Clinical Case Reports – 1970 by P. Syamasundar Rao in Journal of Clinical Case Reports Medical Images and Health Sciences
Abstract
In
this paper five case reports were presented and include congenital
pulmonary cyst, Wilson-Mikity syndrome, diaphragmatic eventration;
foreign body in the bronchus, and cor pulmonale that developed after
implantation of a ventriculo-atrial shunt with a Pudenz-Heyer valve for
treatment of hydrocephalus. For each case report, clinical, chest x-ray,
electrocardiogram and other pertinent findings were presented. This was
followed by discussion of etiology, diagnosis, and treatment options,
as appropriate.
Keywords: congenital pulmonary cyst; diaphragmatic eventration; Wilson-Mikity syndrome; foreign body in the bronchus; cor pulmonale
Introduction
During
the academic clinical practice for over five decades, the author had
the unique opportunity to observe and document many interesting clinical
case scenarios. The purpose of this review is to revisit these
interesting cases. Because of the voluminous amount of this material,
the material may be divided into a five-part series. Each of these case
reports, while rare and important clinical observations, do demonstrate a
clinical point that is useful to the pediatricians, pediatric
cardiologists and/or other physicians.
Congenital Pulmonary Cyst
Case Report
A female infant with a birth weight of 6 lb 7 oz, born after a
full-term, normal pregnancy and delivery with an Apgar score of 9
presented at three weeks of age with a two-week history of tachypnea.
There were no other symptoms and the infant’s physical examination was
normal except for tachypnea (respiratory rate of 50 per minute) and mild
inter-costal and sub costal retractions. A chest roentgenogram was
obtained (Figure 1) which was interpreted as pulmonary cyst. The heart
was pushed to the right by the cyst (dextroposition of the heart). At
thoracotomy, a huge lung cyst, involving the lower lobe of the left
lung, was found, and was resected and the patient made an uneventful
recovery.
Congenital pulmonary cysts in the neonate are uncommon and are
considered as errors in embryological development. They are of several
categories namely, bronchogenic cell, alveolar cell, and combined cell
types, based on the cellular component of the cell wall of the cyst. The
symptoms depend largely upon the size of the cyst. These patients may
not be discovered until a chest x-ray is performed for other reasons or
may present with symptoms of tachypnea, dyspnea, and cyanosis in the
neonatal period secondary to compression of lung tissue. The findings
depend upon the size and location of the cyst. Dextroposition of the
heart or tracheal shift and hyper-resonance, diminished breath sounds,
and rales may be detected on physical examination. The chest x-ray
findings may demonstrate a cyst, as in our case (Figure 1) or may be
misinterpreted as pneumothorax. Other conditions simulating the cyst are
staphylococcal pneumonia, diaphragmatic hernia, congenital lobar
emphysema, sequestrated lobe, and hydro-pneumothorax or
pyo-pneumothorax. In symptomatic cases, cystectomy, segmentectomy,
lobectomy, or pneumonectomy, depending upon the size and location of the
cyst is suggested. Percutaneous aspiration of the cyst is not
recommended except as an emergency measure to relieve the tension. Some
authorities advocate no surgical intervention because of the possibility
of spontaneous regression of the pulmonary cysts, but most authorities
recommend surgical excision of the cysts [1].
Late Respiratory Distress in a Premature Infant
Case Report
A premature male infant was born at 25 weeks of gestation and weighed 2 lb 12 oz at birth. Abruptio placenta and prolapse of the umbilical cord
complicated the delivery and required resuscitation with oxygen. The
chest x-ray was normal at that time. The baby was placed in an incubator
in 35 percent oxygen, which was discontinued within 24 hours. At the
age of 31 days, tachypnea and recurrent apnea with cyanosis developed. Auscultation revealed bilateral rales in the chest, again necessitating resuscitation with O2, administered by bag and mask. Chest x-ray (Figure 2)
revealed a diffuse parenchymal reticular pattern with multifocal areas
of radiolucency. This roentgenographic pattern, along with the clinical
findings, is essentially diagnostic of the Wilson-Mikity syndrome.
