over 3 months because if the death occurs while the bones are cartilaginous, absorption will be rapid and complete; Santoro /Laganà /Sturlese /Giacobbe / Retto /Palmara
Table 7. Lithopaidion is generally an asymptomatic phenomenon,
Table 8. The lithopaidion model: principal features to be consid-
but complications from the lithopaidion are sometimes discovered
ered
First author, year
Complications
Glass, 1953 Leke, 1983 Jain, 2000
volvulus of the cecum obstructed labor in a term pregnancy presentation of a lithopedion-like pelvic abscess Odom, 2006 erosion into the bowel with fecal fistula Mishra, 2007 features of intestinal obstruction Allodé, 2012 small bowel obstruction
Reference 48 50 30 23 34 64
Age of woman at time of diagnosis Retain time of lithopaidion Dimension of lithopaidion Time of fetal death Types of lithopaidion Place of lithopaidion Concomitant pathologies Symptoms: specific, aspecific Instrumental diagnosis Differential diagnosis Complications Type of intervention
(2) sterility of the fetus; (3) failure of medical detection; (4) presence of conditions favorable for the deposition of calcium. Cave [19], in 1937, supposed the presence of another requirement: the pregnancy had to be extrauterine. He affirmed that in the very early literature, approximately half a dozen cases were intrauterine, but given that no such cases have been reported within the last 100 years, it is reasonable to view the authenticity of these cases with extreme skepticism. Instead, analyzing the available literature has allowed us to find different papers that attest to a tight relationship between the uterus and the lithopaidion: in 1966, Binns [51] found a lithopaidion formation following the silent rupture of the uterus, similar to that found by Reiss [52] in 1996. In 2001, Lachman et al. [31] found a fetus that was hyperflexed with other signs of intrauterine growth. Moreover, recently, a calcified fetal skull was located in the uterine cavity of a 34-year-old patient, whereas the other body parts, including the spine and limbs, were situated in the abdominal cavity. A uterine incision surrounded by an old scar was observed in the anterior wall of the uterus. Therefore, it seems likely that rupture of the cesarean scar had caused the fetus to partly enter into the abdominal cavity [37]. D’Aunoy and King [60], as reviewed by Chase in 1968 [21], listed four processes that an abdominal fetus may undergo if it is not removed: (1) skeletonization: only the bones of the fetus remain following the disintegration and absorption of the soft parts; (2) formation of adipocere: the soft parts are replaced by fatty acids, soaps and salts of palmitic and stearic acids;
(3) suppuration: the fetus is destroyed after an abscess has formed, usually due to Escherichia coli infection; (4) true lithopaidion formation: this occurs if the fetus remains sterile and becomes infiltrated with calcium salts to varying degrees. From a biochemical point of view, a lithopaidion has a shell consisting mostly of inorganic constituents, and in the internal tissue there is significant depletion of K and Cl, significant enrichment of Mg, P and Na, and enormous enrichment of Ca (compatible with the calcification of the tissues). In the liver and muscle, there are discernible LDH isoenzymes, and in the brain and muscle discernible CK isoenzymes are present [53]. Considering that the surgical management of lithopaidion is usually simple with a very positive course even in the elderly, no intraoperative death due to lithopaidion has been reported recently [64], with the exception of 2 deaths that were not related to lithopaidion in which the patient died of pulmonary embolus postoperatively after a formal left above-knee amputation performed for dry gangrene [25] or the death was the result of renal failure [31]. Considering the possibility of future complications, the proper procedure is surgical removal. Although laparoscopy is used to explore the pelvic cavity and manage pelvic masses worldwide, the majority of discovered lithopaidions have been removed by classic laparotomic surgery, with the exception of a case of a young woman undergoing laparoscopy for infertility during which a lithopaidion was discovered [28] and our case of a 32-yearold woman who underwent a laparoscopy for an ectopic fallopian pregnancy.
Overview of Lithopaidion
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Conclusion
Lithopaidion is generally a single asymptomatic obstetric phenomenon that evolves from an undiagnosed and untreated advanced abdominal pregnancy and is discovered accidentally long after the pregnancy or even postmortem. With the inclusion of the latter, the total number of cases is higher than the approximately 330 cases reported in the literature, and its modern presentation,
particularly in countries with low socioeconomic status, is little more than 1 case per year because of increased prenatal care and surveillance. In the pathogenesis of lithopaidion, a role for the specialized pacemaker cells that populate the human oviduct has been hypothesized. Considering the fragmentation of the data presented in the literature, we suggest a model (lithopaidion model) unifying the evidence of lithopaidion (table 8). When it is diagnosed, the proper procedure is surgical removal.
