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A 21-year-old nulligravid woman presents to the office reporting three months of dull and aching right lower quadrant pain. She rates the pain a 4 out of 10. She denies any associated nausea, vomiting, or changes in bowel or bladder function. She is sexually active with one male partner and uses condoms for contraception. She has no significant past medical or surgical history. She is not taking medications and she has no known drug allergies.
The gynecology service is consulted on a 16-year-old gravida 0 admitted to the inpatient psychiatric service to rule out physiologic concerns of acute psychosis. On day 2 of her admission, she had a witnessed grand mal seizure. EEG was performed and demonstrated slow delta waves in the frontal temporal area. An MRI showed no abnormalities and a lumbar puncture revealed lymphocytic pleocytosis and was positive for N-methyl-D-aspartate receptor (NMDAR) antibodies. Her last menstrual period was two weeks ago. She has never been sexually active. She has no past medical or surgical history. Her parents deny concern for alcohol, drug use or ingestion, or tobacco use. She is not taking any medications and has no known drug allergies.
Advanced imaging of the mid-gestation fetus has allowed for the early detection and monitoring of conditions that may impact the delivery of the baby. Large head and neck masses that will likely compromise ventilation at birth can therefore be easily diagnosed with plans for securing the airway at birth. Ex-utero intrapartum therapy has greatly enhanced survival for such affected infants at birth. The success of this approach in providing advanced airway management at delivery has also been extended to the treatment of other conditions that may be life-threatening within the first few minutes to hours of life.
Ex utero intrapartum treatment (‘EXIT’ procedure) is a well described method for maintaining maternal–fetal circulation in the setting of airway obstruction from compressive neck masses. When ex utero intrapartum treatment to airway is not feasible, ex utero intrapartum treatment to extracorporeal membrane oxygenation (‘ECMO’) has been described in fetal cardiopulmonary abnormalities.
Objective
This paper presents the case of a massively compressive midline neck teratoma managed with ex utero intrapartum treatment to extracorporeal membrane oxygenation, allowing for neonatal survival, with controlled airway management and subsequent resection.
Case report
A 34-year-old-female presented with a fetal magnetic resonance imaging scan demonstrating a 15 cm compressive midline neck teratoma. Concern for failure of ex utero intrapartum treatment to airway was high. The addition of the ex utero intrapartum treatment to extracorporeal membrane oxygenation procedure provided time for the planned subsequent resection of the mass and tracheostomy.
Conclusion
Ex utero intrapartum treatment procedures allow for securement of the difficult neonatal airway, while maintaining a supply of oxygenated blood to the newborn. Ex utero intrapartum treatment circulation lasts on average less than 30 minutes. The arrival of extracorporeal membrane oxygenation has enabled the survival of neonates with disease processes previously incompatible with life.
This chapter surveys the entire spectrum of tumours that are described in the fetus, infant and child. The commoner tumours (rhabdomyoma, fibroma, teratoma, myxoma) are discussed in detail, but there is also extensive discussion of rarer tumours such as fatty tumours and vascular tumours and even rarer entities such as juvenile xanthogranuloma or inflammatory myofibroblastic tumour. A brief section is devoted to primary malignant tumours of the heart and to metastatic tumours. Finally, there is discussion of pseudoneoplasms that may be seen in children.
Intracardiac teratomas are rare primary tumours. We report the case of an infant prenatally diagnosed with an isolated multi-cystic mass developed in the right ventricle causing neonatal refractory ventricular arrhythmia. Despite rescue extracorporeal support and partial surgical resection, he died as almost all the previous reported perinatal intracardiac teratomas whatever the prenatal tolerance and the size of the tumour. The common poor outcome of fetal intracardiac teratomas should be known when counselling parents during pregnancy.
An intrapericardial vacuolated mass compressing and displacing the heart was diagnosed by echocardiography in a foetus of 22 weeks gestation. The birth was induced for early signs of foetal distress at 29 weeks and, after two initial pericardial evacuation procedures, the tumour was resected radically 7 days after birth at a weight of 1.55 kg. Mass histology showed teratoma associated with yolk sac tumour. We comment on the overall approach adopted after foetal diagnosis and the histopathological features of the tumour, and try to draw conclusions on patient outcome data.
