Abstract
Between 1987 and 1997, two sets of female conjoined twins were documented in Seychelles. The diagnostic of conjoined twins was made at the occasion of routine ultrasonographic examinations. Both sets of twins were of the thoraco-omphalopagus type. Both sets of conjoined twins died after delivery by caesarean before term as they had multiple malformations not compatible with survival. The incidence of conjoined twins was approximately 1 per 7,800 deliveries, or 1 per 67.5 multiple births or 1 per 104 congenitally malformed babies. The literature on conjoined twins is reviewed briefly. (SMDJ, 1999;6:29-30.)
Case report No 1
A 29 year old woman, gravida 2, para 1, was admitted at 28 weeks of gestation with suspicion of conjoined twins based on ultrasonography. Her last childbirth was 4 years earlier, full term normal delivery (FTND). There was no significant past history. She had been previously on oral contraceptives stopped for some time. The pregnancy was unplanned but accepted and there was no history of attempt to terminate the pregnancy. She booked for the first time at 9 weeks of pregnancy. She was treated for threatened abortion at 12 weeks and for urinary tract infection at 22 weeks. An ultrasonography showed twin gestation of thoraco-omphalocele type. A therapeutic abortion was done for malformations not compatible with life. The joined twins were delivered as breech through a classical caesarean section. The patient refused a post-mortem to be performed. The postoperative period was uneventful. The patient became pregnant later and delivered a healthy normal child.
Case report No 2
A 25 year old women, gravida 2, para 1. Last childbirth - FTND male baby. She had regular menstrual periods before getting pregnant. Her grandmother had a history of twins. Her first ante natal booking was at 16 weeks and subsequently had 6 antenatal check-ups. Ultrasonography was done at 18, 23, 33 and 36 weeks respectively. Ultrasonography revealed thoracopagus conjoint twins, both breech presentation, female foetuses. There was a single heart and a single enlarged liver not compatible with life. Plain x-ray of abdomen showed both breech presentation with head at the same level, face to face. An elective and classical caesarean section was done at 36 weeks of gestation. Conjoined twins were delivered as breech, both females, weighing 4.94 kg together. Placenta was single. Both twins had an Apgar score of 2/1, 2/5, and 1/15. No active resuscitation was attempted. Cardiotocography confirmed the presence of a single fetal heart. Autopsy showed conjoined twins with multiple defects: twins weight 4.9 kg, crown-heel distance 45 cm, head circumference 33 cm each, fusion from thoracic cage at 3rd rib level till pelvis. All limbs were separate and free. There was a fibrous adhesion and omphalocele of single umbilical cord in the centre. There was a single large flabby heart placed centrally with several chambers and pairs of all other viscera. Liver and stomach were placed anteriorly and posteriorly in a mirror image fashion. The postoperative period of the mother was uneventful.
Discussion
The first recorded successful separation of two alive ‘Siamese’ twins dates back to 974 AD, as reported by Byzantine author Leon Diakonos. Around 150 years later, Johannes Skylitzer described surgical procedures for the separation of conjoined twins illustating the high standard of byzantine medicine of that time (1).
The minimal diagnostic criterion for conjoint twins is the fusion of some portion of the mononuclear or monozygotic twins. The fusion occurs as a teratogenic event due to incomplete duplication of the embryonic plate after the 12th day of ovulation (2). Monozygotic twins, who result from a single ovum, are often associated with congenital defects or teratogenic events. The incidence of monozygotic twins is constant world wide irrespective of race, parity and maternal age.
In an extensive review of the literature of conjoined twins, considerable difficulty was encountered in ways of interpreting the variation in nomenclature, identifying the individual twins and assessing their anatomical relationships to one another (3). It is therefore difficult to draw sweeping general conclusions about the blastogenesis of conjoined twins (4). They often present with two full notochord axes, a reasonably complete embryogenesis and severe disorientation, malformation or aplasia of the structures in the ventral zones arranged within one body.
Most conjoined twins are classified into main anatomical types or paradigms. An attempt to standardise and classify conjoined twins according to the external forms of conjunction has been proposed by Leacham (Table 1) (5) and took advantage of information derived from gross or electronic dissection of conjoined twins and the abundance of well documented such cases in the recent past.