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Overview of Lithopaidion
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66 Tay JI, Moore J, Walker JJ: Ectopic pregnancy. BMJ 2000;320:916–919. 67 Bouyer J, Coste J, Fernandez H, Pouly JL, JobSpira N: Sites of ectopic pregnancy: a 10 year population-based study of 1,800 cases. Hum Reprod 2002;17:3224–3230. 68 Poole A, Haas D, Magann EF: Early abdominal ectopic pregnancies: a systematic review of the literature. Gynecol Obstet Invest 2012; 74:249–260. 69 Croxatto HB: Physiology of gamete and embryo transport through the fallopian tube. Reprod Biomed Online 2002;4:160–169. 70 Moore GD, Croxatto HB: Effects of delayed transfer and treatment with oestrogen on the transport of microspheres by the rat oviduct. J Reprod Fertil 1988;83:795–802. 71 Talo A: How the myosalpinx works in gamete and embryo transport. Arch Biol Med Exp (Santiago) 1991;24:361–375. 72 Dixon RE, Hwang SJ, Hennig GW, Ramsey KH, Schripsema JH, Sanders KM, Ward SM: Chlamydia infection causes loss of pacemaker cells and inhibits oocyte transport in the mouse oviduct. Biol Reprod 2009; 80: 665– 673. 73 Brundin J: Pharmacology of the oviduct; in Hafez ES, Blandau RJ (eds): The Mammalian Oviduct. Chicago, University of Chicago Press, 1969, pp 261–269.
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Copyright: S. Karger AG, Basel 2014. Reproduced with the permission of S. Karger AG, Basel. Further reproduction or distribution (electronic or otherwise) is prohibited without permission from the copyright holder.
Received: May 7, 2013 Accepted after revision: January 18, 2014 Published online: March 26, 2014
Developmental and Clinical Overview of Lithopaidion Giuseppe Santoro a Antonio Simone Laganà b Emanuele Sturlese b Valentina Giacobbe b Annalisa Retto b Vittorio Palmara b Departments of a Biomedical Sciences and Morpho-Functional Images and b Pediatric, Gynaecological, Microbiological and Biomedical Sciences, University of Messina, Messina, Italy
Key Words Lithopaidion · Lithopedion · Lithopaedion · Lithokelyphos · Lithokelyphopedion · Lithokelyphopaedion · Abdominal pregnancy · Fallopian ectopic pregnancy
Abstract Lithopaidion, or stone child, is generally a single rare asymptomatic formation that evolves from an undiagnosed and untreated advanced abdominal pregnancy. The dead fetus is retained in the maternal abdominal cavity, which causes calcification. In this paper, we review the literature on the epidemiology, etiopathogenesis and clinical features of lithopaidion and report a unique case of lithokelyphos in a patient with an ectopic fallopian pregnancy. We propose a model to unify the data. The new word ‘lithopaidion’ can be utilized instead of lithopedion. © 2014 S. Karger AG, Basel
Introduction
Lithopaidion (stone child) is a rare formation that evolves from an undiagnosed and untreated advanced abdominal pregnancy in which the dead fetus is retained in © 2014 S. Karger AG, Basel 0378–7346/14/0784–0213$39.50/0 E-Mail [email protected] www.karger.com/goi
the maternal abdominal cavity with consequent calcification [1, 2]. In this review, we performed a MEDLINE search using ‘lithopedion’, ‘lithopaedion’, ‘lithopaidion’, ‘lithokelyphos’, ‘lithokelyphopedion’, ‘lithokelyphopaedion’ and ‘calcified abdominal pregnancy’ as keywords. A total of 162 articles were identified through this search, with publication dates from 1893 to 2013. Overall, 21 articles were cited twice, 1 article was cited 3 times, and 86 papers were excluded because the abstract was not available (n = 40), the study was not published in English (n = 30) or the subject was not related to the topic of our review (n = 17). Thus, 52 references were selected [1, 3–53]. The full paper was available for 30 articles, and 22 abstracts provided sufficient information. All of these articles had been published in international peer-reviewed journals. In addition, a total of 20 other references were considered: 11 about lithopaidion (9 papers quoted in MEDLINE articles, 1 book chapter and 1 open access article) and 9 articles related to the physiopathology of the female genital tracts; of these, 8 were published in international peerreviewed journals, and 1 was a book chapter [54–73]. The essential data of the papers are shown in table 1.
Giuseppe Santoro Department of Biomedical Sciences and Morpho-Functional Images AOU Policlinic ‘G. Martino’, Via Consolare Valeria IT–98125 Messina (Italy) E-Mail giuseppe.santoro @ unime.it
Table 1. Essential data of included papers First author, year
Category
Essential data
Chang, 2001
case report
1
Pescetto, 2001 Frayer, 1999
book chapter case report
Rothschild, 1993
case report
7 × 4 × 4 cm lithopaidion retained for 50 years, corresponding to 20th week of fetal death, was found in a 76-year-old woman affected by cervical neoplasm site of ectopic pregnancy lithopaidion retained for 37 years, extending from pelvis to lower costal margins, was found in a 67-year-old woman identification of a 3,100-year-old lithopaidion in the Archaic Southwest antedates its first clinical notation by 2,100 years lithopaidion corresponding to 15th week of fetal death was diagnosed lithopaidion retained for 4 years was found in the abdominal cavity, during laparotomy for a complete rupture of the uterus, in a 41-year-old woman lithokelyphopedion, retained for at least 20 years, was found in a 69-year-old woman lithokelyphopedion was diagnosed by magnetic resonance rare case of lithopaidion with coexistent cervical pregnancy historical case of lithopaidion in a 50-year-old woman in 1582 a lithopaidion was found during a necroscopy performed on a Madame Chatri who died at the age of 68 comments to Dr. Bondeson’s assumptions about the D’Ailleboust family rare case of lithopaidion, found in the omentum