We describe two cases of confirmed anti-NMDA receptor encephalitis; one patient initially presented with a clinical picture that resembled delirium and later appeared to present with a conversion reaction and the second patient presented with a first psychotic break followed by the clinical picture of neuroleptic malignant syndrome with catatonia. Neither patient had a previous history of psychiatric illness or recreational drug use. These cases illustrate the diagnostic and treatment challenges associated with this neuropsychiatric condition and underscore the role of psychosomatic medicine psychiatrists in diagnosing anti-NMDA receptor encephalitis.
To review the incidence, treatment and prognosis of testicular carcinoma metastatic to the neck, and to propose a selective neck dissection be performed for residual disease post-chemotherapy.
Case report:
A 17-year-old young man with metastatic testicular carcinoma presented with a left neck mass. A palpable neck mass is the initial sign in approximately 5 per cent of cases of metastatic testicular teratoma. Approximately 30 per cent of patients with advanced-stage testicular cancer have extra-retroperitoneal disease post-chemotherapy, which requires resection. The presented patient underwent a left selective neck dissection, thymectomy, median sternotomy, left thoracotomy and mediastinal lymph node dissection. Eighteen months later, a computed tomography scan of the thorax showed no evidence of disease recurrence in the neck or mediastinum.
Conclusion:
While surgical management of testicular cancer retroperitoneal metastases is well described, there is limited literature on the management of cervical lymph node metastases. In the presented case of metastatic testicular carcinoma with cervical lymph node metastases, a selective neck dissection was successfully performed for a post-chemotherapy mass.
Intrapericardial teratomas are extremely rare and most often benign tumours. In this paper, we have described a case of intrapericardial teratoma diagnosed prenatally and successfully operated. The presented case is noteworthy as an example of potentially catastrophic cardiorespiratory distress caused by the space-occupying nature of the tumour. A multi-disciplinary approach is mandatory because the tumour most often arises from the ascending aorta and in some cases may require the use of cardiopulmonary bypass.
Many of the tumors that are regarded as malignant in older children may behave in a more benign fashion in the fetus and neonate, including neuroblastoma, congenital myeloproliferative disorder in Trisomy 21, congenital fibrosarcoma, and hereditary retinoblastoma. A number of genetic, chromosomal, and syndromic associations are reported for fetal and neonatal tumors. Germ cell tumors comprise both benign and malignant tumors and can arise in both gonadal and extragonadal locations; the latter are usually found in the midline, including the sacrococcygeal area, mediastinum, and neck. Teratomas contain tissues derived from all three embryonic layers (viz. ectoderm, mesoderm, and endoderm) with a wide range of histological patterns. Neuroblastoma represents a classical embryonal tumor of neuronal lineage that may occur in the adrenal medulla and any other sites of sympathetic ganglia, from the neck to the presacral region.
This paper reports on two rare cases of neonatal airway lesions with differing aetiology that were successfully managed by surgery, and provides a review of the literature on neonatal stridor and airway lesions.
Case reports:
In the first case report, a newborn presented with a nasopharyngeal teratoma. In the second case report, a newborn presented with a congenital laryngeal saccular cyst. Difficulties in the diagnosis of these lesions, and surgical and anaesthetic challenges in their management are discussed.
Conclusion:
Every case of neonatal airway distress must be evaluated and the cause of stridor needs to be established. It is important that rare lesions such as teratomas and laryngeal cysts are not overlooked; a high index of suspicion for these congenital anomalies is necessary. These airway lesions should be managed in an institutional setting by a multidisciplinary team.
A case is presented of a nine-month-old male infant who presented acutely with an anterior neck and mediastinum mass compressing the trachea. Radiological assessment by ultrasound, magnetic resonance imaging and computed tomography suggested the nature of the mass to be a lymphatic malformation (cystic hygroma). The excised specimen was revealed as a mature teratoma. The inability to distinguish between lymphatic malformation and teratoma on multi-imaging modalities confers a risk of mismanaging these lesions with the use of sclerotherapy; surgical excision is the necessary treatment. The potential consequences are discussed.