Table. Leacham classification of conjoined twins
| Designation | Description |
| Thoracopagus | Joined at chest |
| Cephalo-thoracopagus | Joined at head and chest |
| Dicephalus | Single trunk and two heads |
| Craniopagus | Joined at head |
| Omphalopagus | Joined at abdomen |
| Rachipagus | Dorsal union of head and trunk |
| Thoraco-omphalopagus | Joined at chest and abdomen |
The worldwide incidence of conjoined twins varies between 1 per 30,000 to 1 per 100,000 deliveries (6). In Seychelles, as presented above, two sets of conjoined twins have been documented between 1987 - 1997 both of the thoraco-omphalopagus type (there are approximately 1600 births per year in Seychelles lately). The incidence of conjoined twins was therefore approximately 1 per 7,800 deliveries, or 1 per 67.5 multiple births (twins or more) and 1 per 104 congenitally malformed babies. The two sets of conjoined twins were delivered through caesarean section made before term as the two sets were considered not to be viable on the basis of the ultrasonography findings. Expectedly thus, both sets of conjoined twins died soon after birth.
In Uganda 16 sets of conjoined twins were seen between 1971 and 1980, an incidence of 1 per 4,242 deliveries, 1 per 69 multiple births and 1 per 139 congenitally malformed babies. All diagnoses were made during labor or at operation and all babies were lost (7). In Lusaka, Zambia, 13 sets of conjoined twins were born in a period of 24 years between 1970 – 1993 for a population of over 8 million. Out of these 13 cases, 10 underwent surgical separation, and 3 separations were successful (8).
Thoraco-omphalopagus is the most common variety of conjoined twins. About 90% of the conjoined thoraco-omphalopagus twins have a common pericardium and 75% have conjoined hearts; options for treatment are therefore largely dependent on the anatomy of the cardiovascular system (9). Thoracopagus twins occur frequently. The University Taipei hospital (Taiwan) has carried out surgical separation of 47 pairs of thoracopagus twins with 48% of the surgically separated twins surviving (10). In Korea, two conjoined twins were successfully separated, a pair of male thoraco-omphalopagus and a pair of female ischiopagus tetrapus, and both are alive and developing normally (11). Twenty cases of rachipagus were seen among a total of 1200 conjoined twins in the world data. Rarer presentations include sternopagus twins which present with a single common sternum, malformed heart, and common bowel (12) and heteropagus twins which present with the fusion of two embryos and the resorption of the cranial half of one of them (13). The presence of a Yolk sac tumor has been described in 5-year-old separated conjoined twins (14).
Meticulous scanning by experienced operators using transvaginal ultrasonography can detect conjoined twins as soon as from the first trimester. Early detection of thoraco-omphalopagus has been made at 9 weeks of gestation with the aid of transvaginal ultrasonography (12). Two-dimensional echocardiography, color Doppler examination and 3D-colour sonography using 5-7 MHz transabdominal and transvaginal transducers are the methods of choice to investigate anatomical malformations of conjoined twins with a high degree of precision and confidence (9). The current medical management to ensure survival of one or both conjoined twins is based on the nature of abnormalities, including the presence of a single heart, liver, spine, urinary bladder, and various cloacal and umbilical cord deformities or the presence of single yolk sac.
References
Abstract
We report the case of a young Indian worker with the history of two episodes of transient hemiparesis and a rounded lesion with prominent ring enhancement on the computed tomographic scan of the brain. Epidemiological, clinical, radiological, and serological results favoured the diagnosis of neurocysticercosis. The differential diagnosis, with special emphasis on tuberculoma, the pathophysiology, the clinical presentation, the treatment and the epidemiology of cysticercosis are reviewed. As Seychelles is free of taeniasis and cysticercosis, this case reminds us that cysticercosis is part of the differential diagnosis of neurological deficits or epilepsy in patients coming from an area which is endemic for cysticercosis. (SMDJ, 1999;6:31-33.)
Case report
A 28-year old right-handed man was admitted to the hospital because of a sudden episode of right hemiparesis. The patient, an Indian citizen working as labourer, came to Seychelles two months ago. He used to eat pork. He had been well until seven months earlier, when he first experienced an episode of weakness in the right side of the body that resolved spontaneously after about 30 minutes. Later he noticed several unusual episodes of headache.
The day of admission, he felt a sudden weakness of the right side of the body with severe headache. He was unable to stand but never lost consciousness. These symptoms lasted about one hour. The patient was brought to this hospital.