during laparotomy, with coexistent tubal pregnancy lithopaidion retained for 1 year was found among coils of small intestine, with the head covered by great omentum, in a 31-year-old woman lithopaidion retained for 15 years, corresponding to the 3rd month of pregnancy, was removed from the right uterine appendages during laparotomy 6 × 4.5 × 3.2 cm lithopaidion retained for 12 years was found in a 33-year-old patient 6 months pregnant, with a 3-week history of acute pain localized in the right groin, a lump in the right groin painful to touch and swelling and pain in the right leg lithopaidion retained for 45 years was found at the brim of the pelvis in a 67-year-old woman; a fibromyoma was also present lithopaidion retained for 29 years was found in the abdominal cavity of a 54-year-old woman suffering from acute dyspnea lithokelyphopedion retained for more than 45 years was found above the pubis of an obese woman, aged 83, who died of cerebral hemorrhage 15 × 25 cm infra-umbilical lithopaidion retained for 18 years, corresponding to 21st week of fetal death, was found in a 40-year-old woman 8 × 4 × 3 cm lithopaidion retained for 9 years was found in the abdomen of a 54-year-old woman
10 11
Shah-hosseini, 1987 case report Kobuch, 1984 case report Korényi-Both, 1978 Chako, 1996 Bustamante Sarabia, 1989 Bjerke, 2007 Noble, 2003
case report case report case report
Claoué, 1996 Venter, 1980 Griffith, 1930
letters to editor case report proceedings
Whitehouse, 1922 Luker, 1914
case report
Fraser, 1913
case report
Dean, 1893
case report
Cave, 1937
note with case report case report
Passini, 2000 Chase, 1968 Bondeson, 1996 Odom, 2006
Schwarz, 1952 Ede, 2011 Fagan, 1980
Speiser, 1995 Burger, 2007
Lammes, 2000 Jain, 2000
214
case report letters to editor
case report
Reference
case report and review historical review historical case of ‘stone-child of Sens’ and review of the literature case report and an unrecognized and long-standing lithopaidion, corresponding to 20th week of fetal death, with review erosion into the bowel with fecal fistula formation in a 33-year-old woman; a literature review was also shown case report 17 × 11 × 8.5 cm lithokelyphopaedion, result of a tubal pregnancy, corresponding to 32nd week of fetal death, was found in a dead woman of 40 – 50 years of age case report lithopaidion, corresponding to 30th week of fetal death, was found in a 69-year-old woman who underwent a left formal above-knee amputation for a dry gangrene case report 2 cases of lithopaidion were encountered; in 1 case, computed tomographic findings were illustrated; moreover, a calcified fetus and investing membranes were readily identified on a plain film of the abdomen case report lithopaidion retained for 60 years, corresponding to 31st week of fetal death, was found in a 92-year-old woman who died 1 week after admission to hospital for marasmus senilis and pneumonia images in lithopaidion was diagnosed and removed by laparoscopy in a 33-year-old woman reproductive medicine case report lithopaidion mimicking an ovarian tumor was diagnosed in a 62-year-old woman with aspecific symptoms in the lower abdomen case report lithopaidion, presenting as a pelvic abscess and corresponding to 16th week of fetal death, with an abdominal pregnancy was diagnosed in a 38-year-old woman
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2 3 4 5 6 7 8 9
12 13 14 15 16
17 18 19 20 21 22 23
24 25 26
27 28
29 30
Santoro /Laganà /Sturlese /Giacobbe / Retto /Palmara
Table 1 (continued) First author, year
Category
Essential data
Reference
Lachman, 2001
case report and review
31
Kim, 2002
case report
El Hag, 2002
case report
Mishra, 2007
case report
Massinde, 2009
case report
lithopaidion retained for at least 40 years, corresponding to 34th week of intrauterine fetal death, was found in an 80-year-old woman affected by severe abdominal pain; she died 2 days later as a result of renal failure 11 × 11 × 8 cm lithopaidion presented as ovarian tumor without a symptom and retained for 40 years, corresponding to 20th week of fetal death, was found embedded within the omentum in a 63-year-old woman by laparotomy lithopaidion 10 cm in diameter, with coexistent extrauterine placental site trophoblastic tumor, retained for 5 years, corresponding to 5th month of fetal death, was found in the pouch of Douglas of a 35-year-old woman, who presented with acute abdomen and peritonitis following rectal perforation by laparotomy twin lithopaidions retained for 8 years, corresponding to 5th month of fetal death, were found behind the left ovary and in the right iliac fossa of a 40-year-old woman, suffering from features of acute intestinal obstruction lithopaidion with coexistent live abdominal ectopic pregnancy were removed by laparotomy in a multigravida with complaints of abdominal swelling that had been occurring for 2 years lithopaidion retained for 35 years was diagnosed by computed tomographic scan for an asymptomatic palpable mass in a 74-year-old woman
Sun G, 2010