Discussion
Wilson an Mikity originally described this condition in 1960, and is now called Wilson-Mikity syndrome.2 The etiology is not clearly understood but is considered to be due to pulmonary dysmaturity with uneven postnatal development of pulmonary alveoli in the premature infants.2 No consistent relationship with O2 therapy has been established. Bronchopulmonary dysplasia is another condition seen in the neonatal period
and should be distinguished from Wilson-Mikity syndrome. The cystic
appearance on the chest x-ray in the third stage of bronchopulmonary
dysplasia resemble those of Wilson-Mikity syndrome; however, it follows
treatment of severe hyaline membrane disease with high concentrations of O2 and artificial ventilation.2
The clinical presentation of Wilson-Mikity syndrome is characteristic
in that the infant is premature with minimal or no respiratory distress
at birth but, develops progressive respiratory distress, with dyspnea, tachypnea, cough, cyanosis, and rales
in a few days to weeks. Diffuse reticular pattern of both lungs with
areas of multifocal radiolucency are usually seen, similar to those seen
in figure 2. Progressive pulmonary insufficiency with signs of right heart failure develop in patients with fatal outcome.
But, about half of the patients eventually recover from their pulmonary
disease. Pulmonary function studies are abnormal with decreased lung
compliance, increased expiratory flow resistance, and increased breathing effort. Respiratory acidosis develops in spite of increased minute volume. Arterial O2 desaturation is thought to be secondary to intrapulmonary right-to-left shunting.2 The treatment is largely supportive [2].
Fever, Vomiting and Dome-Shaped Density in Right Thorax
Case Report
A four-month-old boy presented with a history of fever, poor feeding, vomiting, and slight cough for two days. Past history is essentially normal except for an Apgar score of 6 at birth. Breath sounds
were diminished at the right base. Laboratory studies were normal.
Chest x-ray (Figure 3) was performed which revealed a dome-shaped
density in the right thorax
which did not coincide with any pulmonary lobe or segment. The
elevation of the inferior liver margin in the abdomen indicated that the
abnormal shadow was liver. Based on these findings eventration of the
right hemi-diaphragm was suspected. To confirm the diagnosis, a
diagnostic pneumoperitonium was performed (Figure 4) which confirmed the diagnosis.
Discussion
Eventration
of the diaphragm is classified into adult and infantile types [3]. It
is generally thought to be the result of congenital mal-development of
the diaphragmatic musculature. However, such an abnormality may
occasionally be caused by phrenic nerve injury
during birth. The true incidence of eventration is not known, but in
mass x-ray surveys of adults, it was found to be one in 10,000 [3].
Total eventration is thought to be more common on the left side and
partial eventration on the right [3].
Clinical findings largely depend on the extent of eventration. There may be no symptoms or the patient may present with dyspnea, tachypnea, and cyanosis in the newborn period,
requiring immediate treatment. Seesaw cyclic motions of the epigastrium
with respiration and Hoover's sign (uninhibited divergence of costal
margin from midline on inspiration), if present, are helpful in making
the diagnosis. Percussion on the affected side may be dull or tympanic depending on the organs migrated under the diaphragm.
Fluoroscopy
and chest x-rays are generally useful in arriving at the diagnosis. In
right-sided eventrations, the lesser amount of liver shadow in the
abdomen, i.e., elevation of the inferior margin of the liver helps to
distinguish eventration from the other conditions [3]. Diagnostic
pneumoperitonium is likely to establish the diagnosis, but the current
availability of ultrasound technology, diagnostic pneumoperitoneum may
not be necessary at the present time.
Symptomatic newborns with diaphragmatic eventration should be treated
surgically; plication of the eventrated diaphragm is successful in
relieving the symptoms with good long-term results. Some authorities
suggest that asymptomatic patients also should be addressed surgically
[3].
Foreign Body (Peanut) in The Left Main Stem Bronchus
Case Report
A 13-month-old girl with a history of poor appetite, loss of weight,
cough, and intermittent low grade fever was admitted to the hospital for
evaluation and treatment. No history of choking episodes was elicited.