The temperature was 37.5o C, the pulse 93/min, and respirations 24/min. The blood pressure was 140/80 mm Hg. The physical examination of the head, neck, lungs, heart, abdomen, arms and legs, and genitalia was normal. On neurological examination, the patient was alert, oriented, and able to follow commands. The cranial-nerve functions were preserved; in particular, no nystagmus was evident. Muscle bulk, tone, and strength were normal. Sensation was intact. Tests of co-ordination were well performed. The deep-tendon reflexes were present and equal; the plantar responses were flexor.
The results of laboratory tests were normal except for the differential count of the 8’900 white blood cells/m l (granulocytes: 86%; lymphocytes: 12%); the erythrocyte sedimentation rate was 7 mm per hour; a random blood sugar was 5.5 mmol/l. A computed tomographic (CT) brain scan performed without and with administration of intra-venous contrast revealed a rounded lesion with prominent ring enhancement in the left parieto-occipital subcortical region measuring 10 mm in maximum diameter and showing mild degree of perifocal vasogenic oedema (Figure 1). The wall of the lesion was isodense on plain scans and the contents were slightly hyperdense to cerebrospinal fluid. There was no significant mass effect or midline shift. No evidence of haemorrhage was detected (Figure). A lumbar puncture was performed: the cerebrospinal fluid was clear, colourless, sterile, with no cells, and normal protein and glucose levels. An enzyme-linked immunoelectrotransfer blot assay (EITB) for cysticercosis was positive (O.D.= 0.430; c/o = 0.250) (result received after the patient’s discharge).
Treatment with oral albendazole (15 mg/kg/day for 15 days), intravenous dexamethasone (4 mg tds for 4 days), oral phenytoin (300 mg nocte for 15 days) and paracetamol on request was started. On the fifth day of admission, the patient had no complaints and his physical examination was normal. He was discharged from hospital and left Seychelles some days later.
Figure 1. Cranial CT scan without (left) and with (right) contrast medium, showing a rounded lesion with prominent ring enhancement in the left parieto-occipital subcortical region.
Discussion
This 28-year old man had recently arrived in Seychelles from India, presented with transient hemiparesis and a single ring enhancing lesion on imaging studies of the brain.
This lesion could be a bacterial brain abscess; brain abscesses are usually secondary to infected bronchiectasis, sinusitis and otitis media, or general infection and befall days to several months after the primary infection; this patient had no disease suggestive of bacterial infection during these last months, making this diagnosis unlikely. In India, a large proportion of space-occupying central nervous system lesions are caused by tuberculosis. Tuberculomas may be most common accounting for 20 to 30 percent of intracranial masses (1). On imaging studies before the administration of contrast material, oedema and necrosis may appear to be iso- or hypodense during the initial phase of a tuberculoma. Two different images are noted after the administration of contrast material: a ringlike lesion with a central area of hypodensity and a nodular or plaquelike enhancement. The enhancement may be homogeneous, or a central radiolucent area may correspond to the central zone of necrosis. Within this area of lucency, a central speck of calcification is considered suggestive of tuberculoma; however, frank calcifications are present in less than 10 percent of cases (2). A low-grade pleocytosis and an elevated cerebrospinal fluid protein level are characteristic of tuberculoma (3). In our patient, although the imaging studies cannot entirely rule out a tuberculoma, the normal erythrocyte sedimentation rate, the normal chest film and the normal cerebrospinal fluid findings make this diagnosis unlikely.
Echinococcus granulosus, the dog tapeworm, is a major cause of hepatic and cerebral infection worldwide. Hydatid cysts can be mistaken for cysticercosis; however, a hydatid cyst in unlikely in our patient because of the lesion’s appearance. Paragonimiasis (caused by Trematoda, a parasite of man, dog, cat, pig, wild carnivore, and snail transmitted by fresh water crab and crayfish) is a frequent cause of solitary brain masses in the Far East; in India, the respiratory symptoms are predominant (from the Global Infectious Diseases and Epidemiology Network); therefore the absence of pulmonary infestation makes this diagnosis unlikely. Amoebiasis can be complicated by suppurative brain abscesses in a small percentage of patients, but they usually have liver abscesses and are acutely ill. Sarcoidosis may present preferentially as a homogeneously enhancing mass and we would expect evidence of extracerebral disease, such as lung involvement or hilar lymphadenopathy. A malignant neoplasm of the brain would be very improbable because of the long duration between the two episodes of transient neurological deficit and the evolution without worsening of the symptoms. A meningioma is also improbable in view of the findings on imaging studies.
