Tracheal agenesis, a frightening scenario.

OBJECTIVE: This paper discusses three cases of tracheal agenesis that presented within a six-week period to the Norfolk and Norwich University Hospital. By reviewing the available literature on tracheal agenesis, the report aims to outline a protocol for future prenatal and postnatal management. METHODS: A case series and a literature review. RESULTS: Three cases of tracheal agenesis presented in the classical manner, with respiratory distress and unsuccessful intubation following delivery. A literature review confirmed that prenatal diagnosis requires future innovation; survival is rare and is predominately reliant on intubation of the oesophagus when a patent tracheoesophageal fistula is present. In most cases, tracheal agenesis represents part of the 'VATER' association: vertebral defects, anal atresia, tracheoesophageal fistula with oesophageal atresia, and radial or renal dysplasia. Complex, multiple-stage surgical procedures have been described; however, no survival to adolescence is documented. CONCLUSION: There is a call for improved prenatal diagnosis to allow both adequate counselling of parents and preparation for multi-specialty management at delivery. In addition, these cases highlight the ongoing need for improved congenital anomaly data within the UK, with currently only 49 per cent of England's births being registered.

Unusual middle-ear mischief: trans-tympanic trauma from a hair grip resulting in ossicular, facial nerve and oval window disruption.

A case of piercing of the tympanic membrane, resulting in unusual consequences, is described. This is the first reported case of the long process of a dislocated incus resulting in trauma to the horizontal portion of a dehiscent facial nerve. Simultaneous depression of the stapes footplate resulted in a perilymph leak, but with delayed presentation.

Spitting in the ear: a falsified disease using video evidence.

We present the case of Munchausen's syndrome by proxy (MSBP) in which the mother spat and placed blood in her child's ear, and videoed the result, in order to gain the attentions of the medical profession. This is the first case report of this kind and may represent a disturbing trend in the use of digital photography and video to support factitious illnesses.

Metastatic adenocarcinoma of the temporal bone.

Metastatic carcinoma involving the temporal bone is extremely rare. A case is reported with an unusual presentation--recurrent episodes of acute mastoiditis. Mastoid exploration and biopsy established the diagnosis. We believe this to be the first reported case to present in this manner.

Functional assessment of the paediatric laryngeal airway.

After resolution of conditions necessitating tracheostomy in children, decannulation may be extremely difficult and associated with significant morbidity. Endoscopy and radiography can identify anatomical abnormalities preventing decannulation. Physiological abnormalities are, however, more difficult to assess. A system has been developed whereby functional upper airway resistance can be calculated in children with a tracheostomy. The instrument used was a modified Mercury Electronics rhinomanometer. A probe was placed through the tracheostome and another probe was situated in an occluding anaesthetic face mask. Upper airway resistance was derived from the differential pressure and flow changes recorded in anaesthetized children who were breathing spontaneously. Studies in 26 children have revealed low airway resistance in all those successfully decannulated. Further studies will hopefully allow identification in advance of children who will have decannulation difficulties.

Computed tomography in choanal atresia.

Computed tomography is replacing contrast radiography as the method of choice in evaluating congenital posterior choanal atresia. Careful preparation of the patient, however, is necessary prior to the examination in order to obtain the maximum of information pre-operatively. We present patients with inadequate preparation and illustrate their misleading scans. The proper method of examination is discussed, with a review of the literature; it is essential to remove nasal secretions by suction and instill vasoconstrictor nose drops, shortly before scanning.

Meningitis after trans-sphenoidal excision of pituitary tumours.

Between 1974 and 1986, eleven of 114 patients undergoing trans-sphenoidal removal of pituitary tumours developed meningitis despite prophylaxis, usually with chloramphenicol. Nine patients had cerebrospinal fluid rhinorrhoea and one died. A variety of pathogens was isolated, including enterobacteria, and four of the eleven were resistant to the antibiotics given as prophylaxis. Enterobacterial meningitis was always associated with infection of the sphenoidal sinus involving the muscle graft or nasal pack (five cases), and removal of the muscle graft was necessary in three cases despite the use of appropriate antibiotics.