History revealed that a relative who had active pulmonary tuberculosis
lived with the infant's family for a short period of time four months
prior to the current admission. Because of this reason, the local health
department performed tuberculin skin test which was positive and
treatment with isoniazid was initiated. On examination her weight and
height were between the third and tenth percentile. Decreased breath
sounds on auscultation and hyper tympanic note on percussion were noted
over the left side of the chest.
Intermediate strength purified protein derivative (PPD) was positive.
Chest roentgenograms were obtained (Figure 5). Based on the history,
physical examination, and chest x-ray findings, a diagnosis of
endobronchial tuberculosis was entertained. However, prior to beginning
treatment, bronchoscopy was performed to appraise the extent of airway
encroachment.
Discussion
Autoimmune
encephalitis is a condition that can be easily missed as it is not
commonly considered in the differential diagnosis of various medical
presentations. However, such diagnosis should be always taken into
consideration when a person, particularly a child, presents with a new
onset of refractory status epilepticus (NORSE) and/or new behavioral or
psychiatric conditions. An early diagnosis of AE is essential, as the
treatment is different from other conditions. With correct timely
interventions the outcome is frequently favorable.
Though SARS-Cov-2 virus rarely invades the nervous system, Covid-19
infection frequently causes neurological symptoms like headache,
delirium, anosmia, and dysgeusia [14]. One of the mechanisms of indirect
nervous system involvement is through inflammatory response and immune
dysregulation. There are few recorded cases of indirect involvement of
CNS by auto-antibodies that are directed against the surface and
synaptic protein. This case is one of the rare cases of Anti NMDA
antibody autoimmune encephalitis that is associated with Covid-19
infection [15]. It indicates that in the era of COVID-19, high vigilance
is required as a possible association may increase AE incidence.
A recent systemic review that analyzed 16 studies, including a total
of 161 patients with NORSE [16], showed that the most frequent cause was
AE. In addition to the well-known association with teratoma and cancer,
AE, and specifically Anti-NMDA receptor Ab encephalitis, could be
associated with a SARS‑CoV‑2 infection, either concomitantly or as
post-infection manifestation. In this reported case, immunotherapy, in
addition to anti-seizure medication, showed to be effective.
The main limitation of this report is the relatively short follow-up
period. Observation of the child is ongoing to detect possible medium-
or long-term consequences.
nfant with poor appetite, loss of weight, and
fever is suggestive of primary tuberculosis. This is particularly so
given the patient's exposure to a subject with active pulmonary
tuberculosis. The x-rays show hyper aeration of the left lung with a
shift of the heart and mediastinum to the right. The left leaf of the
diaphragm is also flattened. While there are no areas of infiltration or
consolidation were seen, prominent shadows suggesting enlarged lymph
nodes were seen (arrows in figure 5). Endobronchial tuberculosis with
compression of the bronchus by adenopathy may produce changes seen
figure 5.
Discussion
Even though there was no history of choking or aspiration, the
possibility of foreign body aspiration should be considered in this age
group. Consequently, bronchoscopy was performed which revealed a peanut
in the left main stem bronchus and was extracted during bronchoscopy.
The peanut and the adjacent edema of the bronchus caused partial
bronchial obstruction and acted as a check valve, so the air entered the
left lung but, unable to leave the left lung since the bronchus becomes
smaller during expiration, producing the roentgenographic appearance
shown in figure 5. The baby improved and the treatment with isoniazid
was continued because of the positive PPD.
Cor Pulmonale as a Complication of Ventriculoatrial Shunts
Introduction
Cerebral ventricle-to-right atrial shunts with Pudenz-Heyer or
Spitz-Holter valves were widely used to treat hydrocephalus in the
1960s. Development of pulmonary hypertension with chronic cor pulmonale
is rare with these shunts. We reported a patient who developed such a
complication along with description of specialized pulmonary function
studies in the early detection of such complication [5].