The cysticercus is a fluid-filled bladder that contains the invaginated scolex of the larval form of Taenia solium, the pork tapeworm. While infections of humans that lead to intestinal tapeworms occur by ingestion of undercooked pork containing cysticerci, ingestion of food and water contaminated by T. solium ova is the most common route by which humans, serving as intermediate hosts, contract systemic cysticercosis. Ingested Taenia ova develop into embryos, penetrate the small bowel and are deposited throughout the body, with a predilection for the central nervous system, muscles, and eyes. Viable encysted larva causes little or no inflammation, but when the larva dies, the disease becomes active: the cyst swells, generates an inflammatory response and develops into a granulomatous abscess undergoing progressive mineralization (4). Cerebral cysticerci are located in grey matter or at the junction of grey and white matter and are solitary in over 25% of cases (5).The racemose cysticercosis is a more aggressive form of the disease: the larvae lie in the subarachnoid space at the base of the brain, where the cysts form grapelike clusters, lack scolices, and are not calcified.
Clinically neurocysticercosis is difficult to diagnose: the central nervous manifestations are diverse and depend on the magnitude, site, and stage of infestation (6). When inflammation surrounds lesions, intermittent, transient protean neurological deficits, and mostly partial, focal, or generalised seizures occur (4,7). Signs of increased intracranial pressure, headache, nausea, vomiting, dizziness, changes in vision, ataxia, and confusion often are present. But the process can be indolent in many cases and discovered incidentally by radiographs or CT scans.
In cerebral parenchymal forms, the typical CT appearance is of a rounded cerebrospinal fluid density lesion with a dense scolex, which shows no enhancement or perifocal oedema when the larva is viable. Once the larva dies, the lesion demonstrates ring enhancement and adjacent parenchymal reaction. Calcification is seen in later stages. Magnetic resonance is superior to CT for detecting subarachnoid and intraventricular cysts, vasogenic oedema after larval death, and for demonstrating contrast enhancement due to the breakdown of the blood-brain barrier (8). More than half of the patients with the intraparenchymal form of the disease have a normal cerebrospinal fluid analysis; the racemose form is more often associated with pleocytosis, elevated protein level, and occasionally low glucose level. The EITB assay is the serological test of choice for confirming a presumptive case of neurocysticercosis. It is 100% specific and around 90% sensitive when there are more than two cysts (9,10).
All cases of parenchymal active neurocysticercosis benefit from treatment, even those with solitary cyst; the treated cysts do not form granulomas in 80% of cases, whereas non-treated cysts form granulomas in 80% of cases and residual granulomas are an important cause of persisting epilepsy (8). Praziquantel (50 mg/kg/day for 15 days) and albendazole (15 mg/kg/day for 15 days) are both effective against neurocysticercosis; most studies favour albendazole because it is the drug of choice for subarachnoid neurocysticercosis, the higher concentrations in the cerebrospinal fluid, the low cost, the absence of interference with phenytoin and carbamazepine, and the increase in plasma level when used concomitantly with steroids (8,9). Steroids are recommended in cases of cysticercotic encephalitis and subarachnoidal cysts to control intracranial hypertension or other intense side effects due to brain inflammation after cyst death (8).
Cysticercosis is widespread in developing countries especially in rural areas. In Brasilia, the prevalence of neurocysticercosis was 0.12-9% in autopsies and the frequency was 0.03-7.5% in clinical series (11). In two rural Guatemalan communities, the seroprevalence of antibodies detected in humans to Taenia solium was 10% and 17% and the prevalence of intestinal taeniasis was 1% and 2.8% respectively (12). In the Indian Ocean region, a very high seroprevalence for cysticercosis of 18% was reported in healthy individuals of Madagascar in 1994 and the serologic markers among 1,132 patients with neurological symptoms was 36% (13). The Seychelles islands can be considered as free of taeniasis and cysticercosis as no cases were reported during the last eight years by the Public Health (involved in meat control) and Hospital Laboratory Services, the Paediatric and Medical Departments of Victoria Hospital, and the Veterinary Services; the Intestinal Parasites Control Program initiated in 1993 did not include taeniasis in the list of parasites considered as a public health problem (14).
The patient of this report is the only case of neurocysticercosis diagnosed in Seychelles during these last ten years at least and he had contracted the disease in India. However, this case report emphasises the necessity to include cysticercosis in the differential diagnosis of neurological deficits or epilepsy if the patient is coming from a country which is endemic for cysticercosis.