images in reproductive medicine Sun J, 2010 brief calcified fetal skull was located in the uterine cavity of a 34-year-old patient, whereas the other body communications parts, including the spine and limbs, were situated in the abdominal cavity Gonçalves, 2011 case report lithokelyphopaedion retained for 50 years was found adherent to the deep plans around the umbilical region and the hypogastrium in a 77-year-old woman suffering from dehydration, urinary tract infection and infected limb pressure ulcers Yeh, 2013 case report and selective reduction of a heterotopic abdominal pregnancy during the 2nd trimester using fetal review intracardiac injection with potassium chloride enabled subsequent vaginal delivery of the intrauterine pregnancy at term and the abdominal fetus remained as a stable lithopaidion Sunday-Adeoye, review retrospective analysis of all 20 cases of abdominal pregnancies between 1976 and 2006 at the Mater 2011 Misericordiae Hospital, Afikpo, southeastern Nigeria; there were 4 live births, 2 early neonatal deaths and 4 cases of lithopaidion (1 coexisting with an ongoing intrauterine pregnancy) Roberts, 1952 medical unusual case of 1 fetus, in a twin pregnancy of a 29-year-old woman; death was due to an infarction memoranda or to a small hemorrhage in the neighborhood of the cord, and becoming almost completely calcified Bustamante Sarabia, case report lithopaidion in a true cervical pregnancy manifested with intermittent vaginal bleeding, fetid 1989 leukorrhea and slight pelvic pain Luwuliza-Kirunda, case report case of primary hepatic pregnancy presenting as a lithopaidion in the right hypochondrium 1978 Huerta Bahena, case report lithopaidion incidentally diagnosed during diagnostic workup in a woman with invasive carcinoma 1994 of the cervix Spirtos, 1987 case report and lithokelyphos and its instrumental diagnosis review Newman, 1983 case report and lithokelyphos was presented as an undiagnosed abdominal mass in a woman with cervical cancer review N’Gbesso, 1998 case report abdominal plain film alone is sufficient for diagnosis of lithopaidion and undertaking surgery Glass, 1953 case report retrocolic lithopaidion adhesed to the ascending colon caused a volvulus of the cecum; the case was further complicated by atresia of the right main stem bronchus with either total atelectasis or congenital absence of the right lung, with a resultant marked shift of the mediastinum Zaheer, 1971 case report lithopaidion was found in the pelvis to the right of the midline of a 36-year-old woman suffering from right-sided colicky abdominal pain accompanied by nausea and vomiting; it determined acute intestinal obstruction after accidental uterine perforation Leke, 1983 case report and case of obstructed labor in a term pregnancy caused by cephalopelvic disproportion as a result of a review lithopaidion retained in a sacculus of the lower uterine segment Binns, 1966 correspondence lithopaidion, after silent uterine rupture, retained for 3 years was found in the right lower quadrant of the abdomen of a 25-year-old woman Reiss, 1996 letters to editor the lithopaidion found in the abdominal cavity by Bondeson was determined by a ruptured uterus Pragay, 1979 case report biochemical analysis of a lithopaidion found in the abdominal cavity of a 69-year-old woman who had died of unrelated causes; quantitative inorganic analysis of tissues showed significant decrease of K and Cl, significant increase in Mg, P, Na and enormous increase in Ca Küchenmeister, 1881 review review of ancient records of lithopaidion
Overview of Lithopaidion
Gynecol Obstet Invest 2014;78:213–223 DOI: 10.1159/000358828
32
33
34
35 36
37 38
39
40
41
42 43 44 45 46 47 48
49
50 51 52 53
54
215
Table 1 (continued) First author, year
Category
Essential data
Reference
Bainbridge, 1912
lithopaidion as a result of extrauterine pregnancy
55
9 lithopaidions in 44 cases of extrauterine pregnancy lithopaidion in which a carcinoma developed review of 236 case of lithopaidions favorable conditions for the deposition of calcium in lithopaidion all changes which an abdominal fetus may undergo if it is not removed lithopaidion as a result of extrauterine pregnancy
56 57 58 59 60 61
Poole, 2012 Croxatto, 2002 Moore, 1988 Talo, 1991 Dixon, 2009 Brundin, 1969
review original article original article original article original article book chapter
coexistent lithopaidion and contralateral tubal pregnancy history of lithopaidion lithokelyphopaedion retained for 2 years, corresponding to 5th month of fetal death, was found in the pelvis of a 32-year-old woman affected by intestinal acute obstruction incidence of ectopic pregnancies in Italy ectopic pregnancy: incidence, risk factors, clinical data and treatment the authors studied the distribution of ectopic pregnancy sites, immediate complications, determining factors, and subsequent fertility abdominal ectopic pregnancy: location and treatment physiology of gamete and embryo transport through the fallopian tube effects of estrogen on contractions of the smooth muscle of rat oviduct role of myosalpinx in gamete and embryo transport oviduct interstitial cells of Cajal were demonstrated for pacemaker activity the mammalian oviduct
62 63 64
Parazzini, 2013 Tay, 2000 Bouyer, 2002
case report and review review case report review case report review case report and review case report book chapter open access case report original article review original article
Masson, 1928 Auvray, 1924 Reeves, 1941 Emmanuel, 1966 D’Aunoy, 1922 Tien, 1949 Temple, 1959 Kelly, 1863 Allodé, 2012
Classification
68 69 70 71 72 73
Epidemiology
The classification proposed by Küchenmeister in 1881 [54] and reviewed by Dean and Marnoch in 1893 [18] and Cave in 1937 [19] describes three types of ‘lithopedion’: (1) the first is called ‘lithokelyphos’ (stone sheath or egg shell) and is when the membranes are calcified but not the fetus; (2) the second is called ‘lithokelyphopaedion’ (stone sheath child) and is when the membranes and the fetus are calcified and are adherent to one another; (3) the last category is ‘true lithopaedion’ or ‘lithoteknon’ (stone child) and is when only the child’s body is calcified; the escape of the fetus into the abdominal cavity, leaving its membranes behind, is said to account for this type. Lately, the suffix ‘pedion’ has been preferred over ‘peadion’. The latter term was a Latin transformation (ae: diphthong) of the Greek term ‘pedion’, which in turn originated from the Greek term ‘παιδιον’ (paidion). For this reason it is possible to use the term ‘lithopaidion’ instead of lithopedion.