Teratomas and dermoids or hairy polyps of the head and neck are rare congenital tumours, often confused and reported to appear as single masses, that may cause upper aerodigestive tract impairment. Their histological classification, origin and embryological basis of development have been a topic of debate in the past and recent literature.
We report a case of an infant presenting with a multifocal bigerminal teratoma in the nasopharynx and the neck, causing a deep neck infection, and discuss the unique clinicopathological aspects of this case.
A choristoma is a benign tumour-like mass consisting of mature tissue derived from one or more germ cell layers that are foreign to the site at which they are located. Choristomas of the pharynx are rare with few cases being reported in the English literature. Management of these lesions is usually complete surgical excision. We report a case of osteocartilaginous choristoma arising from the pharynx.
We describe a case of a newborn baby with a prenatal diagnosis of an epignathus (oropharyngeal teratoma). With the potential for airway problems at birth, he was delivered by an elective EXIT (Extra Utero Intrapartum Treatment) procedure at 38 weeks of pregnancy. The airway was secured and rigid bronchoscopy performed. Initially he was stable, but developed cardiorespiratory difficulties 40 minutes after birth and died from a cardiac arrest 17 minuteslater. Tension pneumothorax is a devastating complication that can occur with lower airway manipulation for anaesthesia and rigid bronchoscopy. The addition of positive pressure during mechanical ventilation converts the pneumothorax into a tension pneumothorax. The possibility of tension pneumothorax should be entertained in a mechanically ventilated patient whose ventilatory pressures are increasing, with diminishing cardiac output. A complicated case is presented, where the diagnosis was missed with a fatal outcome.
A case of synchronous cystic teratomas presented with the complaints of a slow-growing facial mass and forward protrusion of the right eye since birth. The patient was examined thoroughly and subjected to magnetic resonance imaging (MRI) scan. The MRI scan revealed the presence of two separate cysts in the right orbit and right temporal fossa. The patient was operated by a combined effort of ophthalmologists and otorhinolaryngologists. The cysts were excised and subjected to pathological examination. The histopathological survey revealed both the cysts to be mature cystic teratomas similar to each other. This coexistence of a primary temporal fossa teratoma with an orbital teratoma is the first of its kind and to the best of our knowledge has not been reported before.
Hairy polyps are rare tumours that can occur anywhere in the body. They are especially rare in the pharynx. We report two cases of hairy polyps which originated from the nasopharynx. One presentedwith intermittent obstruction of the airway and the second presented as a visible pedunculated mass protruding from the mouth of a neonate.
Teratomas are germ cell tumours usually found in the young and are characteristically composed of tissue foreign to the place where they arise. Two teratomas of the middle ear were first described in 1866 and since then, and to the best of our knowledge, 19 additional cases have been reported in the literature under different terms such as hairy congenital polyps, epidermoid cysts, dermoid cysts, hamartoma, cutaneous teratoma and teratomas. The difficulty in classifying germ cell tumours may explain the different terminologies encountered in the literature. The authors describe a case of teratoma of the eustachian tube presenting as a mass in the middle and external ear of a 10-week-old girl. Using this case, a review of the literature is performed in light of the new classification of germ cell tumours proposed by the World Health Organization (WHO). It is of note that most of the teratomas that present in the middle ear arise from, or involve, the eustachian tube.
Malignant neoplasms of the nasal cavity and paranasal sinuses are uncommon. Choriocarcinoma is a highly malignant germ cell tumour occurring in the reproductive organs. Metastasis may be principally by the lymphatic route as in other germ cell tumours but choriocarcinoma is also known to spread haematogenously. We present a rare case of metastatic choriocarcinoma to the nasal cavity from testicular teratoma presenting with intractable epistaxis in a 32-year-old Caucasian male, who ultimately succumbed to this disease.