References
Abstract
Dermoid cysts of the ovary or teratomas of the ovary are tumors that arise from the germ cells. These tumors constitute about 15-20% of all ovarian tumors and over 98% of these teratomas are benign mature cystic teratomas. About 1% of dermoid cysts undergoes malignant transformation of any one of the component elements, the most common being the squamous element, forming squamous cell carcinoma. We report the first case in the Seychelles of an invasive squamous cell carcinoma arising from malignant transformation in a mature cystic teratoma of an ovary. (SMDJ, 1999;6:33-34.)
Case report
A 56 year old female patient from La Digue island presented in November and December 1997 at the local hospital with low backache and constipation. Clinical examination revealed swelling in the umbilical region and a provisional diagnosis of fibroids was made. She was seen once again in January ‘98 for similar complaints and with tenderness in both hips and was treated symptomatically. In January 1998, the patient consulted a private practitioner who referred her to the Consultant Surgeon in Victoria Hospital with a suspicion of colonic carcinoma after rectal examination. Clinical examination at the hospital revealed a pelvic and an umbilical mass with an enlarged uterus.
A barium enema showed a persistent narrowing and irregularity of the sigmoid and descending colon with irregular mucosal pattern. Ultrasonography of the abdomen showed a left ovarian mass suspect to be a dermoid cyst. CT scan of the pelvis demonstrated the classical sign of a dermoid cyst with calcified nodule, large mass lower down, multiple fibroids arising from the uterus. An exploratory laparotomy revealed a pelvic malignancy arising probably from a left ovarian cystic tumor with involvement of the ileum causing obstruction, and multiple peritoneal nodules. No fibroids of uterus were seen. Per operative microscopic examination of a peritoneal nodule was suggestive of a squamous cell carcinoma. An ovarian cystectomy and a jejuno-ileal anastomosis were performed to bypass multiple bowel obstructions.
The pathological examination of the removed tissues showed macroscopically a left ovarian cyst without solid areas, measuring 11 x 11 x 6 cm filled with yellowish greasy material with areas of cartilage and hair. Microscopically, sections from the left ovarian cyst showed cystic space lined by stratified squamous epithelium, lumen filled with eosinophilic keratinous material, and anucleate squames. Malignant changes of the squamous epithelium were seen with individual tumor cells showing eosinophilic cytoplasm, hyperchromatic to vesicular nuclei, prominent nucleoli, scattered mitosis and keratin pearls. The tumor was invading the ovarian stroma.
The final histological diagnosis was infiltrating, well differentiated squamous cell carcinoma arising from malignant transformation of squamous component of mature cystic teratoma of the ovary.
As this patient was considered to be in a terminal stage of the disease, no further specific treatment was given and the patient died five weeks after the laparatomy.
Discussion
This is an unusual presentation of a dermoid cyst turning malignant, the first case documented in Seychelles.
Teratomas or dermoid cysts of the ovary arise from the germ cells and are composed of all the three derivatives: the ectoderm, endoderm and mesoderm. They can be classified as: (a) immature (malignant); (b) mature-cystic and solid teratomas which are benign with potential for malignant change. These tumors are unilateral in 88% of the cases and provoke only symptoms relating to mass. They are most common in childhood and in premenopausal women. About 1% of dermoids undergo malignant transformation of any one of the component elements and result in thyroid carcinoma, melanoma, adenosquamous and most commonly squamous cell carcinoma.
A review of the literature (1) reveals that primary squamous cell carcinoma of the ovary is rare so that most cases represent malignant transformation of the squamous component in dermoid cysts (2). Other cases of ovarian cancer have been associated with ovarian endometriosis, preexisting Brenner tumor (1) and pure primary squamous cell carcinoma (SCC). Reports of concurrent cervical squamous cell carcinoma in situ, recurrent cervical intraepithelial neoplasia (CIN) and recurrent vulvar intraepithelial neoplasia (VIN) along with primary SCC of ovary have been documented (1).
Human papilloma virus (HPV) DNA (HPV type 16/18) has been identified in an in situ and invasive SCC in the ovary raising the possibility of a causal relationship of HPV with primary ovarian squamous carcinoma (2). Studies have shown that the overall survival for these tumors correlated best with the stage and grade of the tumor. Patients with SCC associated with dermoid cyst had a better survival than patients with SCC associated with endometriosis (2).