216
65 66 67
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According to the literature, the incidence of abdominal pregnancy is approximately 1 in 11,000 pregnancies, and lithopaidions represent 1.5–1.8% of these cases [20]. Küchenmeister [54], to whom we owe so much knowledge of this condition prior to 1881, informs us that an Arabic doctor called Albucasis found a lithopaidion approximately 1,000 years ago. Other cases were described later. In 1540, Christopher Bain removed a lithopaidion from an Italian woman [21]. In 1557, Spach, as quoted by Bainbridge [55] and Chase [21], showed the figure of a lithopaidion drawn in situ with the mother’s belly laid open in surgery. In 1582, the so-called ‘lithopedion Senonensis’ (lithopedion of Sens), which was removed from a dead woman in the town of Sens in France, was reported and became one of the foremost curiosities of France [22]; it has since become immortal among English-speaking people through its misuse in Tristram Shandy [63]. At Leinzell in 1720, a lithopaidion was observed during the autopsy of a 74-year-old woman who had carried it within her for 48 years, during which time she had several children [63]. Similarly, in 1721, Anna Mullern, 46 years before her death, declared herself pregnant; the swelling Santoro /Laganà /Sturlese /Giacobbe / Retto /Palmara
remained until she died at the age of 94. She desired that her body be examined after death; the surgeon broke open the mass with a hatchet and determined it to be a lithopaidion [21]. In 1881, Küchenmeister [54] reviewed 49 cases of lithopaidion published between 1582 and 1881 (table 2), which were quoted by Dean and Marnoch in 1893 [18]. Around 1880/1885, 46 cases were reported [24], although until 1900 there had been only 38 recorded cases, and in the next 12 years only 12 more cases were reported [21]. Masson and Simon [56] referred to 9 cases of lithopaidion at the Mayo Clinic from 1903 to 1926, and 9 more cases were seen there subsequently [21]. In 1924, Auvray [57] reported the first case of a lithopaidion in which a carcinoma developed [21]. In 1937, Cave [19] reported 196 cases up to 1933 and an insignificant number after 1933. In 1941, Reeves and Lipman [58] reported 236 cases, and an additional 15 cases were reported between 1947 and 1951 [23]. In 1952, Schwarz [24] referred to 196 cases recorded by 1937. In 1968, Chase [21] reported 270 cases. In 2000, Passini et al. [20] found fewer than 300 reported cases in 400 years of world medical literature; however, from 1968 to 1999 at least 47 new cases of lithopaidion were published. Moreover, it has been confirmed that in the last 10 years, as prenatal care and surveillance have increased, the presentation of lithopaidions is rare [28], even though at least 17 cases of lithopaidions were demonstrated in the last century [1, 20, 23, 25, 28–39, 64]; only in this case, we have not considered the 4 cases of lithopaidions reported by Sunday-Adeoye et al. [40] because it was not possible to determine the exact century of their occurrence. However, a complete analysis of the English literature has revealed more than 330 case of lithopaidion so far. See table 2 for a list of studies showing the number of lithopaidion cases reviewed. Based on a literature search in this field, the patient’s age at the time of diagnosis varied from 23 [61] or 30 years [25] to 100 years [25, 61], with a mean age of 55 years. The mean tolerated duration of lithopaidion was 22 years; 9 of 128 women selected by Tien [61] had carried them for more than 50 years, and more than 60% of women were over 40 years old, as recorded for women of the last century [20]. The period over which the fetus was retained in the abdominal cavity varied from 4 to 60 years [23, 25–27], and fetal death occurred between the third and sixth month of gestational age in 1/5 of cases, between the seventh and eighth month in approximately 1/4 of cases and at full term in approximately 2/5 of cases [20]. A lithopaidion can originate from a primary abdominal pregnancy, an aborted tubal pregnancy or an intrauterine Overview of Lithopaidion
Table 2. List of studies showing the number of lithopaidion cases
reviewed First author, year
Number of lithopaidion cases reviewed
Kuchenmeister, between 1582 and 1881, 49 cases 1881 were reviewed Schwarz, 1952 during 1880 – 1885 approximately, 46 cases were reported Chase, 1968 until 1900, only 38 cases had been reported, and in the next 12 years only 12 more case were added Masson, 1928; there were 9 cases of lithopaidion at Chase, 1968 the Mayo Clinic from 1903 to 1926, and later 9 more cases were seen there Cave, 1937 up to 1933, there were 196 cases Reeves, 1941 up to 1941, 236 cases were reported Odom, 2006 an additional 15 cases were reported between 1947 and 1951 Schwarz, 1952 196 cases were recorded by 1937 Chase, 1968 270 cases were reported Passini, 2000 fewer than 300 cases Currently more than 330 cases
Reference 54 24 21
56; 21
19 58 23 24 21 20
The first case of lithopaidion was discovered by Albucasis in approximately 880. The subsequent reviews show dissimilarities. A complete analysis of the English literature has revealed more than 330 cases of lithopaidion so far.