Case Report
An 11-year-old white boy was hospitalized in April 1969 with a
history of progressive weakness, dyspnea, and pedal edema. He was
diagnosed to have hydrocephalus and had a ventriculo-atrial shunt with a
Pudenz-Heyer valve implanted at the age of 6 months. The shunt was
thought to be functioning well when he was evaluated at the age of 2
years. He was asymptomatic until he was 9.5 years old, when he developed
signs of congestive heart failure (CHF) and was treated at another
hospital with digitalis and diuretics with some improvement. Right heart
catheterization at the same institution revealed a mean right atrial
pressure of 35 mmHg and right atrial angiography revealed slow emptying
of the contrast, filling defects on the right lateral atrial wall and in
the right and left pulmonary arteries. The ventriculo-atrial shunt was
removed shortly thereafter. The patient was referred to our group for
further evaluation and management [5].
Pertinent findings on examination included height and weight below
the third percentile, head circumference above the 97th percentile,
pretibial edema, prominent “a” wave in the left side of the neck, no
venous pulsations on the right side, palpable right ventricular heave,
markedly accentuated single second heart sound, an audible fourth heart
sound at left lower sternal border, a Grade I/VI ejection systolic
murmur at the mid-left sternal border, liver edge palpable 5 cm below
the right costal margin, clear lung fields on auscultation, and normal
neurological examination.
Electrocardiogram (ECG) (Figure 6) and the vectorcardiogram (not
shown) revealed right atrial and ventricular hypertrophy. Chest
roentgenogram (Figure 7) showed moderate cardiomegaly and prominent main
pulmonary artery (PA) segment and clear lung fields. Lung scan with 131I-labeled macro-aggregated albumin was suggestive of multiple pulmonary emboli. Blood gas analysis showed pH 7.56; PaO2 80 mmHg, PaCO2
23 mmHg and bicarbonate 24 mEq/liter. Routine pulmonary function
studies revealed restrictive lung disease. The ratio of wasted
ventilatory volume (physiological dead space) to tidal volume (VD:VT) using Bohr's equation was 0.58 (normal 0.3 or less).
Vigorous treatment with digitalis and diuretics resulted in only
temporary relief. During the next year, he continued to deteriorate and
died of intractable right ventricular failure. Postmortem revealed right
atrial thrombosis, severe right ventricular hypertrophy, multiple
thrombo-emboli in the large and medium-sized pulmonary arteries, and
intimal proliferation of the pulmonary arterioles.
Discussion
The case presented demonstrated development of cor pulmonale
secondary to pulmonary thrombo-embolism which was produced by thrombi
that arose following a ventriculo-atrial shunt with a Pudenz-Heyer valve
for treatment of hydrocephalus. The causes of thrombo-embolic
complications were not well understood, but the hypotheses, as reviewed
by us [5], include infection, periarteritis due to autoimmune reaction
of the pulmonary vessels to protein of cerebrospinal fluid, release of
brain thromboplastin resulting in thrombosis at the point of contact
with plasma coagulation factors, and simply the presence of a foreign
body in the cardiovascular system for prolonged periods of time.
Early detection of pulmonary hypertension by periodic (every six
months) evaluation by chest x-ray and ECG studies was suggested by some
investigators, but early detection of pulmonary hypertension is of
limited value since obstruction of 60% of the pulmonary vascular bed
occurs by the time pulmonary hypertension develops [5]. Detection of
multiple filling defects on radioisotope scanning in a child with a
ventriculo-atrial shunt would be suggestive of pulmonary embolization
and might be useful in early identification. Based on the observations
of Nadel and associates [6] and those of ours [5], we suggested that
specialized pulmonary function studies such as VD:VT,
pulmonary diffusing capacity, pulmonary capillary blood volume, blood
gas, and pH be performed periodically to detect obstruction of pulmonary
vasculature prior to the development of pulmonary hypertension and cor
pulmonale [5]. However, it should be noted that ventriculo-atrial shunts
are no longer performed to treat hydrocephalus, but instead
ventriculo-peritoneal shunts are used at the present time.
In summary, a rare case of pulmonary thrombo-embolism with resultant
pulmonary hypertension and cor pulmonale following ventriculo-atrial
shunt for hydrocephalus was presented with the recommendation to use of
special pulmonary function studies for early detection and if found to
be positive, immediate removal of the shunt system may eliminate further
embolization into the lungs and prevent irreversible pulmonary vascular
disease.
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