The etiology of ovarian cancer is poorly understood (3). Factors associated with an increased risk for invasive epithelial ovarian cancer include age, race, nulliparity, family history of ovarian cancer and history of endometrial or breast cancer. Factors associated with reduced risk are history of one or more full-term pregnancies (3,4), use of oral contraceptives, history of breast feeding, tubal ligation and hysterectomy. Other factors such as infertility drugs (3,5), hormone replacement therapy, age at menarche, age at menopause, dietary factors (3,4), lactose intolerance, talc use, coffee and alcohol consumption have been suggested, but their role is still inconclusive.
In Seychelles, the number of ovarian cancers is significant with the following incidence over the past five years: 1992: 2 cases, 1993: 3 cases, 1994: 2 cases, 1995: 1 case. There has been an increase in the number of ovarian cancers in the last two years with five cases in 1996 and 1997 each and 3 cases during the first six months of 1998 (whether this is a true increase in incidence or due to a detection bias is unclear). Most of these patients were perimenopausal and postmenopausal women.
The majority of these malignant ovarian tumors were mucinous or serous cystadenocarcinomas with few having secondary seeding at the time of diagnosis, other tumors like germ cell tumors, malignant teratomas have also been documented. These cases were followed up with regular screening for tumor markers like CA-125, CEA, CA19-9 and AFP.
The preoperative and postoperative use of tumor markers (CA-125, CA19-9, CEA with CA 72-4 and TPA in borderline ovarian tumors).is useful to determine the prognosis of these patients (6-8).
Ovarian cancer is the second most common cancer of the female reproductive system (3) and the main cause of death from gynecologic malignancies. Between 1986-1990, the overall age-adjusted incidence of ovarian cancer was 14.3/100,000 women and its mortality was 7.8/100,000 women. Ovarian cancer is rare before the age of 40, increases steeply thereafter, and peaks at ages 65-75. Over the last three decades, ovarian cancer incidence has remained stable in high-risk countries, while an increasing trend has been reported in low-risk countries. Incidence and mortality rates are higher among white women than among African-American women.
Despite recent advancement in treatment, the overall five-year survival rate is still low (39%) (3). Over 70% of ovarian cancers are diagnosed when regional or distant involvement has already occurred, causing survival rates to remain stable.
In conclusion, ovarian cancer offers a difficult challenge for early diagnosis and requests from practitioners a high awareness of the disease.
References
Abstract
A 67-year old female with hoarseness of voice of two months duration was diagnosed as laryngeal tuberculosis after clinical, radiological and histopathological investigations. Findings were granulations on both vocal cords, a raised ESR and a fibro-cavitory lesion in the left pulmonary apex on chest radiograph. Clinical presentation, differential diagnosis, pathology, and treatment are reviewed. This patient reminds us that laryngeal tuberculosis, although a rare condition, is part of the differential diagnosis of voice hoarseness. (SMDJ, 1999;6:35-36.)
Introduction
Laryngeal tuberculosis (LTB) is actually a rare presentation of respiratory tuberculosis. The incidence of LTB has universally dropped dramatically from 25% to less than 2.5% in patients with pulmonary tuberculosis (PTB) (1). In Seychelles, 199 cases of tuberculous infection (of any localisation) were reported during the period 1983-1996 (2). We present the first case of LTB documented in Seychelles.
Case report
A 67-year old female was referred because of hoarseness of voice for the past two months. She lived on Praslin Island with her grand daughter. The patient had never left Praslin Island and had never worked outside her home. There was no history of contact with tuberculosis and she was never diagnosed or treated for tuberculosis. Twenty years ago, she was treated for leprosy with a full course of dapsone. There was no recent history of cough, chest pain, haemoptysis, fever or loss of weight.
On examination she was looking healthy with a well-built constitution. The temperature was 37.0 degree Celsius, pulse 82/minute, respiratory rate 18/minute, blood pressure 130/90 mmHg. The throat was normal. Laryngeal examination revealed granulations over both vocal cords and there was decreased mobility of the left vocal cord. Epiglottis and pyriform sinuses were normal. Examination of the neck was normal. Auscultation of the lungs revealed coarse rales over the right mid and left apical zones. Laboratory tests were normal except for an elevated ESR (114 mm/hour). Three sputum specimens examined by smear and culture were negative for tuberculous bacilli. Chest radiograph showed a fibro-cavitory lesion in the left apical zone and infiltration in the right mid zone (Figure 1).
The histopathological examination of the granulations removed by micro-laryngeal excision revealed a tuberculous granulomatous inflammatory process. A diagnosis of LTB was made. The patient was treated with streptomycin, isoniazide, rifampicin and pyrazinamide for six months. After treatment, the hoarseness had disappeared and the voice was normal. On examination of the larynx, the granulations over the vocal cords had disappeared and both vocal cords looked normal. The mobility of the left vocal cord had returned to normal. The ESR was normalised.