pregnancy followed by uterine rupture [1, 2]. Tien [61] collected 114 cases of lithopaidions, of which 74 were the result of the gestational sac rupture of a tubal pregnancy, 13 originated from an ovarian pregnancy, 8 were the result of a primary abdominal pregnancy, and in 5 patients the lithopaidion originated in the horn of a bicornuate uterus. Lithopaidion is generally a single obstetric phenomenon; the literature includes 1 report of twins with lithopaidion [34] and a case of lithopaidion in twins [41], 1 case of lithopaidion with a coexistent live abdominal ectopic pregnancy [35], a case of lithopaidion formation after a cervical pregnancy [42], a case of lithopaidion evolving from hepatic pregnancy [43], 1 case with a coexistent placental site trophoblastic tumor that was discovered upon the removal of the lithopaidion (after a colostomy for rectal perforation was performed) [33], and 2 cases with coexistent cervical neoplasm [1, 44] (table 3). Moreover, in this report, we document the first case of lithopaidion in a patient with an ectopic fallopian pregGynecol Obstet Invest 2014;78:213–223 DOI: 10.1159/000358828
217
Fig. 1. Ultrasound observation: right fallopian tube with an apparent gestational chamber (arrow).
Fig. 2. Ultrasound observation: left adnexa with corpus luteum (section A).
Table 3. Lithopaidion is generally a single obstetric phenomenon, but coexistent pathologies are sometimes discovered
diuretics and angiotensin II receptor antagonists. An examination revealed external genitals and a nulliparous vagina, a uterus with increased volume and a posterior well-closed cervix. The left adnexa were not palpable, and the posterior fornix was painful. Upon hospitalization, her body temperature, blood pressure and heart rate were normal. The hematic values were normal, and her β-HCG value was 4,141 mIU/ml. Mycoplasma urealyticum vaginal infection was discovered. The patient underwent a transvaginal ultrasound, which revealed an enlarged uterus showing a myoma. The right fallopian tube was enlarged with an apparent gestational chamber (fig. 1). The left adnexa had increased volume and showed a 37 × 36-mm corpus luteum (fig. 2). There was 20 mm of hematic fluid in the pouch of Douglas. After being briefed on the possible surgical procedures, she underwent a laparoscopy with general anesthesia. In hemodynamically stable patients, laparoscopic treatment of ectopic pregnancy offers major economic and health benefits compared with laparotomy. After the pneumoperitoneum was performed with 3.5 liters of preheated CO2, the principal intra-umbilical opening was prepared for the introduction of the laparoscopic optics. We performed two accessory openings: left and right. We observed hemoperitoneum, a uterus with increased volume and a right fallopian tube with increased volume. The fallopian tube was expanded because of an ectopic pregnancy in the ampulla. The right adnexa were regular, and the left adnexa had increased volume from a bleeding corpus luteum. Coagulation of bleeding from the left adnexa and ovarian
First author, year
Coexistent pathologies
Auvray, 1924
case of a lithopaidion in which a carcinoma developed Roberts, 1952 lithopaidion in twins Luwuliza-Kirunda, lithopaidion evolving from 1978 hepatic pregnancy Bustamante Sarabia, lithopaidion formation after a 1989 cervical pregnancy Huerta Bahena, 1994; coexistent cervical neoplasm Chang, 2001 El Hag, 2002 coexistent placental site trophoblastic tumor Mishra, 2007 twins with lithopaidion Massinde, 2009 coexistent live abdominal ectopic pregnancy
Reference 57 41 43 42 44; 1 33 34 35
nancy. After 6 weeks of amenorrhea, a 32-year-old woman with parity 0-0-1-0 was admitted to the hospital for pelvic pain and metrorrhagia. Her history included the following parameters: menarche occurred at the age of 14, and menses had been regular since. She had undergone a cystectomy of the right ovary by laparoscopy 13 years previously. At the age of 30, she had amenorrhea with positive β-HCG; her β-HCG values decreased after 10 days. She had high blood pressure and was treated with thiazide 218
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Santoro /Laganà /Sturlese /Giacobbe / Retto /Palmara
Color version available online
drilling were performed. A right salpingectomy was performed through electrical resection. In the pouch of Douglas, we found a new 1 × 2 × 2.5-cm formation (fig. 3) that was removed and sent for a histological examination. The postsurgical course was regular, and the day after surgery the woman was discharged normally. The notes from the histological examination referred to ovular tissue with extended coagulative necrosis and a confluent area of calcification, which was defined as a lithokelyphos. The principal features of this case are summarized in table 4. Approval by the ethics committee and informed consent from the patient were obtained.
Etiopathogenesis
Lithopaidion is a rare formation that evolves from an undiagnosed and untreated advanced abdominal pregnancy. Ectopic pregnancy is a public health problem that has increased in the last years [65]; it represents the major cause of maternal mortality, but its etiology [66] and pathogenesis are not clearly understood. Several risk factors have been identified such as pelvic inflammatory disease (especially for infections involving Chlamydia trachomatis), previous ectopic pregnancy, tubal damage from infection or surgery, a history of infertility treatment, treatment for in vitro fertilization, increased age, smoking, and past or current use of an intrauterine device [66, 67]. Abdominal pregnancies should be considered in all patients until an intrauterine location can be confirmed [68]. Until now, ectopic pregnancy and lithopaidion have been observed as single, separate obstetric phenomena. Our observed case documents an occurrence of lithopaidion in a patient with an ectopic fallopian pregnancy. Only 3 similar cases have been reported previously [13, 62]. Temple and Hester [62] reported a lithopaidion and a contralateral tubal pregnancy, while Venter and Campher [13] described a tubal pregnancy and a lithopaidion in the omentum; it has not been possible to read these reports for an appropriate evaluation of the data. The third case was referred to as a coexistent lithopaidion of advanced gestation with a live abdominal ectopic pregnancy at 15 weeks of gestation [35]. Ectopic pregnancy and lithopaidion imply a change in embryo transport. When they are observed in the same patient, we can hypothesize a common pathogenesis that is caused by altered fallopian contractions. The embryo is passively transported to the uterus by highly regulated and coordinated mechanical events in which the stochasOverview of Lithopaidion
Fig. 3. Macroscopic find of right fallopian ectopic pregnancy (im-
age A) and lithopaidion (image B).
Table 4. Principal features of a patient with lithopaidion and an ectopic fallopian pregnancy
Age of woman at time of diagnosis Retain time of lithopaidion Dimension of lithopaidion Time of fetal death Types of lithopaidion Place of lithopaidion Concomitant pathologies Symptoms Instrumental diagnosis Differential diagnosis Complications Type of intervention
32 years 2 years 1 × 2 × 2.5 cm 6 weeks lithokelyphos pouch of Douglas fallopian ectopic pregnancy, myoma pelvic pains, metrorrhagia during laparoscopy none none laparoscopy
tic contractile activity of the myosalpinx plays a dominant role [69]. Moreover, the embryonic transit is characterized by rapid back-and-forth movements, as the simultaneous and successive contractions and relaxations of the myosalpinx along the isthmus causes changes in the gradients of intraluminal pressure [70]. These movements are generated by several pacemakers that are constantly changing locations [71]. The interstitial cells of Cajal, Gynecol Obstet Invest 2014;78:213–223 DOI: 10.1159/000358828
219
Table 5. Percentage of frequent symptoms in women with litho-
Table 6. Percentage of symptomatic and asymptomatic women
paidion Symptoms
Percentage
Pain Heaviness Compression syndromes
39.4% 24.2% 36.4%
which populate the human oviduct, have recently been identified as specialized pacemaker cells [72]. The propagation of the myoelectric activity that is initiated by the pacemakers occurs in both directions over short stretches at a pace of 1–2 mm/s. The permanent relocation of the pacemakers along the oviduct causes the luminal content to move at random. The tonic contractions that occur at the ampullary-isthmic junction and the uterotubal junction are responsible for the transient arrest of transport that occurs at these points [73]. Alteration of the contractile function of the myosalpinx can determine the fallopian implantation or expulsion of the embryo. In the present paper, we report a lithopaidion and a right fallopian pregnancy with a bleeding corpus luteum in the left adnexa. This condition presumes an internal (from the left fallopian tube to the right fallopian tube through the uterus) or external (from the left fallopian tube to the right fallopian tube through the peritoneal cavity) expulsion of the fertilized egg. The report of a lithopaidion together with a tubal pregnancy highlights the fundamental importance of evaluating the clinical history of the patient to avoid further episodes and to target therapy and repeated controls. Even if the presence of a lithopaidion did not alter the surgical strategy for the ectopic pregnancy, the presence of anatomical and functional abnormalities of the female genitalia suggest the possibility that the ectopic pregnancy could be repeated, which would compromise the procreative capacity of the patient.
Clinical Features
Most lithopaidions are found incidentally [1]. No laboratory tests or typical symptoms are currently able to diagnose a lithopaidion [1]. The patient is often unaware of her condition. The usual history is of a false pregnancy followed by gradual decrease in the size of the abdomen and the return of menstruation. Frequent symptoms are abdominal/pelvic pain (even if its character is variable), abdominal/pelvic heaviness (that is not specific) and 220
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Patients
Percentage
Asymptomatic Symptomatic
90% 10%
compression syndromes particularly affecting the urinary tract and bowel (table 5). The differential diagnosis on the presentation of lithopaidion may include ovarian neoplasm [32], myomas, inflammatory masses, epiploon calcifications, and urinary tract or bladder tumors [45]. The majority of cases are asymptomatic (table 6) and are discovered incidentally in the course of clinical examination, on a straight abdominal radiograph for other pathologies [20, 21, 25, 35], or even postmortem [19, 22, 24]. This last detection method in particular led us to believe that there are more than 330 reported lithopaidions because many cases remain unrecognized. The X-ray differential diagnosis may include other masses that contain large amounts of calcium, including ovarian tumors, epiploic appendage and aortic aneurism. Computed tomography could better define the cause of the pelvic mass [26, 36, 46, 47]. Moreover, the lithopaidion is more common in situations linked to low socioeconomic status and lack of prenatal care [23, 25]. Particularly in the last millennium, 80% of patients with lithopaidion presented from areas lacking accurate diagnostic instruments. Lithopaidion