Figure 1. Chest X-ray showing fibro-cavitory changes in the left upper zone and focal infiltration in the right mid zone
Discussion
LTB is one of the extra pulmonary manifestations of tuberculosis. It was more frequent in the pre-streptomycin era. But cases do occur in countries where PTB is highly prevalent. In developed countries, LTPB is found more frequently in immuno-compromised patients.
LTB appears to be transmitted by bronchogenic route in 85% of the patients and appears to be primary LTB in 15%. It is more common in PTB with positive sputum for tuberculous bacilli. The mean age of involvement is 48 years and the male/female ratio is 2/1. Hoarseness of the voice (85%) is the most frequent presenting symptom followed by painful dysphagia (15%) (3). Rarely it can present as a case of epiglottitis (4). Other symptoms include weakness of the voice, cough and referred otalgia. The common laryngeal sites of involvement are, in the order of frequency, true vocal cords, epiglottis, false vocal cords, arytenoids and posterior commissure. Sub-glottis is very rarely involved (5).
In the common sputogenic type of LTB, the tuberculous bacillus infects the intact laryngeal mucosa, especially, in the posterior third of the larynx. The sub-mucosal layer becomes infected and small round cell infiltration occurs. Before infiltration is marked there may be considerable myositis. One or more surface nodules appear which caseate and leads to ulceration. Progression leads to masses of granulation tissue that are usually asymmetrical (Fig 2).
The investigations recommended are: full blood count and ESR, chest radiograph, computerized tomographic scan (CT) of the larynx, smear and culture of sputum for tuberculous bacilli, micro culture and histological examination of the excised tissues.
The most important differential diagnosis is neoplasic growth, especially squamous cell carcinoma (6). Rarely both conditions coexist. Both conditions can present in a similar manner with hoarseness of voice but painful dysphagia is more typical for LTB. Past or actual PTB favors the diagnosis of LTB. CT scan of the larynx pre-operatively can help to differentiate tuberculosis from malignant condition. Characteristic CT scan findings of LTB include bilateral involvement, thickening of the free margin of epiglottis and good preservation of the pre-epiglottic and para-laryngeal fat spaces even in the extensive mucosal involvement. By comparison, laryngeal carcinoma generally presents with unilateral involvement, infiltration of pre-epiglottic and para-laryngeal fat spaces, cartilage destruction and extra laryngeal invasion (7). The conclusive diagnostic procedure for LTB is the isolation of the tuberculous bacillus in the excised tissues.
Treatment for PTB is generally also effective for treating extrapulmonary tuberculosis. Tracheostomy is indicated when the patient develops stridor. Laryngoplasty may be needed in the rare cases where extensive fibrosis leads to distortion of the larynx.
This first documented case of LTB in Seychelles should remind us that tuberculous infection is part of the etiologic differential diagnosis of voice hoarseness.
References
Introduction
Isolated fallopian tube torsion is a very rare condition that mostly occurs during the reproductive years, but has also been reported in premenarcheal and postmenopausal women (1). Diagnosis of this condition is often delayed because of the rarity of its occurrence and prolonged investigations to rule out more common causes of acute abdominal pain (1). Predisposing factors for torsion are hydrosalpinx (2), hematosalpinx (3), adhesions, prior tubal operation, sterilization (4,5), pelvic congestion, ovarian and paraovarian masses (6) and trauma but also may occur in the absence of adnexal disease (7) and during pregnancy (8,9). The leading clinical symptom is abdominal pain in lower quadrants. Signs, symptoms, or ultrasound findings are consistent with acute pelvic inflammatory disease (9). Laparoscopy can establish the definitive diagnosis of tubal torsion (4). The established diagnosis is usually late and salpingectomy is almost always necessary (10). Ultrasound diagnostics followed by early laparoscopy are compulsory procedures to salvage the tube (11,12). A case of a 13-year-old girl with isolated left fallopian tube torsion is hereby reported.
Case report
A 13 years old virgin premenarcheal girl was reported to surgery for a colicky left iliac fossa pain irradiating to the left flank. Generally healthy and active in ballet she had had a sudden onset of pain in the left iliac fossa which went on and off in attacks.