can cause bowel obstruction in rare cases; in 1953, Glass and Abramson [48] reported the occurrence of volvulus of the cecum due to a lithopaidion, and Zaheer [49] (in 1971) and recently Allodé et al. [64] showed a small bowel obstruction due to the same cause. Mishra et al. [34] reported a case with features of intestinal obstruction. Jain and Eckert [30] reported a lithopaidion-like pelvic abscess, while Leke et al. [50] reported a case of obstructed labor in a term pregnancy caused by cephalopelvic disproportion as a result of a lithopaidion retained in a sacculus of the lower uterine segment. Odom et al. [23] managed an event of erosion into the bowel with fecal fistula formation caused by a lithopaidion (table 7). To form a lithopaidion, the following four conditions are required [2, 19, 59]: (1) survival of the fetus for over 3 months because if the death occurs while the bones are cartilaginous, absorption will be rapid and complete; Santoro /Laganà /Sturlese /Giacobbe / Retto /Palmara
Table 7. Lithopaidion is generally an asymptomatic phenomenon,
Table 8. The lithopaidion model: principal features to be consid-
but complications from the lithopaidion are sometimes discovered
ered
First author, year
Complications
Glass, 1953 Leke, 1983 Jain, 2000
volvulus of the cecum obstructed labor in a term pregnancy presentation of a lithopedion-like pelvic abscess Odom, 2006 erosion into the bowel with fecal fistula Mishra, 2007 features of intestinal obstruction Allodé, 2012 small bowel obstruction
Reference 48 50 30 23 34 64
Age of woman at time of diagnosis Retain time of lithopaidion Dimension of lithopaidion Time of fetal death Types of lithopaidion Place of lithopaidion Concomitant pathologies Symptoms: specific, aspecific Instrumental diagnosis Differential diagnosis Complications Type of intervention
(2) sterility of the fetus; (3) failure of medical detection; (4) presence of conditions favorable for the deposition of calcium. Cave [19], in 1937, supposed the presence of another requirement: the pregnancy had to be extrauterine. He affirmed that in the very early literature, approximately half a dozen cases were intrauterine, but given that no such cases have been reported within the last 100 years, it is reasonable to view the authenticity of these cases with extreme skepticism. Instead, analyzing the available literature has allowed us to find different papers that attest to a tight relationship between the uterus and the lithopaidion: in 1966, Binns [51] found a lithopaidion formation following the silent rupture of the uterus, similar to that found by Reiss [52] in 1996. In 2001, Lachman et al. [31] found a fetus that was hyperflexed with other signs of intrauterine growth. Moreover, recently, a calcified fetal skull was located in the uterine cavity of a 34-year-old patient, whereas the other body parts, including the spine and limbs, were situated in the abdominal cavity. A uterine incision surrounded by an old scar was observed in the anterior wall of the uterus. Therefore, it seems likely that rupture of the cesarean scar had caused the fetus to partly enter into the abdominal cavity [37]. D’Aunoy and King [60], as reviewed by Chase in 1968 [21], listed four processes that an abdominal fetus may undergo if it is not removed: (1) skeletonization: only the bones of the fetus remain following the disintegration and absorption of the soft parts; (2) formation of adipocere: the soft parts are replaced by fatty acids, soaps and salts of palmitic and stearic acids;
(3) suppuration: the fetus is destroyed after an abscess has formed, usually due to Escherichia coli infection; (4) true lithopaidion formation: this occurs if the fetus remains sterile and becomes infiltrated with calcium salts to varying degrees. From a biochemical point of view, a lithopaidion has a shell consisting mostly of inorganic constituents, and in the internal tissue there is significant depletion of K and Cl, significant enrichment of Mg, P and Na, and enormous enrichment of Ca (compatible with the calcification of the tissues). In the liver and muscle, there are discernible LDH isoenzymes, and in the brain and muscle discernible CK isoenzymes are present [53]. Considering that the surgical management of lithopaidion is usually simple with a very positive course even in the elderly, no intraoperative death due to lithopaidion has been reported recently [64], with the exception of 2 deaths that were not related to lithopaidion in which the patient died of pulmonary embolus postoperatively after a formal left above-knee amputation performed for dry gangrene [25] or the death was the result of renal failure [31]. Considering the possibility of future complications, the proper procedure is surgical removal. Although laparoscopy is used to explore the pelvic cavity and manage pelvic masses worldwide, the majority of discovered lithopaidions have been removed by classic laparotomic surgery, with the exception of a case of a young woman undergoing laparoscopy for infertility during which a lithopaidion was discovered [28] and our case of a 32-yearold woman who underwent a laparoscopy for an ectopic fallopian pregnancy.
Overview of Lithopaidion
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Conclusion
Lithopaidion is generally a single asymptomatic obstetric phenomenon that evolves from an undiagnosed and untreated advanced abdominal pregnancy and is discovered accidentally long after the pregnancy or even postmortem. With the inclusion of the latter, the total number of cases is higher than the approximately 330 cases reported in the literature, and its modern presentation,
particularly in countries with low socioeconomic status, is little more than 1 case per year because of increased prenatal care and surveillance. In the pathogenesis of lithopaidion, a role for the specialized pacemaker cells that populate the human oviduct has been hypothesized. Considering the fragmentation of the data presented in the literature, we suggest a model (lithopaidion model) unifying the evidence of lithopaidion (table 8). When it is diagnosed, the proper procedure is surgical removal.
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Gynecol Obstet Invest 2014;78:213–223 DOI: 10.1159/000358828
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Copyright: S. Karger AG, Basel 2014. Reproduced with the permission of S. Karger AG, Basel. Further reproduction or distribution (electronic or otherwise) is prohibited without permission from the copyright holder.