The patient did not have nausea and vomiting. Stools and urine were normal and regular. A year ago the patient had appendectomy. On examination the patient did not have aspect of an intoxicating illness. The blood pressure and the pulse rate were normal. The abdomen was soft and flat, slightly tender in the left iliac fossa with left lumbal sensitivity on percussion. Bowel sounds were present. Proctorectal examination revealed normal stools in the rectal ampulla. External genitalia inspection confirmed an intact hymen with slight vaginal discharge. She had no fever and the blood and urine tests were within the normal range. A routine ultrasound did not reveal any pathology of the left iliac fossa: the kidney did not show sings of kidney stone or caliceal and pyelic dilatation and the left ureter was free of obstruction. Small cyst on the left ovary was considered to be the first De Graaf follicle which was leading to menarcheal symptoms. She was given spasmolitics and put on bed rest at home. After a transient improvement the pain worsened next day and the patient vomited.
She was admitted to hospital and put on observation with intravenous fluids and spasmolitics. Apart from a vaginal whitish discharge, no other new findings were detected. The repeated ultrasound examination reported normal findings. During the day the pain became rather permanent, radiating to the suprapubic area and during the night the patient got fever. Lower abdomen showed signs of peritonism, bowel sounds disappeared and X ray abdomen showed free fluid levels in the pelvic bowel loops. The ultrasound assessment proved some peritoneal fluid collection in the Pouchot Douglas’ recess and a new, round shaped echo, in the retrouterine position.
The diagnosis of ovarian torsion was made during surgery. We found normal ovaries on both sides and isolated torsion of the left Fallopian tube. The tube was three times twisted and unfortunately gangrenous. After being untwisted the tube showed extra wide ligamenta and unusual mobility. The opposite right side tube was of a normal aspect, length and position. It was too late to salvage the tube and surgery ended up with excision.
Recovery was quick and uneventful. The tube itself did not show any histological anomalies. Four weeks after the surgery the patient got her first menstrual discharge.
Discussion
Isolated Fallopian tube torsion is a rare clinical pathology. Only 17 cases of isolated Fallopian tube torsion have been internationally reported in the last five years. The following conditions have been related to this pathology: previous tubal ligation in 5 cases (4,6), pregnancy in 3 cases (8,9), unspecified in 3 cases and in 6 premenarcheal virgin girls two had anatomical duplication of the tubes (12). Only one in this group of six was successfully treated with salvaged Fallopian tube (10). The others developed gangrenous changes and underwent salpingectomy. One can also presume that the condition might be reversible. Spontaneous torsion might be followed in certain cases by spontaneous detorsion. Under the same consideration the condition may be habitual with recurrent pain especially in premenstrual periods when congested and heavy tubes with some anatomical predisposition and mobility go to semirotation.
Our 13 year patient had menarcha only four weeks after the event of torsion. Her anatomical specific finding was the left elongated ligamentum latum, which allowed the torquation of the tube. We consider that three different etiological factors played the role in the torsion of the tube: 1) anatomical predisposition with mobile tube on a wide ligamentum latum 2) immediate premenstrual congestion of the tube, and 3) patients’ physical hyperactivity in playing ballet. During surgery we did not find any relation to previous appendectomy as the abdomen and pelvis were free of adhesions. The opposite Fallopian tube appeared normal in size, shape and position. Torsion of the Fallopian tube is commonly encountered in the diseased tube. Hematosalpinx with torsion in a virgin girl who experienced no prior predisposing factors suggested for tubal obstruction and adhesions, was already reported (3). Due to gangrenous changes no histology abnormalities were possible to detect in our patient. As in the majority of cases the intervention took place when the tube became gangrenous, clinical picture clear and diagnostic procedures undoubtful. This delay seems to be common due to the variety of differential diagnosis problems. Presented as a colicky pain or inflammatory pelvic disease the tubal torsion may be misjudged and delay the decision. A wide range of diagnostic procedures for this condition from ultrasound (11,12), Doppler (5,13), CT scan (15) or MRI (16) have been reported. It only proves that looking for a surgical justification is time consuming and once it is provided the tube is already gangrenous.
Conclusion
The ultrasound examination seems to be a sufficient standard with eventual color Doppler facilities for evaluation of the vitality of the tube. Laparoscopic diagnostic and/or an early surgical intervention is the golden standard to detect the condition at time and selvage the tube. The importance of a gangrenous tube excision is not the same in young girls in the pre-reproductive period and in-patients with previous tubal ligation. For that reason we think that the differential diagnosis of a low abdominal or pelvic pain in young girls has to be given high consideration.
References